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Sample records for sporadic renal tumors

  1. Renal inflammatory myofibroblastic tumor

    DEFF Research Database (Denmark)

    Heerwagen, S T; Jensen, C; Bagi, P

    2007-01-01

    Renal inflammatory myofibroblastic tumor (IMT) is a rare soft-tissue tumor of controversial etiology with a potential for local recurrence after incomplete surgical resection. The radiological findings in renal IMT are not well described. We report two cases in adults with a renal mass treated...

  2. Clinical Significance of Microsatellite Instability in Sporadic Epithelial Ovarian Tumors

    OpenAIRE

    Yoon, Bo-Sung; Kim, Young-Tae; Kim, Jae-Hoon; Kim, Sang-Wun; Nam, Eun-Ji; Cho, Nam-Hoon; Kim, Jae-Wook; Kim, Sunghoon

    2008-01-01

    Purpose We evaluated the expression of microsatellite instability (MSI) in sporadic ovarian tumors using 5 standard and 9 new MSI markers to determine the clinical significance of MSI in sporadic epithelial ovarian tumors. Materials and Methods MSI was examined in 21 borderline and 25 malignant ovarian tumors. Polymerase chain reaction (PCR) was performed using the 5 markers recommended by the National Cancer Institute (NCI) for colon cancer and 9 additional markers. MSI was determined using ...

  3. Systematic comparison of sporadic and syndromic pancreatic islet cell tumors.

    Science.gov (United States)

    Erlic, Zoran; Ploeckinger, Ursula; Cascon, Alberto; Hoffmann, Michael M; von Duecker, Laura; Winter, Aurelia; Kammel, Gerit; Bacher, Janina; Sullivan, Maren; Isermann, Berend; Fischer, Lars; Raffel, Andreas; Knoefel, Wolfram Trudo; Schott, Matthias; Baumann, Tobias; Schaefer, Oliver; Keck, Tobias; Baum, Richard P; Milos, Ioana; Muresan, Mihaela; Peczkowska, Mariola; Januszewicz, Andrzej; Cupisti, Kenko; Tönjes, Anke; Fasshauer, Mathias; Langrehr, Jan; von Wussow, Peter; Agaimy, Abbas; Schlimok, Günter; Lamberts, Regina; Wiech, Thorsten; Schmid, Kurt Werner; Weber, Alexander; Nunez, Mercedes; Robledo, Mercedes; Eng, Charis; Neumann, Hartmut P H

    2010-12-01

    Pancreatic islet cell tumors (ICTs) occur as sporadic neoplasias or as a manifestation of multiple endocrine neoplasia type 1 (MEN1) and von Hippel-Lindau disease (VHL). Molecular classification of ICTs is mandatory for timely diagnosis and surveillance. Systematic comparison of VHL-ICTs and sporadic ICTs has been lacking. Our registry-based approaches used the German NET-Registry with 259 patients with neuroendocrine tumors (NETs), who were primarily diagnosed with NETs, and the German VHL-Registry with 485 molecular genetically confirmed patients who had undergone magnetic resonance imaging or computed tomography of the abdomen. All patients provided blood DNA for testing of the MEN1 and VHL genes for intragenic mutations and large deletions. In the NET-Registry, 9/101 patients (8.9%) with ICTs had germline mutations, 8 in MEN1 and 1 in VHL. In the VHL-Registry, prevalence of NETs was 52/487 (10.6%), and all were ICTs. Interestingly, of those with VHL p.R167W, 47% developed ICTs, compared to 2% of those with p.Y98H. In total, there were 92 truly sporadic, i.e. mutation-negative ICT patients. Comparing these with the 53 VHL-ICT patients, the statistically significant differences were predominance of female gender (P=0.01), multifocal ICTs (P=0.0029), and lower malignancy rate (PICTs compared to sporadic cases. VHL was prevalent in ICTs, which are rarely the first presentation. Patients with NETs should not be subjected to genetic testing of the VHL gene, unless they have multifocal ICTs, other VHL-associated tumors, and/or a family history for VHL.

  4. Malignant renal tumors in pediatrics

    International Nuclear Information System (INIS)

    Pena, C.; Torterolo, J.; Irigoyen, B.; Bel, M.; Elias, E.

    2004-01-01

    Introduction: Professionals who work in pediatric oncology, we see childhood cancer as a common disease, but in fact constitutes about 2% of all cancers diagnosed worldwide. Wilms tumor accounts for 6% of all childhood tumors and presentation bilateral accounts for 4-6% of all Wilms tumors diagnosed. Theoretical Framework: In the period between the year 1994-2003 period were attended in the Pediatric Hematology-Oncology Center, a total of 29 cases of malignant renal tumors, corresponding to 86% (25 cases) to Wilms tumor or nephroblastoma tumor. The Wilms is of embryonic origin, capable of metastatic spread, (85% lungs 15% liver). Very sensitive to chemotherapy and radiotherapy, which confers high cure rates (85%); having a multidisciplinary treatment model, combining surgery, chemotherapy, and radiotherapy. The role of nursing in comprehensive cancer care child is essential in the prevention and early detection of side effects or complications. Case report: S.D. currently 10 years old. In 10/1994, at 8 months of age, was diagnosed with bilateral Wilms tumor. On admission her weight was 8200gr with abdominal circumference 50cm. Conducted pre-operative MDT and 02/1995 nephrectomy of the left kidney and right kidney lumpectomy (tumor nodule 420gr. and a 250gr.). MDT begins in 03/1995 01/1996 ending. 09/2003 with abdominal pain and vomiting, and kidney failure. 10/2003 lumpectomy biopsy (sclerotic nodule associated with maturation nephroblastoma). Currently severe renal insufficiency plan enters dialysis. Nursing process: Objectives: 1) To prepare the child and family to the side effects and possible complications of chemotherapy and / or radiotherapy 2) Prevent and minimize related complications tumor and / or treatment. Care Plan comprises four stages: A) rating and customer income. B) Implement care chemotherapy C) post-operative Care D) Implement radiation care

  5. Ossifying renal tumor of infancy

    International Nuclear Information System (INIS)

    El-Husseini, Tareq K.; Al-Orf, Ali M.; Egail, Siddig A.; Mostert, C.

    2005-01-01

    Ossifying renal tumor of infancy ORTI is a benign neoplasm, which presents with gross hematuria and less frequently as an abdominal mass, histologically it comprises a large cell with an epithelial nature and osteoid formation. We report a case of a 10-month-old girl who developed ORTI as non-opacified upper calyces of left kidney on excretory urography. We outlined the calcific and tumors nature of the lesion by ultrasound and computed tomography. The macroscopic and histological features were pathognomonic. (author)

  6. Sporadic Endolymphatic Sac Tumor-A Very Rare Cause of Hearing Loss, Tinnitus, and Dizziness

    DEFF Research Database (Denmark)

    Schnack, Didde Trærup; Kiss, Katalin; Hansen, Søren

    2017-01-01

    Sporadic endolymphatic sac tumor is a very rare neoplasm. It is low malignant, locally destructive and expansive, but non-metastasizing. The tumor is very rare in the sporadic form, but more often associated with Von Hippel-Lindau disease. A 65-year old man with left sided tinnitus and hearing loss......-operative freeze-microscopy showed inflammation tissue, whereas subsequent microscopy showed papillary-cystic endolymphatic sac tumor. Endolymphatic sac tumor is a rare neoplasm. The tumor may present with asymmetrically sensory neural hearing loss with or without tinnitus, dizziness and facial nerve paresis...

  7. Carcinoid tumor of the kidney: An unusual renal tumor

    Directory of Open Access Journals (Sweden)

    P P Singh

    2009-01-01

    Full Text Available Carcinoid tumors are low-grade malignant tumors that arise from neuroendocrine cells. Primary renal carcinoid is extremely rare. We present a case of 57-year-old male with primary renal carcinoid tumor. Presently, the patient is on regular follow up and is doing well.

  8. Birt-Hogg-Dubé renal tumors are genetically distinct from other renal neoplasias and are associated with up-regulation of mitochondrial gene expression

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    Yonneau Laurent

    2010-12-01

    Full Text Available Abstract Background Germline mutations in the folliculin (FLCN gene are associated with the development of Birt-Hogg-Dubé syndrome (BHDS, a disease characterized by papular skin lesions, a high occurrence of spontaneous pneumothorax, and the development of renal neoplasias. The majority of renal tumors that arise in BHDS-affected individuals are histologically similar to sporadic chromophobe renal cell carcinoma (RCC and sporadic renal oncocytoma. However, most sporadic tumors lack FLCN mutations and the extent to which the BHDS-derived renal tumors share genetic defects associated with the sporadic tumors has not been well studied. Methods BHDS individuals were identified symptomatically and FLCN mutations were confirmed by DNA sequencing. Comparative gene expression profiling analyses were carried out on renal tumors isolated from individuals afflicted with BHDS and a panel of sporadic renal tumors of different subtypes using discriminate and clustering approaches. qRT-PCR was used to confirm selected results of the gene expression analyses. We further analyzed differentially expressed genes using gene set enrichment analysis and pathway analysis approaches. Pathway analysis results were confirmed by generation of independent pathway signatures and application to additional datasets. Results Renal tumors isolated from individuals with BHDS showed distinct gene expression and cytogenetic characteristics from sporadic renal oncocytoma and chromophobe RCC. The most prominent molecular feature of BHDS-derived kidney tumors was high expression of mitochondria-and oxidative phosphorylation (OXPHOS-associated genes. This mitochondria expression phenotype was associated with deregulation of the PGC-1α-TFAM signaling axis. Loss of FLCN expression across various tumor types is also associated with increased nuclear mitochondrial gene expression. Conclusions Our results support a genetic distinction between BHDS-associated tumors and other renal

  9. KCNQ1OT1 hypomethylation: A Novel disguised genetic predisposition in sporadic pediatric adrenocortical tumors?

    NARCIS (Netherlands)

    Wijnen, Mark; Alders, Mariëlle; Zwaan, Christian M.; Wagner, Anja; van den Heuvel-Eibrink, Marry M.

    2012-01-01

    Pediatric adrenal tumors, other than neuroblastoma, are rare and can be associated with a genetic predisposition. In this report we describe two patients with an isolated and apparently sporadic adrenocortical tumor; one girl with a carcinoma, the other girl with an adenoma. In both patients genetic

  10. Usefulness of MR angiography in renal tumor

    International Nuclear Information System (INIS)

    Oka, Toshitsugu; Morimoto, Kouji; Nishimura, Kenji; Tsujimura, Akira; Yasunaga, Yutaka; Matsumiya, Kiyomi; Takaha, Minato

    1992-01-01

    MR angiography using a gradient-echo, pulse sequence FLASH (fast, low-angle shot) method during breath-hold with a 'MAGNETOM H-15' scanner (1.5 Tesla; Siemens Medical System) was performed on 27 patients with renal tumor at our clinic between Feburary 20, 1990 and September 30, 1991 and we studied to evaluate its usefulness. Of these 27 patients, 22 patients including one patient under hemodialysis treatment had renal cell carcinoma and one patient had oncocytoma pathologically proven from the excised specimens. The remaining four patients including two patients associated with inferior vena cava tumor thrombus were clinically diagnosed as renal cell carcinoma based on the result of imaging examinations such as excretory urography, ultrasonography, computed tomography and conventional angiography. However, they could not be operated on because their tumors were too advanced. By reconstruction of the data of consecutive coronal scans of the abdominal blood vessels such as the abdominal aorta, inferior vena cava and renal arteries and veins simultaneously without any intravenous contrast materials. Our present study revealed that MR angiography has some advantages, especially with regard to preoperative angiographic information about the abdomen of patients with renal tumor. That is, MR angiography can delineate many kinds of arteries and veins of the abdomen simultaneously and in a broader range, as well as it can be performed on the patients with hypersensitivity to iodinate contrast materials or renal insufficiency in a usual fashion. Furthermore, our present study suggested that the MR angiography is useful for assessing the presence and extent of inferior vena caval tumor thrombus of renal cell carcinoma and for clearly distinguishing tumor lesion and the surrounding normal renal parenchyma in the patients with renal tumor. (author)

  11. Radiofrequency ablation for renal tumors. Our experience

    International Nuclear Information System (INIS)

    Hiraoka, Kenji; Kawauchi, Akihiro; Nakamura, Terukazu; Soh, Jintetsu; Mikami, Kazuya; Miki, Tsuneharu

    2009-01-01

    The objective of this study was to report our results of percutaneous radiofrequency ablation (RFA) for renal tumors and to assess predictors of therapeutic efficacy. Forty patients (median age 73 years) with renal tumors were treated with RFA under local or epidural anesthesia. All of them had high surgical risk or refused radical surgery. Tumors were punctured percutaneously using the Radionics Cool-tip RF System under computed tomography or ultrasonographic guidance. Median tumor diameter was 24 mm. After RFA, contrast-enhanced computed tomography or magnetic resonance imaging was performed within 1 month. Complete response (CR) was defined as no enhancement inside the tumor. Factors related to the outcome and to renal function were assessed. Median follow up was 16 months. CR was observed in 34 cases (85.0%). A significant difference in CR rate was observed between tumors ≤30 mm and those >30 mm. Outcomes tended to be better for tumors in the mid to lower kidney, and those away from the renal hilum. Recurrence was observed in one case (2.9%), but a CR was obtained again by additional RFA. Out of a total of 77 RFA procedures, complications occurred in only three cases (3.9%), and conservative treatment was possible in all cases. Serum creatinine levels 3 months after RFA did not differ from those before RFA. Percutaneous RFA is a safe and effective treatment for small renal tumors in patients with high surgical risk or who refuse radical surgery. (author)

  12. Differential response of idiopathic sporadic tumoral calcinosis to bisphosphonates

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    Karthik Balachandran

    2014-01-01

    Full Text Available Context: Tumoral calcinosis is a disorder of phosphate metabolism characterized by ectopic calcification around major joints. Surgery is the current treatment of choice, but a suboptimal choice in recurrent and multicentric lesions. Aims: To evaluate the efficacy of bisphosphonates for the management of tumoral calcinosis on optimized medical treatment. Settings and Design: The study was done in the endocrine department of a tertiary care hospital in South India. We prospectively studied two patients with recurrent tumoral calcinosis who had failed therapy with phosphate lowering measures. Materials and Methods: After informed consent, we treated both patients with standard age adjusted doses of bisphosphonates for 18 months. The response was assessed by X ray and whole body 99mTc-methylene diphosphonate bone scan at the beginning of therapy and at the end of 1 year. We also estimated serum phosphate levels and urinary phosphate to document serial changes. Results: Two patients (aged 19 and 5 years with recurrent idiopathic hyperphosphatemic tumoral calcinosis, following surgery were studied. Both patients had failed therapy with conventional medical management − low phosphate diet and phosphate binders. They had restriction of joint mobility. Both were given standard doses of oral alendronate and parenteral pamidronate respectively for more than a year, along with phosphate lowering measures. At the end of 1 year, one of the patients had more than 95% and 90% reduction in the size of the lesions in right and left shoulder joints respectively with total improvement in range of motion. In contrast, the other patient (5-year-old had shown no improvement, despite continuing to maintain normophosphatemia following treatment. Conclusions: Bisphosphonate therapy in tumoral calcinosis is associated with lesion resolution and may be used as a viable alternative to surgery, especially in cases with multicentric recurrence or treatment failure to other

  13. Absence of RET proto-oncogene abnormalities in sporadic parathyroid tumors

    Energy Technology Data Exchange (ETDEWEB)

    Pausova, Z.; Janicic, N.; Konrad, E. [McGill Univ. and Royal Victoria Hospital, Montreal (Canada)] [and others

    1994-09-01

    Parathyroid tumors can occur either sporadically or as a part of inherited cancer syndromes such as multiple endocrine neoplasia (MEN) type 2A. Recently, development of this syndrome has been shown to be related to specific mutations in the RET proto-oncogene, a putative receptor tyrosine kinase. Activation of this proto-oncogene has been demonstrated not only in tumors of the MEN 2A syndrome, but also in other neoplasia of neuroectoderm origin, namely papillary thyroid carcinoma where a rearrangement of the RET proto-oncogene has been found. In the present study, a role of the RET proto-oncogene in the development of sporadic parathyroid tumors was investigated by analyzing DNA samples obtained from 13 parathyroid adenomas and 6 parathyroid hyperplasias. Southern blot, using BamHI restricted DNA, did not reveal any gross alteration of the gene. Polymerase chain reaction (PCR) was then employed to amplify DNA fragments corresponding to exons 10 and 11 in which all MEN 2A mutations have been identified. Amplified DNA fragments were all of expected size (exon 10, 182 bp; exon 11, 233 bp). Since a single point mutation at codon 634 has been found to be associated in close to 90% of cases with development of parathyroid tumors in patients with the MEN 2A syndrome, exon 11, containing this codon, was further examined by direct sequence analysis. Sequences obtained from all tumors tested, however, did not differ from the wild type sequence. Therefore, the mutation of the RET proto-oncogene commonly associated with parathyroid neoplasias in MEN 2A is uncommon in sporadic parathyroid tumors. This suggests that the pathogenesis of parathyroid tumors occurring sporadically may be different from those occurring in patients with the MEN 2A syndrome.

  14. Tumor Enucleation for Renal Cell Carcinoma

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    Zachary L Smith

    2015-03-01

    Full Text Available The increased number of small renal masses (SRMs detected annually has led to a rise in the use of nephron-sparing surgery (NSS.  These techniques aim to preserve the largest amount of healthy renal tissue possible while maintaining the same oncologic outcomes as radical nephrectomy (RN.  Additionally, partial nephrectomy (PN has been linked to a lower risk of chronic kidney disease, cardiovascular morbidity, and mortality when compared to RN.  There has been continual progress toward resecting less renal parenchyma.  While the predominant surgical method of performing NSS is through traditional PN, simple enucleation (SE of the tumor has increased in popularity over recent years.  SE is a technique that aims to preserve the maximal amount of renal parenchyma possible by utilizing the renal tumor pseudocapsule to bluntly separate the lesion from its underlying parenchyma, offering the smallest possible margin of excised healthy renal tissue.  Several studies have demonstrated the oncological safety of SE compared with PN in the treatment of SRMs, with lower overall incidence of positive surgical margins.  Additionally, SE has been shown to have similar 5- and 10-year progression-free and cancer-specific survival as PN.  We present a review of the literature and an argument for SE to be a routine consideration in the treatment of all renal tumors amenable to NSS.

  15. Conservative management of small renal tumors

    International Nuclear Information System (INIS)

    Matsuzaki, Masato; Kawano, Yoshiyuki; Morikawa, Hirofumi; Shiga, Yoshiyuki; Murata, Hirokatsu; Komatsu, Hideki

    2007-01-01

    With the widespread use of imaging modalities, incidentally discovered small renal cell carcinomas have increased. Some patients, however, are too old or weak due to various diseases to undergo surgery and other patients occasionally refuse surgery. To investigate the natural history of small renal cell carcinoma, we retrospectively reviewed patients with small renal tumors suggestive of carcinoma. We retrospectively reviewed 15 patients with contrast-enhancing renal masses less than 4.0 cm in diameter who were observed without treatment. The mean follow-up period was 38 months (range, 8-91). The average patient age was 67 years (range, 44-87). The initial average tumor diameter was 2.2 cm (range, 1.0-3.9). The average growth rate was 0.06 cm per year (range, -0.09-0.28). Only 4 tumors grew obviously during the follow-up period. Three tumors were removed surgically by radical nephrectomy, and all tumors were pathologically diagnosed as renal cell carcinoma. None of the patients developed metastases during the follow-up period or after surgery. Two patients died of other causes. Nonsurgical watchful waiting may be an acceptable treatment option for elderly or severely comorbid patients; however, it is not known whether this conservative management can he applied to young or otherwise healthy patients. (author)

  16. Contemporary treatment of renal tumors

    DEFF Research Database (Denmark)

    Nisen, Harry; Järvinen, Petrus; Fovaeus, Magnus

    2017-01-01

    Objective: The five Nordic countries comprise 25 million people, and have similar treatment traditions and healthcare systems. To take advantage of these similarities, a collaborative group (Nordic Renal Cancer Group, NORENCA) was founded in 2015. Materials and methods: A questionnaire of 17...

  17. Expression of the Circadian Clock Genes Pert, Per2 in Sporadic, Familial Breast Tumors

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    Sherry L. Winter

    2007-10-01

    Full Text Available There is a growing body of evidence implicating aberrant circadian clock expression in the development of cancer. Based on our initial experiments identifying a putative interaction between BRCA1, the clock proteins Per1, Per2, as well as the reported involvement of the circadian clock in the development of cancer, we have performed an expression analysis of the circadian clock genes Per1, Per2 in both sporadic, familial primary breast tumors, normal breast tissues using real-time polymerase chain reaction. Significantly decreased levels of Per1 were observed between sporadic tumors, normal samples (P < .00001, as well as a further significant decrease between familial, sporadic breast tumors for both Per1 (P < .00001, Per2 (P < .00001. Decreased Per1 was also associated with estrogen receptor negativity (53% vs 15%, P = .04. These results suggest a role for both Perl, Per2 in normal breast function, show for the first time that deregulation of the circadian clock may be an important factor in the development of familial breast cancer. Aberrant expression of circadian clock genes could have important consequences on the transactivation of downstream targets that control the cell cycle, on the ability of cells to undergo apoptosis, potentially promoting carcinogenesis.

  18. Primary renal carcinoid tumor: A radiologic review

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    Leslie Lamb, MD, Msc, Bsc

    2014-01-01

    Full Text Available Carcinoid tumor is the classic famous anonym of neuroendocrine neoplasms. Primary renal carcinoid tumors are extremely rare, first described by Resnick and colleagues in 1966, with fewer than a total of 100 cases reported in the literature. Thus, given the paucity of cases, the clinical and histological behavior is not well understood, impairing the ability to predict prognosis. Computed tomography and (occasionally octreotide studies are used in the diagnosis and followup of these rare entites. A review of 85 cases in the literature shows that no distinctive imaging features differentiate them from other primary renal masses. The lesions tend to demonstrate a hypodense appearance and do not usually enhance in the arterial phases, but can occasionally calcify. Octreotide scans do not seem to help in the diagnosis; however, they are more commonly used in the postoperative followup. In addition, we report a new case of primary renal carcinoid in a horseshoe kidney.

  19. Carotid, aorta and renal arteries intima-media thickness in patients with sporadic idiopathic hypoparathyroidism

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    Deshraj Meena

    2015-01-01

    Full Text Available Background: Alteration in homeostasis of calcium, phosphate and parathyroid hormone (PTH predispose to vascular calcification that increases the risk of cardiovascular morbidity and mortality. The data on this aspect are scarce in patients with sporadic idiopathic hypoparathyroidism (SIH. Objective: The aim was to assess the effect of altered calcium, phosphate and PTH homeostasis in patients with SIH on intima media thickness (IMT, a surrogate marker of increased vascular risk. Methods: In this case-control study, we measured carotid IMT (CIMT, aortic IMT (AIMT and renal arteries IMT (RIMT in 30 consecutive patients with SIH, and compared with healthy subjects. IMT was measured by ultrasound by a single operator blinded to subject′s details. Results: CIMT, AIMT, RIMT values in patients with SIH were significantly more than healthy subjects (0.60 ± 0.08 mm vs. 0.52 ± 0.09 mm, P = 0.001; 0.73 ± 0.09 mm vs. 0.65 ± 0.10, P = 0.004; and 0.34 ± 0.04 mm vs. 0.30 ± 0.05, P = 0.003, respectively. Clinical or biochemical parameters did not correlate with CIMT, AIMT and RIMT in patients with SIH. Conclusion: The vascular risk is increased in patients with SIH as assessed by CIMT, AIMT, and RIMT.

  20. Acute renal insufficiency after radiofrequency of renal tumor

    OpenAIRE

    Barreto,Francualdo; Dall'Oglio,Marcos F.; Srougi,Miguel

    2007-01-01

    Recent advances in techniques of imaging and ablation have led to the application of several minimally invasive modalities, such as radiofrequency ablation (RFA) with a success rate varying from 79 to 96% and a serious complication rate of 1 to 4% in the treatment of small renal tumors. The authors report on the case of a 67-year-old patient with a radiofrequency ablation complication, stenosis of the ureteropelvic junction in one kidney, and analyze the results of this modality for the treat...

  1. Molecular characteristics of mismatch repair genes in sporadic colorectal tumors in Czech patients.

    Science.gov (United States)

    Vymetalkova, Veronika Polakova; Slyskova, Jana; Korenkova, Vlasta; Bielik, Ludovit; Langerova, Lucie; Prochazka, Pavel; Rejhova, Alexandra; Schwarzova, Lucie; Pardini, Barbara; Naccarati, Alessio; Vodicka, Pavel

    2014-01-31

    Mismatch repair (MMR) genes are known to be frequently altered in colorectal cancer (CRC). Both genetics and epigenetics modifications seems to be relevant in this phenomenon, however it is still not clear how these two aspects are interconnected. The present study aimed at characterizing of epigenetic and gene expression profiles of MMR genes in sporadic CRC patients from the Czech Republic, a country with one of the highest incidences of this cancer all over Europe. Expression levels and CpG promoter methylation status of all MMR genes were evaluated in DNA from tumor and adjacent mucosal samples of 53 incident CRC patients. We have found significantly increased transcription levels in EXO1 gene in tumor tissues (P = 0.05) and significant over-expression of MSH3 gene in colon tumors when compared to adjacent mucosal tissues (P = 0.02). Interestingly, almost all MMR genes were differently expressed when localization of tumors was compared. In particular, colon tumors showed an up-regulation of EXO1, MSH2, MSH3, MSH6, and PMS2 genes in comparison to rectal tumors (P = 0.02). Expression levels of all MMR genes positively correlated between each other. The promoter methylation of MLH1 gene was observed in 9% of CRC tissues only. In our study, we have observed different pattern of MMR genes expression according to tumor localization. However, a lack of association between methylation in MMR genes and their corresponding expressions was noticed in this study, the relationship between these two aspects is worthy to be analyzed in larger population studies and in pre-malignant stages.

  2. Are there tumor suppressor genes on chromosome 4p in sporadic colorectal carcinoma?

    Science.gov (United States)

    Zheng, Hai-Tao; Jiang, Li-Xin; Lv, Zhong-Chuan; Li, Da-Peng; Zhou, Chong-Zhi; Gao, Jian-Jun; He, Lin; Peng, Zhi-Hai

    2008-01-07

    To study the candidate tumor suppressor genes (TSG) on chromosome 4p by detecting the high frequency of loss of heterozygosity (LOH) in sporadic colorectal carcinoma in Chinese patients. Seven fluorescent labeled polymorphic microsatellite markers were analyzed in 83 cases of colorectal carcinoma and matched normal tissue DNA by PCR. PCR products were electrophoresed on an ABI 377 DNA sequencer. Genescan 3.7 and Genotype 3.7 software were used for LOH scanning and analysis. The same procedure was performed by the other six microsatellite markers spanning D4S3013 locus to make further detailed deletion mapping. Comparison between LOH frequency and clinicopathological factors was performed by c2 test. Data were collected from all informative loci. The average LOH frequency on 4p was 24.25%, and 42.3% and 35.62% on D4S405 and D4S3013 locus, respectively. Adjacent markers of D4S3013 displayed a low LOH frequency (4p15.2) and D4S405 (4p14) locus are detected. Candidate TSG, which is involved in carcinogenesis and progression of sporadic colorectal carcinoma on chromosome 4p, may be located between D4S3017 and D4S2933 (about 1.7 cm).

  3. The clinical factors associated with benign renal tumors

    International Nuclear Information System (INIS)

    Yamashita, Ryo; Nakamura, Masafumi; Matsuzaki, Masato; Matsui, Takashi; Yamaguchi, Raizo; Niwakawa, Masashi; Tobisu, Kenichi; Asakura, Koiku; Ito, Ichiro

    2009-01-01

    In this study, we sought to define the incidence of benign renal tumors in our institute and to clarify the clinical factors associated with benign renal tumors, in order to assist in forming preoperative differential diagnoses. From October 2002 to July 2007, we performed 157 nephrectomies in patients preoperatively diagnosed with renal cell carcinoma. We chose 81 tumors, all of which were less than 5 cm, for further study. We reviewed double-phase helical CT imaging retrospectively, specifically focusing on attenuation patterns and homogeneity. We also compared clinical factors, including age, sex and tumor size, between the benign and malignant renal tumors. The patient's median age was 67 years (mean age, 63 years), and the median tumor diameter was 3.0 cm (mean, 3.2 cm). Benign renal tumors were found in 10 (12%) of the 81 tumors; these included seven cases of oncocytoma and three cases of angiomyolipoma with minimal fat. Several factors were significant clinical determinants of differentiation between benign and malignant renal tumors: homogeneity in CT, female gender, and small tumor size all predominated in cases of benign tumors. Attenuation pattern in CT, however, was not a significant factor (p=0.344). When a patient, especially a female, presents with a small and homogeneous renal tumor, careful consideration should be given to the possibility of a benign process, which needs further consideration before performing excessive surgery. (author)

  4. Diagnostic Efficacy of Percutaneous Renal Tumor Biopsy - Concomitant Use of Frozen Section to Accurately Diagnose Renal Tumor with Necrosis.

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    Hori, Junichi; Kobayashi, Shin; Tamaki, Gaku; Azumi, Makoto; Kakizaki, Hidehiro

    2017-09-01

    To evaluate the diagnostic efficacy of percutaneous renal tumor biopsy. We retrospectively investigated 23 patients who underwent percutaneous renal tumor biopsy since 2008 at Department of Renal and Urologic Surgery, Asahikawa Medical University Hospital. We examined indications of biopsy, diagnostic concordance rate between urologists, radiologists and biopsy findings, pathological findings and biopsy-related complications. Renal tumor biopsy was performed under ultrasonography guidance in 21 patients and computed tomography guidance in 2. The most frequent indication to perform biopsy was to determine histological subtype of renal cell carcinoma(RCC)before treatment. The second indication was to clarify the nature of renal tumor. Biopsy findings revealed RCC in 17 patients and urothelial carcino- ma(UC)in 6. Diagnostic concordance rate between urologist's diagnosis and biopsy findings was 91%(21/23), which showed the same result between radiologists and biopsy findings. Biopsy-related adverse event included needle tract implantation in 1 patient with UC. Another patient who had central necrosis in the tumor showed insufficient material causing repeat biopsy with frozen section. Except this patient, initial renal tumor biopsy was successful in all patients by concomitant use of frozen section for tumor with central necrosis. The overall diagnostic rate of initial biopsy was 95.7%(22/23). Despite a small number of patients and a retrospective nature, the present study shows that renal tumor biopsy plays an important role in diagnosis of renal tumor. Concomitant use of frozen section might be considered at the time of renal tumor biopsy in patients with necrotic renal tumor to avoid repeat biopsy. However, we should take into consideration that there are some possible risks of needle tract implantation in cases with UC when we perform percutaneous renal tumor biopsy.

  5. Primary renal carcinoid tumor mimicking non-clear cell renal cell carcinoma: A case report

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    Joo, Lee Hi; Kim, See Hyung; Kim, Mi Jeong; Choe, Mi Sun [Keimyung University School of Medicine, Dongsan Medical Center, Daegu (Korea, Republic of)

    2016-07-15

    Carcinoid tumors are neoplasms with neuroendocrine differentiation, and they are most commonly found in the gastrointestinal and respiratory systems. Primary renal carcinoid tumor has rarely been reported. Here, we present a case of primary renal carcinoid tumor manifesting as a small but a gradually enhancing mass with calcification and a cystic component.

  6. Tumoral retroperitoneal masses and their impact to renal function

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    Azis Olgun

    2016-02-01

    Full Text Available Retroperitoneal tumors, either benign or malignant, usually cause problems both for an accurate diagnosis and for therapeutic approach, due to their reduce incidence, late presentation and anatomical location, in the neighborhood of vital structures from the retroperitoneal space. Materials and methods: Aim of study was to search correlations between, gender, age, and histo-pathological type of retroperitoneal tumors, as well as their impact upon renal function. Results: Sarcomas represented about 30% of retroperitoneal tumors. The most frequent benign retroperitoneal tumors included: neurogenic tumors, paragangliomas, renal angiomyolipomas and benign retroperitoneal lipomas. Renal function was altered in about 55% of the patients, and postsurgical mortality was 8.73%.

  7. Genetic and epigenetic silencing of the beclin 1 gene in sporadic breast tumors

    Directory of Open Access Journals (Sweden)

    Wu Yiqing

    2010-03-01

    Full Text Available Abstract Background Beclin 1, an important autophagy-related protein in human cells, is involved in cell death and cell survival. Beclin 1 mapped to human chromosome 17q21. It is widely expressed in normal mammary epithelial cells. Although down-regulated expression with mono-allelic deletions of beclin 1 gene was frequently observed in breast tumors, whether there was other regulatory mechanism of beclin 1 was to be investigated. We studied the expression of beclin 1 and explored the possible regulatory mechanisms on its expression in breast tumors. Methods 20 pairs of tumors and adjacent normal tissues from patients with sporadic breast invasive ductal cancer (IDCs were collected. The mRNA expression of beclin 1 was detected by real-time quantitative RT-PCR. Loss of heterozygosity (LOH was determined by real-time quantitative PCR and microsatellite methods. The protein expression of beclin 1, p53, BRCA1 and BRCA2 was assessed by immunohistochemistry. CpG islands in 5' genomic region of beclin 1 gene were identified using MethylPrimer Program. Sodium bisulfite sequencing was used in examining the methylation status of each CpG island. Results Decreased beclin 1 mRNA expression was detected in 70% of the breast tumors, and the protein levels were co-related to the mRNA levels. Expression of beclin 1 mRNA was demonstrated to be much higher in the BRCA1 positive tumors than that in the BRCA1 negative ones. Loss of heterozygosity was detected in more than 45% of the breast tumors, and a dense cluster of CpG islands was found from the 5' end to the intron 2 of the beclin 1 gene. Methylation analysis showed that the promoter and the intron 2 of beclin 1 were aberrantly methylated in the tumors with decreased expression. Conclusions These data indicated that LOH and aberrant DNA methylation might be the possible reasons of the decreased expression of beclin 1 in the breast tumors. The findings here shed some new light on the regulatory mechanisms of

  8. Genetic and epigenetic silencing of the beclin 1 gene in sporadic breast tumors

    International Nuclear Information System (INIS)

    Li, Zidong; Chen, Bo; Wu, Yiqing; Jin, Feng; Xia, Yongjing; Liu, Xiangjun

    2010-01-01

    Beclin 1, an important autophagy-related protein in human cells, is involved in cell death and cell survival. Beclin 1 mapped to human chromosome 17q21. It is widely expressed in normal mammary epithelial cells. Although down-regulated expression with mono-allelic deletions of beclin 1 gene was frequently observed in breast tumors, whether there was other regulatory mechanism of beclin 1 was to be investigated. We studied the expression of beclin 1 and explored the possible regulatory mechanisms on its expression in breast tumors. 20 pairs of tumors and adjacent normal tissues from patients with sporadic breast invasive ductal cancer (IDCs) were collected. The mRNA expression of beclin 1 was detected by real-time quantitative RT-PCR. Loss of heterozygosity (LOH) was determined by real-time quantitative PCR and microsatellite methods. The protein expression of beclin 1, p53, BRCA1 and BRCA2 was assessed by immunohistochemistry. CpG islands in 5' genomic region of beclin 1 gene were identified using MethylPrimer Program. Sodium bisulfite sequencing was used in examining the methylation status of each CpG island. Decreased beclin 1 mRNA expression was detected in 70% of the breast tumors, and the protein levels were co-related to the mRNA levels. Expression of beclin 1 mRNA was demonstrated to be much higher in the BRCA1 positive tumors than that in the BRCA1 negative ones. Loss of heterozygosity was detected in more than 45% of the breast tumors, and a dense cluster of CpG islands was found from the 5' end to the intron 2 of the beclin 1 gene. Methylation analysis showed that the promoter and the intron 2 of beclin 1 were aberrantly methylated in the tumors with decreased expression. These data indicated that LOH and aberrant DNA methylation might be the possible reasons of the decreased expression of beclin 1 in the breast tumors. The findings here shed some new light on the regulatory mechanisms of beclin 1 in breast cancer

  9. Predictors of future growth of sporadic vestibular schwannomas obtained by history and radiologic assessment of the tumor.

    NARCIS (Netherlands)

    Artz, J.C.; Timmer, F.C.A.; Mulder, J.J.S.; Cremers, C.W.R.J.; Graamans, K.

    2009-01-01

    Management of a sporadic vestibular schwannoma (VS) is still a subject of controversy, mainly due to distinct and unpredictable growth patterns. To embark on an appropriate therapy it is necessary to dispose of a reliable prediction about tumor progression. This study aims to design a risk profile

  10. Deterministic Evolutionary Trajectories Influence Primary Tumor Growth: TRACERx Renal

    DEFF Research Database (Denmark)

    Turajlic, Samra; Xu, Hang; Litchfield, Kevin

    2018-01-01

    The evolutionary features of clear-cell renal cell carcinoma (ccRCC) have not been systematically studied to date. We analyzed 1,206 primary tumor regions from 101 patients recruited into the multi-center prospective study, TRACERx Renal. We observe up to 30 driver events per tumor and show that ...

  11. Expression of parafibromin in major renal cell tumors

    Directory of Open Access Journals (Sweden)

    C. Cui

    2012-10-01

    Full Text Available Parafibromin, encoded by HRPT2 gene, is a recently identified tumor suppressor. Complete and partial loss of its expression have been observed in hyperparathyroidism-jaw tumor (HPT-JT, parathyroid carcinoma, breast carcinoma, lung carcinoma, gastric and colorectal carcinoma. However, little has been known about its expression in renal tumors. In order to study the expression of parafibromin in a series of the 4 major renal cell tumors - clear cell renal cell carcinoma (ccRCC, papillary renal cell carcinoma (pRCC, chromophobe renal cell carcinoma (chRCC and oncocytoma. One hundred thirty nine renal tumors including 61 ccRCCs, 37 pRCCs, 22 chRCCs and 19 oncocytomas were retrieved and used for the construction of renal tissue microarrays (TMAs. The expression of parafibromin was detected by immunohistochemical method on the constructed TMAs. Positive parafibromin stains are seen in 4 out of 61 ccRCCs (7%, 7 out of 37 pRCCs (19%, 12 out of 23 chRCCs (52% and all 19 oncocytomas (100%. Parafibromin expression varies significantly (P< 8.8 x10-16 among the four major renal cell tumors and were correlated closely with tumor types. No correlation of parafibromin expression with tumor staging in ccRCCs, pRCCs and chRCCs, and Fuhrman nuclear grading in ccRCCs and pRCCs. In summary, parafibromin expression was strongly correlated with tumor types, which may suggest that it plays a role in the tumorigenesis in renal cell tumors.

  12. Computerized tomography for diagnosis and staging of renal pelvic tumor

    International Nuclear Information System (INIS)

    Fukuoka, Hiroshi; Goto, Akihiko; Kitamura, Hajime

    1985-01-01

    Although we have no definite criteria available yet for clinical staging of renal pelvic tumor, the preoperative staging of this disease is nevertheless important in view of the current tendency that the necessity for renal conservative operation is considered. CT is now a routine work also for diagnosing renal pelvic tumor. The present study was performed in order to validate its usefulness for diagnosing and staging the disease. Our series consisted of 8 patients with renal pelvic tumor, in 6 of whom a definite diagnosis was established after demonstrating filling defect on pyelogram, but in the remaining two with extensive infiltration, and squamous cell carcinoma associated with staghorn calculus respectively, CT failed to provide a definite diagnosis. CT findings of an extension of the mass in the renal pelvis or renal calyces into adipose tissue of the renal sinus or renal parenchyma were judged to indicate an invasive type (Stage III), while a non-invasive type (Stage I or II) was defined on the basis of otherwise CT findings. Consistency with pathological stages was noted in 7 of the 8 cases (87.5 %). It was difficult to differentiate Stage I and Stage II on CT findings. CT was considered to be extremely useful tool for preoperative staging of renal pelvic tumor. (author)

  13. A rare case of retroperitoneal malignant triton tumor invading renal vein and small intestine

    Directory of Open Access Journals (Sweden)

    Mijović Žaklina

    2013-01-01

    Full Text Available Introduction. Malignant Triton tumor is a very rare malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation. Most of those tumors occur in patients with von Recklinghausen’s disease or as a late complication of irradiation and commonly seen in the head, neck, extremities and trunk. Case report. We reported retroperitoneal malignant Triton tumor in a 57-year-old female patient. Skin lesions were not present, and there was no family history of neurofibromatosis or previous irradiation. The presented case is one of a few recorded in the specialized literature that occurs in the retroperitoneal space in sporadic form. In this case, tumor consisted of a multilobular mass was in close relation with the abdominal aorta and inferior vena cava and involved the renal vein with gross invasion of the small intestine. The patient underwent total resection of the tumor and left nefrectomy was performed. The small intestine 10 cm in length was also resected and end-to-end anastomosis was conducted. The postoperative course was uneventful and the patient was discharged from the hospital ten days after the surgery. Conclusion. Diagnostically, it is crucial to recognize this uncommon histological variant because malignant Triton tumor has a worse prognosis than classic malignant peripheral nerve sheath tumor does. The use of the immunohistochemistry is essential in making the correct diagnosis. Only appropriate pathological evaluation supported by immunostaining with S-100 protein and desmin confirmed the diagnosis. Aggressive surgical management treatment improves the prognosis of such cases with adjuvant radiotherapy.

  14. Unusual Renal Tumors — Report of Four Cases

    African Journals Online (AJOL)

    band and bone marrow ... view showing many collecting ducts lined by atypical malignant epithelial lining cells embedded in a desmoplastic stroma. .... ogy and molecular genetics of renal tumors: toward unifica- tion of a classification system.

  15. Gonadal vein tumor thrombosis due to renal cell carcinoma

    Directory of Open Access Journals (Sweden)

    Hamidreza Haghighatkhah

    2015-01-01

    Full Text Available Renal cell carcinoma (RCC had a tendency to extend into the renal vein and inferior vena cava, while extension into the gonadal vein has been rarely reported. Gonadal vein tumor thrombosis appears as an enhancing filling defect within the dilated gonadal vein anterior to the psoas muscle and shows an enhancement pattern identical to that of the original tumor. The possibility of gonadal vein thrombosis should be kept in mind when looking at an imaging study of patients with RCC

  16. A molecular analysis by gene expression profiling reveals Bik/NBK overexpression in sporadic breast tumor samples of Mexican females

    International Nuclear Information System (INIS)

    García, Normand; Salamanca, Fabio; Astudillo-de la Vega, Horacio; Curiel-Quesada, Everardo; Alvarado, Isabel; Peñaloza, Rosenda; Arenas, Diego

    2005-01-01

    Breast cancer is one of the most frequent causes of death in Mexican women over 35 years of age. At molecular level, changes in many genetic networks have been reported as associated with this neoplasia. To analyze these changes, we determined gene expression profiles of tumors from Mexican women with breast cancer at different stages and compared these with those of normal breast tissue samples. 32 P-radiolabeled cDNA was synthesized by reverse transcription of mRNA from fresh sporadic breast tumor biopsies, as well as normal breast tissue. cDNA probes were hybridized to microarrays and expression levels registered using a phosphorimager. Expression levels of some genes were validated by real time RT-PCR and immunohistochemical assays. We identified two subgroups of tumors according to their expression profiles, probably related with cancer progression. Ten genes, unexpressed in normal tissue, were turned on in some tumors. We found consistent high expression of Bik gene in 14/15 tumors with predominant cytoplasmic distribution. Recently, the product of the Bik gene has been associated with tumoral reversion in different neoplasic cell lines, and was proposed as therapy to induce apoptosis in cancers, including breast tumors. Even though a relationship among genes, for example those from a particular pathway, can be observed through microarrays, this relationship might not be sufficient to assign a definitive role to Bik in development and progression of the neoplasia. The findings herein reported deserve further investigation

  17. Concomitant tumor and autoantigen vaccination supports renal cell carcinoma rejection.

    Science.gov (United States)

    Herbert, Nicolás; Haferkamp, Axel; Schmitz-Winnenthal, Hubertus F; Zöller, Margot

    2010-07-15

    Efficient tumor vaccination frequently requires adjuvant. Concomitant induction of an autoimmune response is discussed as a means to strengthen a weak tumor Ag-specific response. We asked whether the efficacy of dendritic cell (DC) vaccination with the renal cell carcinoma Ags MAGE-A9 (MAGE9) and G250 could be strengthened by covaccination with the renal cell carcinoma autoantigen GOLGA4. BALB/c mice were vaccinated with DC loaded with MHC class I-binding peptides of MAGE9 or G250 or tumor lysate, which sufficed for rejection of low-dose RENCA-MAGE9 and RENCA-G250 tumor grafts, but only retarded tumor growth at 200 times the tumor dose at which 100% of animals will develop a tumor. Instead, 75-100% of mice prevaccinated concomitantly with Salmonella typhimurium transformed with GOLGA4 cDNA in a eukaryotic expression vector rejected 200 times the tumor dose at which 100% of animals will develop tumor. In a therapeutic setting, the survival rate increased from 20-40% by covaccination with S. typhimurium-GOLGA4. Autoantigen covaccination significantly strengthened tumor Ag-specific CD4(+) and CD8(+) T cell expansion, particularly in peptide-loaded DC-vaccinated mice. Covaccination was accompanied by an increase in inflammatory cytokines, boosted IL-12 and IFN-gamma expression, and promoted a high tumor Ag-specific CTL response. Concomitant autoantigen vaccination also supported CCR6, CXCR3, and CXCR4 upregulation and T cell recruitment into the tumor. It did not affect regulatory T cells, but slightly increased myeloid-derived suppressor cells. Thus, tumor cell eradication was efficiently strengthened by concomitant induction of an immune response against a tumor Ag and an autoantigen expressed by the tumor cell. Activation of autoantigen-specific Th cells strongly supports tumor-specific Th cells and thereby CTL activation.

  18. Lithium and renal and upper urinary tract tumors

    DEFF Research Database (Denmark)

    Kessing, Lars Vedel; Gerds, Thomas Alexander; Feldt-Rasmussen, Bo

    2015-01-01

    OBJECTIVES: A recent alarming finding suggested an increased risk of renal tumors among long-term lithium users. The objectives of the present study were to estimate rates of renal and upper urinary tract tumors (RUT), malignant and benign, among individuals exposed to successive prescriptions......) benign. Analyses were adjusted for the number of prescriptions for lithium/anticonvulsants, antipsychotic agents, antidepressants, and use of all other types of medication; age; gender; employment status; calendar year; and a diagnosis of bipolar disorder. RESULTS: Continued treatment with lithium...

  19. Rapidly enlarging renal tumor during pregnancy: diagnostic and management dilemma

    Directory of Open Access Journals (Sweden)

    Kor Wei Tiang

    2014-04-01

    Full Text Available Urological tumors diagnosed during pregnancy are rare. However, the incidence seems to be increasing largely due to advancements in modern imaging techniques and improved antenatal care. The diagnosis and management of renal tumors during pregnancy poses a dilemma to clinicians. This case report highlights the challenges in managing a large chromophobe renal cell carcinoma in a young primigravida patient. Proper antenatal assessment, a multidisciplinary team approach and appropriate discussion with patient are important determinants to achieve the best clinical outcomes for both the mother and the baby. 

  20. Radiological features of progressive tumoral calcinosis in chronic renal failure.

    LENUS (Irish Health Repository)

    Hodnett, P

    2012-02-03

    We present the case of a young adult patient with chronic renal failure who developed painful subcutaneous nodules after failed renal transplant and recommencing dialysis. These nodules were juxta-articular in location and initially located over both shoulders. Radiological evaluation suggested tumoral calcinosis. The patient was placed on a strict dialysis and dietary regimen but was suboptimally compliant with same. The patient developed progressive disease with an increase in size and number of juxta-articular calcified soft-tissue masses. However, 6 months following a second renal transplant clinical and radiological follow up demonstrated marked resolution both in symptomatology and radiographic findings. We present the plain radiographic, CT and MRI findings which demonstrate the typical radiological features of tumoral calcinosis. We correlate these findings with clinical course and histological findings following surgical excision of one of these masses.

  1. Potentialities of scanning in the diagnosis of renal tumors

    International Nuclear Information System (INIS)

    Lysenko, V.F.; Tabakman, Yu.Yu.; Ar'e, V.M.

    1980-01-01

    Renal scanning was performed in 1600 patients of which 259 had solitary focal renal lesions. While using the standard method of scanning 12.3 % of patients had the ''non-functioning'' kidney. Differentiated adjustment of the scanner for each kidney made it possible to obtain high-quality scans in 55.3 % of the patients with the ''non-functioning'' kidney. Of these, focal renal lesion was found in 38.4% Renal scanning was performed in 55 patients using the posterior, lateral an anterior projections. During scanning in the posterior projection renal lesions were detected in 72.3 % of patients. The results were negative in 3.6 % and doubtful in 24.7 %. The use of the 3 projection resulted in the enhanced accuracy of tumor detection up to 94.5 %, doubtful results were obtainded in 5.5 % of patients. Proposed is a scheme of 7 types of focal renal lesions and discussed are problems arizing in differential diagnosis of some renal diseases such as nephroptosis, spheroidal and polycystic kidney

  2. Renal Cell Carcinoma Mimicking Adrenal Tumor

    Directory of Open Access Journals (Sweden)

    Mohammad Kazem Moslemi

    2010-01-01

    Full Text Available There are a variety of causes of adrenal pseudotumors on computerized tomography (CT scan, including upper-pole renal mass, gastric diverticulum, prominent splenic lobulation, pancreatic mass, hepatic mass, and periadrenal varices. We present a case of a large subhepatic mass that discrimination of its origin from neighborhood organs was difficult preoperatively. Our patient was a 58 years old man, that three months after an unsuccessful operation in another center for a pseudoadrenal mass underwent a very difficult subcapsular tumorectomy in our center.

  3. Absence of activating mutations in ras and gsp oncogenes in a cohort of nine patients with sporadic pediatric thyroid tumors.

    Science.gov (United States)

    Pauws, E; Tummers, R F; Ris-Stalpers, C; de Vijlder, J J; Voûte, T

    2001-06-01

    Characterization of the genetic background of pediatric thyroid carcinomas could aid in distinguishing between differently staged tumors with respect to treatment and prognosis. Two known genetic factors associated with thyroid carcinoma, the proto-oncogenes gsp and ras were investigated. DNA was extracted from paraffin sections from both tumor and normal thyroid tissue of nine patients (ages 9-16 years). Of these patients, eight were diagnosed with papillary carcinoma and one with follicular adenoma. The coding exons of gsp and the three known ras genes (H, K, and N-ras) were screened for mutations using SSCP-analysis. There were no mutations present in the ras and gsp proto-oncogenes hot spots, however, LOH of H-ras (chromosome location 11p15.5) was found in tumor tissue from one patient and a homozygous mutation in exon 12 of gsp causing a Pro-->Ser conversion was present in the thyroid tumor tissue from another patient. Two silent polymorphisms were detected, H-ras exon1, 86T-->C and gsp exon 5, 81T-->C. Our results indicate that the ras/gsp mutations found are probably late events in the tumorigenesis representing general oncogenic stress. In conclusion, it seems that ras/gsp activation is not a factor in the mechanism causing sporadic thyroid carcinoma in children. Copyright 2001 Wiley-Liss, Inc.

  4. SARC006: Phase II Trial of Chemotherapy in Sporadic and Neurofibromatosis Type 1 Associated Chemotherapy-Naive Malignant Peripheral Nerve Sheath Tumors

    Directory of Open Access Journals (Sweden)

    Christine S. Higham

    2017-01-01

    Full Text Available Background. Worse chemotherapy response for neurofibromatosis type 1- (NF1- associated compared to sporadic malignant peripheral nerve sheath tumors (MPNST has been reported. Methods. We evaluated the objective response (OR rate of patients with AJCC Stage III/IV chemotherapy-naive NF1 MPNST versus sporadic MPNST after 4 cycles of neoadjuvant chemotherapy, 2 cycles of ifosfamide/doxorubicin, and 2 cycles of ifosfamide/etoposide. A Simon optimal two-stage design was used (target response rate 40%. Results. 34 NF1 (median age 33 years and 14 sporadic (median age 40 years MPNST patients enrolled. Five of 28 (17.9% evaluable NF1 MPNST patients had a partial response (PR, as did 4 of 9 (44.4% patients with sporadic MPNST. Stable disease (SD was achieved in 22 NF1 and 4 sporadic MPNST patients. In both strata, results in the initial stages met criteria for expansion of enrollment. Only 1 additional PR was observed in the expanded NF1 stratum. Enrollment was slower than expected and the trial closed before full accrual. Conclusions. This trial was not powered to detect differences in response rates between NF1 and sporadic MPNST. While the OR rate was lower in NF1 compared to sporadic MPNST, qualitative responses were similar, and disease stabilization was achieved in most patients.

  5. A rare renal pelvis tumor: Mucinous cystadenocarcinoma

    Directory of Open Access Journals (Sweden)

    Gül Türkcü

    2015-03-01

    Full Text Available Urothelial carcinomas are the most common neoplasms in the renal pelvis. However mucinous cystadenocarcinomas (MCA are very rare in this localization. Although some theories are attributed on the patogenesis of MCA, its exact etiology is not known. Herein, we present histopathological characteristic of a case with MCA. Multiple cystic lesions and millimetric calculi with ectasia of the left kidney were detected by abdominal ultrasound and magnetic resonance imaging. Left simple nephrectomy was performed because of a pre-diagnosis of atrophic pyonephrotic kidney. The sections of the nephrectomized kidney revealed, multilocular mucinous cysts and histopathological appearance of MCA. We aimed to present this rare case mimicking atrophic cystic kidney with clinical, radiological findings, and histopathological characteristics in the lights of literature.J Clin Exp Invest 2015; 6(1: 78-80

  6. Renal Tumors: Technical Success and Early Clinical Experience with Radiofrequency Ablation of 18 Tumors

    International Nuclear Information System (INIS)

    Sabharwal, Rohan; Vladica, Philip

    2006-01-01

    Purpose. To evaluate the feasibility, safety, and technical efficacy of image-guided radiofrequency ablation (RFA) for the treatment of small peripheral renal tumors and to report our early results with this treatment modality. Methods. Twenty-two RFA sessions for 18 tumors were performed in 11 patients with renal tumors. Indications included coexistent morbidity, high surgical or anesthetic risk, solitary kidney, and hereditary predisposition to renal cell carcinoma. Ten patients had CT-guided percutaneous RFA performed on an outpatient basis. One patient had open intraoperative ultrasound-guided RFA. Technical success was defined as elimination of areas that enhanced at imaging within the entire tumor. With the exception of one patient with renal insufficiency who required gadolinium-enhanced MRI, the remaining patients underwent contrast-enhanced CT for post-treatment follow-up assessment. Follow-up was performed after 2-4 weeks and then at 3, 6, 12 months, and every 12 months thereafter. Results. Fourteen (78%) of 18 tumors were successfully ablated with one session. Three of the remaining four tumors required two sessions for successful ablation. One tumor will require a third session for areas of persistent enhancement. Mean patient age was 72.82 ± 10.43 years. Mean tumor size was 1.95 ± 0.79 cm. Mean follow-up time was 10.91 months. All procedures were performed without any major complications. Conclusions. Our early experience with percutaneous image-guided radiofrequency ablation demonstrates it to be a feasible, safe, noninvasive, and effective treatment of small peripheral renal tumors

  7. Laparoscopic Cryoablation Of Small Renal Tumors – Does Anatomical Tumor Complexity Affect Treatment Outcome?

    DEFF Research Database (Denmark)

    Nielsen, Tommy Kjærgaard; Østraat, Øyvind; Andersen, Gratien

    Background: Renal cryoablation is a valid treatment option for localized pT1a renal tumors and has been the modality of choice at Aarhus University Hospital since 2005. Anatomical tumor classification systems such as PADUA and RENAL scores were initially introduced as tools to evaluate complication...... August 2005 and December 2013. Patients were followed-up postoperatively with contrast-CT for up to 5 years or until a residual unablated tumor was diagnosed. Results: Mean patient age: 63 years (95%CI 61;65). Mean BMI: 27 kg/m2 (95%CI 26;28). Mean ASA-score: 2.1 (95%CI 2;2.2). Mean ECOC performance...

  8. Clumped perinuclear BAP1 expression is a frequent finding in sporadic epithelioid Spitz tumors.

    Science.gov (United States)

    Gammon, Bryan; Traczyk, Tom N; Gerami, Pedram

    2013-06-01

    BRCA1-associated protein 1 (BAP1) represents a recently identified tumor suppressor protein. Loss of BAP1 has been observed in cutaneous epithelioid Spitz tumors. These cutaneous melanocytic tumors show a distinct histopathologic phenotype characterized by an intradermal sheet-like proliferation of epithelioid melanocytes. We retrospectively reviewed the clinical outcomes, histopathologic findings and immunophenotype in spitzoid melanocytic neoplasms with the morphologic features seen in BAP1 mutated Spitz tumors. Cases were obtained from our files. BAP1 immunohistochemistry was evaluated dichotomously for the presence of nuclear staining. Fifteen of 19 cases showed loss of nuclear BAP1 expression. Of the 15 cases displaying nuclear loss of BAP1, clumped perinuclear staining was observed in 8 cases while 7 cases showed complete loss. Follow up ranging from 0-45 months (mean 17 months) was uneventful. Our data are consistent with an indolent overall clinical course for epithelioid Spitz tumors with loss of BAP1. Furthermore, a large subset of epithelioid Spitz tumors display loss of nuclear expression but show a reproducible clumped perinuclear staining pattern. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  9. Fanconi Anemia Gene Mutations Are Not Involved in Sporadic Wilms Tumor

    NARCIS (Netherlands)

    Adank, Muriel A.; Segers, Heidi; van Mil, Saskia E.; van Helsdingen, Yvette M.; Ameziane, Najim; van den Ouweland, Ans M. W.; Wagner, Anja; Meijers-Heijboer, Hanne; Kool, Marcel; de Kraker, Jan; Waisfisz, Quinten; van den Heuvel-Eibrink, Marry M.

    2010-01-01

    Bi-allelic germline mutations of the Fancom anemia (FA) genes, PALB2/FANCN and BRCA2/FANCD1, have been reported in a few Wilms tumor (WT) patients with an atypical FA phenotype Therefore, we screened a random cohort of 47 Dutch WT cases for germline mutations in these two FA-genes by DNA sequencing

  10. Differential expression of hMLH1 in sporadic human colorectal cancer tumors and distant metastases

    DEFF Research Database (Denmark)

    Larsen, Nicolai Balle; Heiberg Engel, Peter Johan; Rasmussen, Merete

    2009-01-01

    hMLH1/hPMS2 negative at the transition zone, but with distinct subclones of hMLH1/hPMS2-expressing cells at the transition zone. All of these tumors expressed hMLH1/hPMS2 at the invasive front and in liver metastases, with three also expressing hMLH/hPMS2 in lymph node metastases. No significant...

  11. Immunohistochemical characterization of renal tumors in patients with Birt-Hogg-Dubé syndrome.

    Science.gov (United States)

    Iribe, Yasuhiro; Kuroda, Naoto; Nagashima, Yoji; Yao, Masahiro; Tanaka, Reiko; Gotoda, Hiroko; Kawakami, Fumi; Imamura, Yoshiaki; Nakamura, Yasushi; Ando, Midori; Araki, Akinobu; Matsushima, Jun; Nakatani, Yukio; Furuya, Mitsuko

    2015-03-01

    Birt-Hogg-Dubé syndrome (BHD) is an autosomal dominant disorder associated with a germline mutation of folliculin (FLCN). The affected families are at a high risk for developing multiple renal cell carcinomas (RCC). Little is known about the immunostaining patterns of mutant FLCN-associated RCCs. We investigated 32 RCCs obtained from 17 BHD patients. The studied tumors included chromophobe RCCs (n = 15), hybrid oncocytic/chromophobe tumors (HOCT) (n = 14) and clear cell RCCs (n = 3). Almost all chromophobe RCCs and HOCTs revealed positive staining for S100A1, Ksp-cadherin and CD82. They stained either focally or diffusely for CK7, and were negative for CA-IX. All clear cell RCCs were positively stained for CA-IX and negative for CK7. These data confirmed that mutant FLCN-associated oncocytic and clear cell RCCs exhibited generally similar immunostaining patterns compared to their sporadic counterparts. Frequent positive staining for S100A1, Ksp-cadherin and CD82 in chromophobe RCCs and HOCTs indicated that these two types were relatively similar rather than distinctively different in their patterns of immunoreactivity. Characteristic peri-nuclear halos and polygonal cells with clear cytoplasm, which often misleads pathologists into the diagnosis of clear cell RCC, should be carefully examined using an immunohistochemical panel including CA-IX, Ksp-cadherin, CD82 and CK7. © 2015 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd.

  12. Diagnostic problems in the subtyping of renal tumors encountered by five pathologists

    DEFF Research Database (Denmark)

    Kümmerlin, Intan; ten Kate, Fiebo; Smedts, Frank

    2009-01-01

    The diagnostic problems in the subtyping of renal tumors were evaluated by a panel of five pathologists studying a set of selected tumors. Five pathologists independently assessed a single hematoxylin-and-eosin (HE)-stained slide from 28 selected renal tumors. After this independent assessment, t...

  13. Revised International Society of Paediatric Oncology (SIOP) working classification of renal tumors of childhood

    NARCIS (Netherlands)

    Vujanić, Gordan M.; Sandstedt, Bengt; Harms, Dieter; Kelsey, Anna; Leuschner, Ivo; de Kraker, Jan

    2002-01-01

    The previous International Society of Paediatric Oncology (SIOP) trials and studies recognized three prognostic groups of renal tumors of childhood: low risk, intermediate risk, and high risk tumors, which were further defined in the SIOP (Stockholm) Working Classification of Renal Tumors of

  14. Investigation of DNA damage response and apoptotic gene methylation pattern in sporadic breast tumors using high throughput quantitative DNA methylation analysis technology

    Directory of Open Access Journals (Sweden)

    Prakash Neeraj

    2010-11-01

    Full Text Available Abstract Background- Sporadic breast cancer like many other cancers is proposed to be a manifestation of abnormal genetic and epigenetic changes. For the past decade our laboratory has identified genes involved in DNA damage response (DDR, apoptosis and immunesurvelliance pathways to influence sporadic breast cancer risk in north Indian population. Further to enhance our knowledge at the epigenetic level, we performed DNA methylation study involving 17 gene promoter regions belonging to DNA damage response (DDR and death receptor apoptotic pathway in 162 paired normal and cancerous breast tissues from 81 sporadic breast cancer patients, using a high throughput quantitative DNA methylation analysis technology. Results- The study identified five genes with statistically significant difference between normal and tumor tissues. Hypermethylation of DR5 (P = 0.001, DCR1 (P = 0.00001, DCR2 (P = 0.0000000005 and BRCA2 (P = 0.007 and hypomethylation of DR4 (P = 0.011 in sporadic breast tumor tissues suggested a weak/aberrant activation of the DDR/apoptotic pathway in breast tumorigenesis. Negative correlation was observed between methylation status and transcript expression levels for TRAIL, DR4, CASP8, ATM, CHEK2, BRCA1 and BRCA2 CpG sites. Categorization of the gene methylation with respect to the clinicopathological parameters showed an increase in aberrant methylation pattern in advanced tumors. These uncharacteristic methylation patterns corresponded with decreased death receptor apoptosis (P = 0.047 and DNA damage repair potential (P = 0.004 in advanced tumors. The observation of BRCA2 -26 G/A 5'UTR polymorphism concomitant with the presence of methylation in the promoter region was novel and emerged as a strong candidate for susceptibility to sporadic breast tumors. Conclusion- Our study indicates that methylation of DDR-apoptotic gene promoters in sporadic breast cancer is not a random phenomenon. Progressive epigenetic alterations in advancing

  15. Distinctive expression patterns of glycoprotein non-metastatic B and folliculin in renal tumors in patients with Birt-Hogg-Dubé syndrome.

    Science.gov (United States)

    Furuya, Mitsuko; Hong, Seung-Beom; Tanaka, Reiko; Kuroda, Naoto; Nagashima, Yoji; Nagahama, Kiyotaka; Suyama, Takahito; Yao, Masahiro; Nakatani, Yukio

    2015-03-01

    Birt-Hogg-Dubé syndrome (BHD) is an inherited disorder associated with a germline mutation of the folliculin gene (FLCN). The affected families have a high risk for developing multiple renal cell carcinomas (RCC). Diagnostic markers that distinguish between FLCN-related RCC and sporadic RCC have not been investigated, and many patients with undiagnosed BHD fail to receive proper medical care. We investigated the histopathology of 27 RCCs obtained from 18 BHD patients who were diagnosed by genetic testing. Possible somatic mutations of RCC lesions were investigated by DNA sequencing. Western blotting and immunohistochemical staining were used to compare the expression levels of FLCN and glycoprotein non-metastatic B (GPNMB) between FLCN-related RCCs and sporadic renal tumors (n = 62). The expression of GPNMB was also evaluated by quantitative RT-PCR. Histopathological analysis revealed that the most frequent histological type was chromophobe RCC (n = 12), followed by hybrid oncocytic/chromophobe tumor (n = 6). Somatic mutation analysis revealed small intragenic mutations in six cases and loss of heterozygosity in two cases. Western blot and immunostaining analyses revealed that FLCN-related RCCs showed overexpression of GPNMB and underexpression of FLCN, whereas sporadic tumors showed inverted patterns. GPNMB mRNA in FLCN-related RCCs was 23-fold more abundant than in sporadic tumors. The distinctive expression patterns of GPNMB and FLCN might identify patients with RCCs who need further work-up for BHD. © 2015 The Authors. Cancer Science published by Wiley Publishing Asia Pty Ltd on behalf of Japanese Cancer Association.

  16. Chronic kidney disease in children with unilateral renal tumor.

    Science.gov (United States)

    Cozzi, Denis A; Ceccanti, Silvia; Frediani, Simone; Schiavetti, Amalia; Cozzi, Francesco

    2012-05-01

    In patients who have undergone nephrectomy lower stage chronic kidney disease may develop, which is an independent risk factor for cardiovascular disease and overall mortality. We investigated whether the prevalence of lower stage chronic kidney disease is related to the amount of renal parenchyma excised in children with unilateral renal tumor. A total of 15 patients treated with nephrectomy and 10 treated with nephron sparing surgery were enrolled at a single academic center. The Kidney Disease Outcomes Quality Initiative guidelines were used to classify patients by chronic kidney disease stage based on estimated glomerular filtration rate values. The Modification of Diet in Renal Disease study equation and Schwartz equation were used in patients older and younger than 17 years, respectively. At a mean followup of more than 12 years 8 patients who had undergone nephrectomy and 1 treated with bilateral nephron sparing surgery presented with stage II chronic kidney disease (estimated glomerular filtration rate 60 to 89 ml/min/1.73 m(2)). Sequential measurements from diagnosis to 12 to 17 years postoperatively showed that stage II chronic kidney disease in patients who had undergone nephrectomy manifested as a negligible postoperative increase in mean ± SD estimated glomerular filtration rate (75.7 ± 25.5 vs 79.4 ± 3.9 ml/min/1.73 m(2), p = 0.6). Five of the 8 patients presented with stage II chronic kidney disease even before nephrectomy. The other 7 patients who had undergone nephrectomy and those treated with nephron sparing surgery presented with a significant postoperative increase in mean ± SD estimated glomerular filtration rate (81.1 ± 24 vs 102.3 ± 3 ml/min/1.73 m(2), p = 0.02, and 88.7 ± 2 vs 107.4 ± 14 ml/min/1.73 m(2), p = 0.005, respectively). A subset of children with unilateral renal tumor presents before and/or after nephrectomy, and not after nephron sparing surgery, with stage II chronic kidney disease, probably due to a reduced renal

  17. Somatic mutations of the ret Protooncogene in sporadic medullary thyroid carcinoma are not restricted to exon 16 and are associated with tumor recurrence

    Energy Technology Data Exchange (ETDEWEB)

    Romei, C.; Elisei, R.; Pinchera, A. [Univ. of Pisa (Italy)] [and others

    1996-04-01

    Germline point mutations in exons 10, 11, and 16 of the ret protooncogene have been identified as causative in multiple endocrine neoplasia type 2 and in familial medullary thyroid carcinoma (MTC). Somatic point mutations of the same gene, exclusively associated with codon 918 of exon 16, have also been reported in few cases of sporadic medullary thyroid carcinoma. We analyzed the blood and tumor DNA of 19 patients with sporadic MTC and 6 patients with primary parathyroid adenoma for point mutations at exons 10, 11, and 16 of the ret protooncogene by restriction analysis of the PCR-amplified product and by sequence analysis of exons 10 and 11. A Cys{sup 634}{r_arrow}Tyr mutation was found in both the tumoral and blood DNA of one patient, indicating that he was affected by an hereditary form of MTC, erroneously considered sporadic. In the other 18 patients with MTC, somatic point mutations of ret were found in 8 cases (44.4%). In 5 cases the mutation affected exon 16 (Met{sup 918}{r_arrow}Thr), and in 3 cases it affected exon 11 (Cys{sup 634}{r_arrow}Arg in 1 and Cys{sup 634}{r_arrow}Trp in 2); these 3 mutations were confirmed by sequence analysis. The remaining 10 patients had no mutation in exon 10 by either restriction analysis or sequence analysis. Clinical data showed that 75% of the patients whose tumor carried ret mutation had tumor recurrence and/or increased serum calcitonin concentrations during the postsurgical follow-up period as opposed to 10% of the patients without mutations (P < 0.02, by {chi}{sup 2} analysis). No ret mutation was found in the tumoral DNA from parathyroid adenomas. Our findings indicate that the somatic ret point mutation frequently found in sporadic MTC may affect not only exon 16 but also exon 11 and is associated with less favorable clinical outcome. 14 refs., 2 figs., 3 tabs.

  18. Integrated imaging (ultrasound, computed tomography, intravenous urography) in diagnosing renal tumors and tumor-like formations

    International Nuclear Information System (INIS)

    Drudi, F.M.; Capanna, G.; Poggi, R.; Occhiato, R.; Iannicelli, E.; Nardo, R.; di Passariello, R.

    1994-01-01

    This is an assessment of semiologic imaging criteria based on computerised tomography, ultrasound diagnosis and intravenous urography in renal tumors. The purpose is to obtain differential diagnostic data capable to modify the treatment approach. Over the last three years, a total of 570 cases of kidney tumors are observed. In 490 of them (86%) the imaging patterns obtained by either of the three techniques leads to correct diagnosis. In 62 of the remaining 80 patients, the integration of two techniques allows to unveil the neoplastic nature of the disease (27 cases), or the presence of a benign process (35 cases). In 15 of the remaining 18 cases only integration of the three techniques results in diagnosing renal tumors or tumor-like conditions (3 adeno-carcinomas, 5 abscesses, 3 cases of tuberculosis, 2 - pyeloxanthogranulomatosis, 2 dysmorphisms). In the last three cases definite diagnosis is made on the basis of needle biopsy. The radiological diagnosis is confirmed intraoperatively or during clinical follow-up study. The obtained data underscore the clinical relevance of imaging integration in evaluating renal lesions. This is particularly valid whenever the clinical data are nonspecific or misleading. 15 refs., 3 figs., 5 tabs. (orig.)

  19. Laparoscopic Radiofrequency Ablation Combined with Surgical Excision for Exophytic Renal Angiomyolipoma: A Novel Technique Based on Tumor Vasculature Features of Enhancing Renal Masses Toward Hilar Off-Clamping Nephron-Sparing Surgery.

    Science.gov (United States)

    Xiong, Wei; Ran, Qing; Du, Yangchun; Lv, Ji; Chen, Fang; Zhong, Shan; Guo, Pu; Dou, Ke; Sun, Minghan

    2017-08-01

    Symptomatic angiomyolipoma (AML) and asymptomatic AML larger than 4 cm in size are usually treated with nephron-sparing surgery or transarterial embolization. We used radiofrequency ablation to treat the vascular pedicle of exophytic AML with low R.E.N.A.L. nephrometry score and investigated its feasibility for hilar off-clamping nephron-sparing surgery. Contrast-enhanced computed tomography (CT) showed enhanced, well-defined lipomatous tumors with a maximum diameter of 4-8 cm in the kidney of 15 patients. Results indicated that the exophytic tumors featured in the enlarged tumor vasculatures extended into the parenchyma of the involved kidney. The patients underwent radiofrequency ablation by using a Cool-tip™ probe placed into the root of the AML mass from different directions under laparoscopic ultrasonography guidance. After sealing the vascular pedicle of the tumor, the bloodless tumors were resected en bloc without renal hilar clamping or suturing the resection defect of the kidney. All patients underwent the procedure smoothly, and no perioperative complications occurred. The contrast-enhanced CT scan showed small defects in the contrast-enhanced renal parenchyma at third month after the procedure, and the decrease in function of the treated kidneys was radiofrequency ablation based on the tumor vasculature features of a renal mass is an alternative to hilar clamping in laparoscopic nephron-sparing surgery. Laparoscopic radiofrequency ablation and tumor excision are a definitive and safe minimally invasive procedure that allows the successful removal of exophytic sporadic AML mass with low R.E.N.A.L. nephrometry score.

  20. Differing von Hippel Lindau genotype in paired primary and metastatic tumors in patients with clear cell renal cell carcinoma

    Directory of Open Access Journals (Sweden)

    Susan A.J. Vaziri

    2012-05-01

    Full Text Available In sporadic clear cell renal cell carcinoma (CCRCC, the von Hippel Lindau (VHL gene is inactivated by mutation or methylation in the majority of primary (P tumors. Due to differing effects of wild-type (WT and mutant (MT VHL gene on downstream signaling pathways regulating angiogenesis, VHL gene status could impact clinical outcome. In CCRCC, comparative genomic hybridization (CGH analysis studies have reported genetic differences between paired P and metastatic (M tumors. We thus sequenced the VHL gene in paired tumor specimens from 10 patients to determine a possible clonal relationship between the P tumor and M lesion(s in patients with CCRCC. Using paraffin embedded specimens, genomic DNA from microdissected samples (>80% tumor of paired P tumor and M lesions from all 10 patients, as well as in normal tissue from 6 of these cases, was analyzed. The DNA was used for PCR-based amplification of each of the 3 exons of the VHL gene. Sequences derived from amplified samples were compared to the wild-type VHL gene sequence (GeneBank Accession No. AF010238. Methylation status of the VHL gene was determined using VHL methylation-specific PCR primers after DNA bisulfite modification. In 4/10 (40% patients the VHL gene status differed between the P tumor and the M lesion. As expected, when the VHL gene was mutated in both the P tumor and M lesion, the mutation was identical. Further, while the VHL genotype differed between the primary tumor in different kidneys or multiple metastatic lesions in the same patient, the VHL germline genotype in the normal adjacent tissue was always wild-type irrespective of the VHL gene status in the P tumor. These results demonstrate for the first time that the VHL gene status can be different between paired primary and metastatic tissue in patients with CCRCC.

  1. Tumor de Wilms extra-renal: relato de caso Extrarenal Wilms' tumor: a case report

    Directory of Open Access Journals (Sweden)

    Guilherme de Castro Dabus

    2004-08-01

    Full Text Available Um caso de tumor de Wilms extra-renal de localização retroperitoneal em uma paciente do sexo feminino de dois anos de idade é apresentado, associado a revisão de literatura. Foram realizados exames de radiografia simples do abdome, urografia excretora, ultra-sonografia e tomografia computadorizada sem e com contraste, que evidenciaram a presença de massa retroperitoneal adjacente ao rim direito. A paciente foi submetida a intervenção cirúrgica, com ressecção de toda a massa, sendo o diagnóstico de tumor de Wilms confirmado com exame anatomopatológico. O tumor de Wilms extra-renal é uma entidade extremamente rara e maligna, descrito na literatura principalmente sob a forma de relato de caso. Pode ocorrer no retroperitônio, útero e ovários, canal inguinal, testículos, pele, e até mesmo no tórax. O mecanismo exato que poderia explicar a ocorrência deste tumor em tecido extra-renal não foi bem estabelecido ainda. O diagnóstico é feito através do estudo anatomopatológico da lesão, geralmente após intervenção cirúrgica.The authors report a case of a two year-old girl with an extrarenal Wilms' tumor in the retroperitoneum. Abdominal plain films, intravenous urography, abdominal ultrasound and computed tomography examinations showed a retroperitoneal mass adjacent to the right kidney. The patient underwent surgery with complete resection of the mass. The diagnosis of Wilms' tumor was confirmed by histopathological study. Extrarenal Wilms' tumor is an extremely rare malignancy which is almost always presented in medical literature as a case report. The tumor may arise in the retroperitoneum, uterus and ovaries, inguinal groove, testes, skin, and even in the thorax. The exact mechanism of occurrence of this tumor in extrarenal tissues has not yet been established. The diagnosis relies on histopathological study, generally after surgical intervention.

  2. Computed tomographic evaluation of the renal in the normal and tumor kidneys

    International Nuclear Information System (INIS)

    Masuda, Fujio; Nakada, Gyojiro; Onishi, Tetsuro; Machida, Toyohei

    1981-01-01

    In 37 normal kidneys and 23 kidneys with renal cell carcinoma, in all of which computed tomography (CT) was taken at our department of the Jikei University Hospital during the 2 years' period from Nov. 1978 to Oct. 1980, the renal vein as revealed by CT was studied. CT scanning was made with Somatom, with scanning time of 4.5 sec, the matrix of 256 x 256, and section thickness of 7 or 8 mm. As a rule, the scanning covered the region from the ensiform process to the iliac crest at intervals of 1 cm. The renal vein could be depicted by this method in 89 and 87% of the normal and tumor kidneys, respectively. There was a tendency that the right renal vein was depicted with lower incidence than the left. The best picture of the renal vein was obtained with scanning in the area at 7 and 8 cm level below the ensiform process for both normal and tumor kidneys. The diameter of the normal renal vein was less than 1.0 cm in 89% of the cases, whith no case exceeding 1.4 cm. Therefore, in cases where there was an extention of the renal vein with a diameter larger than 1.4 cm, tumor thrombus in the renal vein or renal arterio-venous fistula should be considered. In fact, among 5 cases where an extended renal vein was observed, a tumor thrombus was detected in the renal vein in 4 cases and a renal arterio-venous fistula in one case. When CT is performed in renal disease, especially for diagnosis of renal tumor, it is important to pay attention to diseases not only in the kidney but in the renal vein also. (author)

  3. PI3K/AKT/mTOR pathway plays a major pathogenetic role in glycogen accumulation and tumor development in renal distal tubules of rats and men.

    Science.gov (United States)

    Ribback, Silvia; Cigliano, Antonio; Kroeger, Nils; Pilo, Maria G; Terracciano, Luigi; Burchardt, Martin; Bannasch, Peter; Calvisi, Diego F; Dombrowski, Frank

    2015-05-30

    Activation of the PI3K/AKT/mTOR pathway is a crucial molecular event in human clear cell renal cell carcinoma (ccRCC), and is also upregulated in diabetic nephropathy. In diabetic rats metabolic changes affect the renal distal tubular epithelium and lead to glycogen-storing Armanni-Ebstein lesions (AEL), precursor lesions of RCC in the diabetes induced nephrocarcinogenesis model. These lesions resemble human sporadic clear cell tubules (CCT) and tumor cells of human ccRCC.Human sporadic CCT were examined in a collection of 324 nephrectomy specimen, in terms of morphologic, metabolic and molecular alterations, and compared to preneoplastic CCT and RCC developed in the rat following streptozotocin-induced diabetes or N-Nitrosomorpholine administration. Diabetic and non-diabetic rats were subjected to the dual PI3K/mTOR inhibitor, NVP/BEZ235.Human sporadic CCT could be detected in 17.3% of kidney specimens. Human and rat renal CCT display a strong induction of the PI3K/AKT/mTOR pathway and related metabolic alterations. Proteins involved in glycolysis and de novo lipogenesis were upregulated. In in vivo experiments, dual inhibition of PI3K and mTOR resulted in a reduction of proliferation of rat diabetes related CCT and increased autophagic activity.The present data indicate that human sporadic CCT exhibit a pattern of morphologic and metabolic alterations similar to preneoplastic lesions in the rat model. Activation of the PI3K/AKT/mTOR pathway in glycogenotic tubuli is a remarkable molecular event and suggests a preneoplastic character of these lesions also in humans.

  4. Kras gene mutation and RASSF1A, FHIT and MGMT gene promoter hypermethylation: indicators of tumor staging and metastasis in adenocarcinomatous sporadic colorectal cancer in Indian population.

    Directory of Open Access Journals (Sweden)

    Rupal Sinha

    Full Text Available Colorectal cancer (CRC development involves underlying modifications at genetic/epigenetic level. This study evaluated the role of Kras gene mutation and RASSF1A, FHIT and MGMT gene promoter hypermethylation together/independently in sporadic CRC in Indian population and correlation with clinicopathological variables of the disease.One hundred and twenty four consecutive surgically resected tissues (62 tumor and equal number of normal adjacent controls of primary sporadic CRC were included and patient details including demographic characteristics, lifestyle/food or drinking habits, clinical and histopathological profiles were recorded. Polymerase chain reaction - Restriction fragment length polymorphism and direct sequencing for Kras gene mutation and Methylation Specific-PCR for RASSF1A, FHIT and MGMT genes was performed.Kras gene mutation at codon 12 & 13 and methylated RASSF1A, FHIT and MGMT gene was observed in 47%, 19%, 47%, 37% and 47% cases, respectively. Alcohol intake and smoking were significantly associated with presence of Kras mutation (codon 12 and MGMT methylation (p-value <0.049. Tumor stage and metastasis correlated with presence of mutant Kras codon 12 (p-values 0.018, 0.044 and methylated RASSF1A (p-values 0.034, 0.044, FHIT (p-values 0.001, 0.047 and MGMT (p-values 0.018, 0.044 genes. Combinatorial effect of gene mutation/methylation was also observed (p-value <0.025. Overall, tumor stage 3, moderately differentiated tumors, presence of lymphatic invasion and absence of metastasis was more frequently observed in tumors with mutated Kras and/or methylated RASSF1A, FHIT and MGMT genes.Synergistic interrelationship between these genes in sporadic CRC may be used as diagnostic/prognostic markers in assessing the overall pathological status of CRC.

  5. Diffuse thyroid metastases and bilateral internal jugular vein tumor thrombus from renal cell cancer

    OpenAIRE

    Jha, Priyanka; Shekhar, Mallika; Wan, Jennifer; Mari-Aparici, Carina

    2016-01-01

    Renal cell cancer rarely metastasizes to the thyroid gland, and it has been reported to present as a solitary mass. We present a case of diffuse thyroid cancer metastases from renal cell cancer. Bilateral internal jugular vein tumor thrombi were also present. To the best of our knowledge, this is the first description of diffuse thyroid metastases from renal cell cancer in the English literature. Renal cell cancer metastases should be considered in the differential of thyroid imaging abnormal...

  6. Evaluation of morphologically unclassified renal cell carcinoma with electron microscopy and novel renal markers: implications for tumor reclassification.

    Science.gov (United States)

    Talento, Romualdo; Hewan-Lowe, Karlene; Yin, Ming

    2013-02-01

    Despite progress in the classification of renal cell carcinomas (RCC), a subset of these carcinomas remains unclassified (RCC-U). Patients with RCC-U usually present at a late stage and have a poor prognosis. Several studies have attempted to extract new classifications of newly recognized renal carcinomas from the group of RCC-U. However, to date, no studies in the literature have attempted to characterize the RCC-U with unrecognizable cell types beyond the morphologic evaluation on H&E-stained sections. The purpose of this study was to evaluate this group of RCC-U using electron microscopy and novel renal markers. Ten cases of such RCC-U were identified for this study. At the ultrastructural level, they did not show typical morphology that resembled any of the well-studied, recognizable subtypes of RCC. However, they did reveal features of renal tubular epithelial differentiation. The histologic, ultrastructural, and immunophenotypic features indicated that these tumors are poorly differentiated renal epithelial tumors, possibly derived from the proximal nephron, with an immunohistochemical profile similar to high-grade clear cell RCC. It is, therefore, proposed that this group of renal carcinomas be renamed "poorly differentiated renal cell carcinoma, not otherwise specified." The current study showed that PAX-8 and carbonic anhydrase IX are reliable markers for this novel group of renal carcinoma, and that electron microscopy is an important adjunct in the evaluation of new and unusual renal entities.

  7. [A case of multilocular cystic renal cell carcinoma treated by partial nephrectomy associated with adrenal tumor].

    Science.gov (United States)

    Fukuoka, H; Ishibashi, Y; Fujinami, K; Tsuchiya, F; Sakanishi, S

    1994-12-01

    A case of multilocular cystic renal cell carcinoma was reported. The patient was 69-year-old male who had been examined for postoperative study of gastric cancer by abdominal CT. The abdominal CT incidentally revealed right adrenal tumor which was non-functional and multilocular cysts in the lower pole of the right kidney. Selective renal arteriography showed a hypovascular mass with fine neovascularity. These two findings of CT and arteriography were though to represent a probable malignant tumor but renal function of the patient decreased moderately. Surgical exploration was done and right renal masses were thought to be seen benign multilocular cysts without capsule. Simple excision of the wall of cysts and right adrenalectomy were performed. Pathological examinations showed multilocular cystic renal cell carcinoma and benign adrenal hyperplasia. Additionally partial nephrectomy was done. Surgical margin of the kidney was tumor free and postoperative course was uneventful. Prognosis of multiocular cystic renal cell carcinoma is good, therefore conservative surgery is recommended.

  8. Myxoma of the kidney - an unusual benign renal tumor: a case report.

    Science.gov (United States)

    Tenkorang, Somuah; Kharbach, Youssef; Omana, Jean-Paul; Efared, Boubacar; Mellas, Soufiane; Tazi, Mohammed Fadl; Sekal, Mohamed; Harmouch, Taoufik; Khallouk, Abdelhak; El Fassi, Jamal Mohammed; El Ammari, Jalal Eddine; Farih, Moulay Hassan

    2017-02-14

    Myxomas are rare benign soft tissue tumors. The kidney is an unusual location for this tumor. For this reason, less than 15 cases of renal myxoma have been reported in the English literature. There are no specific clinical and radiological features reported for this tumor that allow a preoperative diagnosis enabling a conservative treatment. We report another case of renal myxoma found in a 50-year-old Moroccan woman who presented with a right dull flank pain. An abdominal computed tomography scan objectified a suspected malignant renal mass. Thus, radical nephrectomy was performed. Histopathology of the specimen revealed the typical appearance of a myxoma. The objective of this report is to add another case report of this rare benign renal tumor to the literature. This benign tumor with excellent prognosis has no specific preoperative features that could justify a conservative management; a radical approach remains the therapeutic option for now.

  9. CT staging of renal cell carcinoma: Emphasis on perinephric tumor extension

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Yun Young; Kim, Sun Mi; Choi, Mun Hwan; Bang, Duk Ja; Koh, Byung Hee; Cho, On Koo [Hanyang University College of Medicine, Seoul (Korea, Republic of)

    1993-07-15

    A total of 47 preoperative CT scans in patients with renal cell carcinoma were retrospectively reviewed and compared with surgical findings to assess the accuracy of CT for determining the perinephric tumor extension. CT criteria for perinephric extension were hazy ill-defined tumor margin, perirenal nodule and fascial thickening. Regardless of the tumor stage, the accuracy of CT in detecting perinephric extension was 76.6% (36/47), with a sensitivity of 88.9% (16/18) and specificity of 68.9% (20/29). The cause of understaging (n=2) was microscopic infiltration of the perinephric space. The causes of overstaging were tumor infiltration to the renal capsule (n=5), partial adhesion with the perinephric fat (n=3) and renal vein thrombosis (n=1). A smooth clear tumor margin is highly reliable sign for stage I but infiltrative findings onto renal capsule and perirenal fat could be considered stage I.

  10. Apparent diffusion coefficient measurement by diffusion weighted magnetic resonance imaging is a useful tool in differentiating renal tumors.

    Science.gov (United States)

    Liu, Jing-Hong; Tian, Shi-Feng; Ju, Ye; Li, Ye; Chen, An-Liang; Chen, Li-Hua; Liu, Ai-Lian

    2015-04-16

    To determine the clinical value of apparent diffusion coefficient (ADC) measurement by diffusion weighted magnetic resonance imaging (DW-MRI) in differentiating renal tumors. Electronic databases were searched using combinations of keywords and free words relating to renal tumor, ADC and DW-MRI. Based on carefully selected inclusion and exclusion criteria, relevant case-control studies were identified and the related clinical data was acquired. Statistical analyses were performed using STATA 12.0 (Stata Corporation, College station, TX). Sixteen case-control studies were ultimately included in the present meta-analysis. These 16 high quality studies contained a combined total of 438 normal renal tissues and 832 renal tumor lesions (597 malignant and 235 benign). The results revealed that ADC values of malignant renal tumor tissues were markedly lower than normal renal tissues and benign renal tumor tissues. ADC values of benign renal tumor tissues were also significantly lower than normal renal tissue. ADC measurement by DW-MRI provided clinically useful information on the internal structure of renal tumors and could be an important radiographic index for differentiation of malignant renal tumors from benign renal tumors.

  11. Massive hematuria due to a congenital renal arteriovenous malformation mimicking a renal pelvis tumor: a case report

    Directory of Open Access Journals (Sweden)

    Sountoulides P

    2008-05-01

    Full Text Available Abstract Introduction Congenital renal arteriovenous malformations (AVMs are very rare benign lesions. They are more common in women and rarely manifest in elderly people. In some cases they present with massive hematuria. Contemporary treatment consists of transcatheter selective arterial embolization which leads to resolution of the hematuria whilst preserving renal parenchyma. Case presentation A 72-year-old man, who was heavy smoker, presented with massive hematuria and flank pain. CT scan revealed a filling defect caused by a soft tissue mass in the renal pelvis, which initially led to the suspicion of a transitional cell carcinoma (TCC of the upper tract, in view of the patient's age and smoking habits. However a subsequent retrograde study could not depict any filling defect in the renal pelvis. Selective right renal arteriography confirmed the presence of a renal AVM by demonstrating abnormal arterial communication with a vein with early visualization of the venous system. At the same time successful selective transcatheter embolization of the lesion was performed. Conclusion This case highlights the importance of careful diagnostic work-up in the evaluation of upper tract hematuria. In the case presented, a congenital renal AVM proved to be the cause of massive upper tract hematuria and flank pain in spite of the initial evidence indicating the likely diagnosis of a renal pelvis tumor.

  12. CT of acquired cystic kidney disease and renal tumors in long-term dialysis patients

    International Nuclear Information System (INIS)

    Levine, E.; Grantham, J.J.; Slusher, S.L.; Greathouse, J.L.; Krohn, B.P.

    1984-01-01

    The kidneys of long term dialysis patients frequently demonstrate multiple small acquired cysts and renal cell tumors on pathologic examination. The original kidneys of 30 long-term dialysis patients and six renal transplant patients were evaluated by computed tomography to determine the incidence of these abnormalities. Among dialysis patients, 43.3% had diffuse bilateral cysts, while 16.7% had occasional cysts (fewer than five per kidney), and 40% showed no renal cysts. Seven solid renal tumors were detected in four dialysis patients with renal cysts. Acquired cystic kidney disease tends to result in renal enlargement, is more common in patients who have been maintained on dialysis for prolonged periods, and may lead to spontaneous renal hemorrhage. The six transplant patients showed no evidence of renal cysts, and all had markedly shrunken kidneys. Acquired cystic disease and renal cell tumors in the original kidneys of dialysis patients may be due to biologically active substances that are not cleared effectively by dialysis but that are removed by normally functioning transplant kidneys

  13. Hyperpolarized 13C MR Markers of Renal Tumor Aggressiveness

    Science.gov (United States)

    2015-12-01

    lactate flux than the normal renal tubule cells. Furthermore, a key feature distinguishing the localized from the metastatic RCC cells is the lactate...cells derived fromhuman renal proximal tubules (the origin of most human RCCs), a localized human RCC, and a metastatic human RCC, with the goal of...cells have significantly higher pyruvate-to-lactate flux than the normal renal proximal tubule cells. Furthermore, we showed that cells derived from

  14. Suprarenal inferior vena cava filter placement prior to transcatheter arterial embolization (TAE) of a renal cell carcinoma with large renal vein tumor thrombus: Prevention of pulmonary tumor emboli after TAE

    International Nuclear Information System (INIS)

    Hirota, Shozo; Matsumoto, Shinnichi; Ichikawa, Satoshi; Tomita, Masaru; Koshino, Tukasa; Sako, Masao; Kono, Michio

    1997-01-01

    To prevent embolization of necrotic renal vein tumor after transcatheter embolization of a left renal cell carcinoma, we placed a suprarenal Bird's nest inferior vena cava filter. The patient tolerated the procedure well and had extensive tumor infarction including the tumor thrombus on 6-month follow-up computed tomography

  15. Crioterapia de tumores renales: estado actual y desarrollos contemporáneos [= Cryotherapy for renal tumors: Current status and contemporary developments

    NARCIS (Netherlands)

    Rioja, J.; Tzortzis, V.; Mamoulakis, C.; Laguna, M. P.

    2010-01-01

    The proportion of renal tumors found incidentally dramatically increased in the past decade. More than half of them were diagnosed in patients over 70 years of age, a population with high associated comorbidity. Nephron-sparing minimally invasive surgical procedures are aimed at treating patients

  16. Renal arteriography

    Science.gov (United States)

    Renal angiogram; Angiography - kidney; Renal angiography; Renal artery stenosis - arteriography ... artery by a blood clot Renal artery stenosis Renal cell cancer Angiomyolipomas (noncancerous tumors of the kidney) Some of these problems can be treated with ...

  17. Management for Patients with De Novo or Recurrent Tumors in the Residual Kidney after Surgery for Nonfamilial Bilateral Renal Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Noboru Hara

    2009-01-01

    Full Text Available The tumor de novo in the residual kidney after surgery for nonfamilial bilateral renal cell carcinoma (RCC is problematic. We reviewed 5 patients who experienced such a situation. Three patients had had metachronous bilateral RCC, treated with radical nephrectomy in one kidney and nephron-sparing surgery (NSS in the other. Two patients had had synchronous disease; one patient had received radical nephrectomy and NSS, and the other bilateral NSS. The 5 patients had another solid mass/de novo tumor in the residual kidney 16–88 (mean 46.8 months after surgery. For the tumor de novo in earlier years (1992–1999, one patient underwent surgery and hemodialysis, and the other selected a conservative observation. In recent years (2000–2007, one patient was conservatively observed; the remaining 2 received computerized-tomography-guided radiofrequency ablation, and the local tumors were well controlled postoperatively for 20 and 12 months with their renal function unimpaired. Ablative techniques can potentially strike a balance between oncological and nephrological outcomes in patients with sporadic multiple RCC, successful management of which was difficult previously.

  18. Brown tumor of lumber spint in patient with chronic renal failure ...

    African Journals Online (AJOL)

    Brown tumors are erosive bone lesions caused by increased osteoclastic activity. They usually occur in the severe forms of secondary hyperparathyroidism, as in patients with hemodialysis-dependent chronic renal disease. Involvement of the lumbar spine with this tumor causing neural compression is extremely rare.

  19. Folliculin contributes to VHL tumor suppressing activity in renal cancer through regulation of autophagy.

    Directory of Open Access Journals (Sweden)

    Prabhat Bastola

    Full Text Available Von Hippel-Lindau tumor suppressor (VHL is lost in the majority of clear cell renal cell carcinomas (ccRCC. Folliculin (FLCN is a tumor suppressor whose function is lost in Birt-Hogg-Dubé syndrome (BHD, a disorder characterized by renal cancer of multiple histological types including clear cell carcinoma, cutaneous fibrofolliculoma, and pneumothorax. Here we explored whether there is connection between VHL and FLCN in clear cell renal carcinoma cell lines and tumors. We demonstrate that VHL regulates expression of FLCN at the mRNA and protein levels in RCC cell lines, and that FLCN protein expression is decreased in human ccRCC tumors with VHL loss, as compared with matched normal kidney tissue. Knockdown of FLCN results in increased formation of tumors by RCC cells with wild-type VHL in orthotopic xenografts in nude mice, an indication that FLCN plays a role in the tumor-suppressing activity of VHL. Interestingly, FLCN, similarly to VHL, is necessary for the activity of LC3C-mediated autophagic program that we have previously characterized as contributing to the tumor suppressing activity of VHL. The results show the existence of functional crosstalk between two major tumor suppressors in renal cancer, VHL and FLCN, converging on regulation of autophagy.

  20. [Conservative surgical treatment of renal carcinoma. Personal experience with 29 surgical excisions of tumors].

    Science.gov (United States)

    Villani, U; Pastorello, M

    1991-03-01

    From 1980 to 1988, elective conservative surgery (tumorectomy by enucleo-resection) was performed for renal cell carcinoma at stage I in 29 patients. An accurate preoperative renal investigation was carried out to identify the exact extension of the tumor and to study all the parenchimal situation, through IVP, ultrasound, CT scanning and, particularly, conventional selective angiography. The operative technique employed was: lymphadenectomy, peri-pararenal fat extirpation, in situ tumor enucleation by circular incision of the renal capsule and blunt dissection of the renal parenchyma with 2 cm safety margin to the tumor; multiple biopsies in the "bed" of resection for histopathologic peroperative evaluation; careful examination of the pseudocapsule and surrounding renal tissue; hemostasis. Follow-up was 10-113 months (mean 40,34 months). 2 of 29 patients died for progression of disease (at 52nd and 16yh month from surgery, 2/29 died for non-neoplastic reasons; 25/29 pts are living without local recurrences or distant metastases. In the same period (1980-1988), radical nephrectomy was performed for renal tumors at stage I in 34 patients. In an average observation period of 49,67 months, 2/34 patients died for progression of disease; 3/34 pts died for non-neoplastic reasons. 1/34 patient is living with pulmonar metastases and 28/34 are living without evidence of cancer. From this study we have got the conclusion that elective renal-sparing excision of the tumor (with macro-micro examination of the abscission surfaces) should be considered as a curative treatment in the case of low stage single tumors smaller than 7 cm, peripherally located in renal cortex, with unbroken pseudocapsule.

  1. Expression of Translationally Controlled Tumor Protein in Human Kidney and in Renal Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Maria R. Ambrosio

    2015-01-01

    Full Text Available Translationally controlled tumor protein is a multifaceted protein involved in several physiological and biological functions. Its expression in normal kidney and in renal carcinomas, once corroborated by functional data, may add elements to elucidate renal physiology and carcinogenesis. In this study, translationally controlled tumor protein expression was evaluated by quantitative real time polymerase chain reaction and western blotting, and its localization was examined by immunohistochemistry on 84 nephrectomies for cancer. In normal kidney protein expression was found in the cytoplasm of proximal and distal tubular cells, in cells of the thick segment of the loop of Henle, and in urothelial cells of the pelvis. It was also detectable in cells of renal carcinoma with different pattern of localization (membranous and cytoplasmic depending on tumor histotype. Our data may suggest an involvement of translationally controlled tumor protein in normal physiology and carcinogenesis. However, functional in vitro and in vivo studies are needed to verify this hypothesis.

  2. A expressão de genes reparadores do DNA nos tumores sincrônicos de câncer colorretal esporádico DNA repair gene expression in synchronic tumors of sporadic colorectal cancer

    Directory of Open Access Journals (Sweden)

    Igor Proscurshim

    2007-03-01

    Full Text Available RACIONAL: Um dos mecanismos genéticos presentes em aproximadamente 80% dos pacientes com síndrome hereditária não-polipóide do câncer colorretal (HNPCC são os defeitos nos genes reparadores de DNA, como o MSH2, MSH6 e MLH1, onde os tumores sincrônicos são relativamente freqüentes. Já no câncer colorretal esporádico as lesões sincrônicas são raras. OBJETIVO: Verificar se o mesmo mecanismo genético presente no HNPCC está presente no câncer colorretal esporádico que apresentam com lesões sincrônicas. MÉTODOS: Foram incluídos no estudo todos os pacientes com câncer colorretal sincrônico não HNPCC. Imunoistoquímica com anticorpos para MSH2,MSH6, e MLH1 foi realizada para cada tumor. RESULTADOS: Todos os pacientes apresentaram expressão normal de MSH2 e MLH1. O único gene com imunoexpressão alterada foi o MSH6. CONCLUSÃO: Possivelmente outro mecanismo genético seja responsável pelo surgimento de dois tumores sincrônicos no câncer colorretal esporádico.BACKGROUND: Mismatch repair genes (such as MSH2, MLH1 and MSH6 mutations are present in over 80% of hereditary non-polyposis colorectal cancer (HNPCC tumors, which frequently exhibit synchronous lesions. Sporadic colorectal cancer is rarely associated with synchronous lesions. AIM: To investigate the role of mismatch repair gene mutation in synchronous sporadic colorectal cancer. METHODS: Patients with sporadic synchronous colorectal adenocarcinomas were included in the study. Immunohistochemistry was performed using MSH2, MLH1 and MSH6 antibodies. RESULTS: All patients had two synchoronous lesions. None of them had altered MSH2 or MLH1 expression. One patient had altered MSH6 expression in both tumors. CONCLUSION: Possibly, other molecular mechanisms are involved in carcinogenesis of sporadic synchronous colorectal cancer.

  3. Renal Cell Carcinoma: Comparison of RENAL Nephrometry and PADUA Scores with Maximum Tumor Diameter for Prediction of Local Recurrence after Thermal Ablation.

    Science.gov (United States)

    Maxwell, Aaron W P; Baird, Grayson L; Iannuccilli, Jason D; Mayo-Smith, William W; Dupuy, Damian E

    2017-05-01

    Purpose To evaluate the performance of the radius, exophytic or endophytic, nearness to collecting system or sinus, anterior or posterior, and location relative to polar lines (RENAL) nephrometry and preoperative aspects and dimensions used for anatomic classification (PADUA) scoring systems and other tumor biometrics for prediction of local tumor recurrence in patients with renal cell carcinoma after thermal ablation. Materials and Methods This HIPAA-compliant study was performed with a waiver of informed consent after institutional review board approval was obtained. A retrospective evaluation of 207 consecutive patients (131 men, 76 women; mean age, 71.9 years ± 10.9) with 217 biopsy-proven renal cell carcinoma tumors treated with thermal ablation was conducted. Serial postablation computed tomography (CT) or magnetic resonance (MR) imaging was used to evaluate for local tumor recurrence. For each tumor, RENAL nephrometry and PADUA scores were calculated by using imaging-derived tumor morphologic data. Several additional tumor biometrics and combinations thereof were also measured, including maximum tumor diameter. The Harrell C index and hazard regression techniques were used to quantify associations with local tumor recurrence. Results The RENAL (hazard ratio, 1.43; P = .003) and PADUA (hazard ratio, 1.80; P systems and other evaluated biometrics for prediction of local tumor recurrence after renal cell carcinoma ablation. © RSNA, 2016.

  4. Absence of activating mutations in ras and gsp oncogenes in a cohort of nine patients with sporadic pediatric thyroid tumors

    NARCIS (Netherlands)

    Pauws, E.; Tummers, R. F.; Ris-Stalpers, C.; de Vijlder, J. J.; Voûte, T.

    2001-01-01

    BACKGROUND: Characterization of the genetic background of pediatric thyroid carcinomas could aid in distinguishing between differently staged tumors with respect to treatment and prognosis. Two known genetic factors associated with thyroid carcinoma, the proto-oncogenes gsp and ras were

  5. THE PRESENCE OF METASTASES IN REGIONAL LYMPH NODES IS ASSOCIATED WITH TUMOR SIZE AND DEPTH OF INVASION IN SPORADIC GASTRIC ADENOCARCINOMA

    Science.gov (United States)

    CAMBRUZZI, Eduardo; de AZEREDO, Andreza Mariane; KRONHART, Ardala; FOLTZ, Katia Martins; ZETTLER, Cláudio Galeano; PÊGAS, Karla Lais

    2014-01-01

    Background Gastric adenocarcinoma is more often found in men over 50 years in the form of an antral lesion. The tumor has heterogeneous histopathologic features and a poor prognosis (median survival of 15% in five years). Aim To estimate the relationship between the presence of nodal metastasis and other prognostic factors in sporadic gastric adenocarcinoma. Method Were evaluated 164 consecutive cases of gastric adenocarcinoma previously undergone gastrectomy (partial or total), without clinical evidence of distant metastasis, and determined the following variables: topography of the lesion, tumor size, Borrmann macroscopic configuration, histological grade, early or advanced lesions, Lauren histological subtype, presence of signet ring cell, degree of invasion, perigastric lymph node status, angiolymphatic/perineural invasion, and staging. Results Were found 21 early lesions (12.8%) and 143 advanced lesions (87.2%), with a predominance of lesions classified as T3 (n=99/60, 4%) and N1 (n=62/37, 8%). The nodal status was associated with depth of invasion (p<0.001) and tumor size (p<0.001). The staging was related to age (p=0.048), histological grade (p=0.003), and presence of signet ring cells (p = 0.007), angiolymphatic invasion (p = 0.001), and perineural invasion (p=0.003). Conclusion In gastric cancer, lymph node involvement, tumor size and depth of invasion are histopathological data associated with the pattern of growth/tumor spread, suggesting that a wide dissection of perigastric lymph nodes is a fundamental step in the surgical treatment of these patients. PMID:24676292

  6. Prostatic adenocarcinoma (PCa metastasizing to renal cell carcinoma (RCC with periureteral tumor deposit: A case of tumor-to-tumor metastasis (TTM

    Directory of Open Access Journals (Sweden)

    Jenissa Amor Dionisio Arceño, MD

    2017-06-01

    Full Text Available Renal cell carcinoma (RCC and prostatic adenocarcinoma (PCa, occurring as a double primary is uncommon, but well documented. However, metastatic PCa in a RCC is quite rare. We report a case of an 81-year old male chemical engineer with history of hematuria and prostatomegaly suspicious for carcinoma, who underwent left radical nephrectomy for a renal mass. Histopathology revealed RCC that harbored an undiagnosed PCa. Periureteral tumor deposit likewise showed combined metastasis of RCC and PCa.

  7. Role of tumor-associated macrophages in renal cell carcinoma pathogenesis

    Directory of Open Access Journals (Sweden)

    O. V. Kovaleva

    2017-01-01

    Full Text Available The role of tumor stroma in malignant tumor pathogenesis cannot be disputed. Macrophages are one of the crucial elements of tumor stroma. Tumor-associated macrophages (TAMs are type 2-activated macrophages (M2. They were first described in 1992. They carry CD206, CD163, FXIIIa, βIG-H3, stabilin 1, YKL-39, SI-CLP, tenascin С, LOX-1, fibronectin, MARCO, interleukin 1 receptor antagonist (IL-1RA and other markers. Unlike proinflammatory macrophages (M1, М2 display high anti-inflammatory activity and are responsible for inflammation reaction suppression and tissue recovery in inflamed area. TAMs significantly contribute to tumor progression by stimulating cell proliferation, angiogenesis, and suppression of antitumor immune response. Identification of macrophages in renal tumors involves a limited number of markers, which doesn’t allow making a conclusive answer about their function. However, a correlation between TAMs content and a negative disease prognosis can be considered proven. Studies of M1 and M2 using different markers have shown that renal tumors contain high levels of TAMs with mixed M1/M2 phenotype. TAMs in renal tumors are highly proangiogenic and immunosuppressive. TAMs density can be used as a prognostic marker, but development of an effective treatment strategy aimed at inhibition of TAMs antitumor activity requires systemic research involving a wide panel of M1 and M2 macrophage markers. 

  8. Tailored Beta-catenin mutational approach in extra-abdominal sporadic desmoid tumor patients without therapeutic intervention

    International Nuclear Information System (INIS)

    Broekhoven, Danique L.M. van; Grünhagenl, Dirk J.; Dalen, Thijs van; Coevorden, Frits van; Bonenkamp, Han J.; Been, Lukas B.; Bemelmans, Marc H.A.; Dijkstra, Sander D.S.; Colombo, Chiara; Gronchi, Alessandro; Verhoef, Cornelis

    2016-01-01

    The efficacy of the classical treatment modalities surgery and radiotherapy in the treatment of aggressive fibromatosis is presently disputed and there is a shift towards a more conservative approach. The aim of the present study is to objectify tumor growth in patients with extra-abdominal or abdominal wall aggressive fibromatosis, while adhering to a “watchful waiting” policy. Other objectives are to investigate quality of life and to identify factors associated with tumor growth, in particular the relation with the presence of a CTNNB1-gene mutation in the tumor. GRAFITI is a nationwide, multicenter, prospective registration trial. All patients with extra-abdominal or abdominal wall aggressive fibromatosis are eligible for inclusion in the study. Main exclusion criteria are: history of familiar adenomatous polyposis, severe pain, functional impairment, life/limb threating situations in case of progressive disease. Patients included in the study will be treated with a watchful waiting policy during a period of 5 years. Imaging studies with ultrasound and magnetic resonance imaging scan will be performed during follow-up to monitor possible growth: the first years every 3 months, the second year twice and the yearly. In addition patients will be asked to complete a quality of life questionnaire on specific follow-up moments. The primary endpoint is the rate of progression per year, defined by the Response Evaluation Criteria In Solid Tumors (RECIST). Secondary endpoints are quality of life and the rate of influence on tumor progression for several factors, such as CTNNB1-mutations, age and localization. This study will provide insight in tumor behavior, the effect on quality of life and clinicopathological factors predictive of tumor progression. The GRAFITI trial is registered in the Netherlands National Trial Register (NTR), number: NTR4714

  9. Diffuse thyroid metastases and bilateral internal jugular vein tumor thrombus from renal cell cancer.

    Science.gov (United States)

    Jha, Priyanka; Shekhar, Mallika; Wan, Jennifer; Mari-Aparici, Carina

    2016-12-01

    Renal cell cancer rarely metastasizes to the thyroid gland, and it has been reported to present as a solitary mass. We present a case of diffuse thyroid cancer metastases from renal cell cancer. Bilateral internal jugular vein tumor thrombi were also present. To the best of our knowledge, this is the first description of diffuse thyroid metastases from renal cell cancer in the English literature. Renal cell cancer metastases should be considered in the differential of thyroid imaging abnormalities arising in the setting of known renal cell carcinoma, particularly late in the course of disease. This is frequently associated with internal jugular vein thrombi, which should be evaluated with an abnormal thyroid. Thyroglobulin levels are usually normal in such patients.

  10. Skull metastasis revealing a renal tumor: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Mohamed Badri

    Full Text Available Background: Renal cell carcinomas represent 85% of malignant renal tumors. Typically, the tumor remains asymptomatic a long time before the appearance of urologic clinical signs. In some cases, metastasis can precede the manifestations of the primary tumor. Different sites are potential metastatic localizations for renal tumors, including skull metastases who represent a very rare location. Case description: We report the case of a 65-year-old man presented after the appearance of a skull mass. This tumefaction developed and had progressively grown up during 9 months. Neurological examination was normal. Brain imaging showed a soft tissue lesion in the left parietal bone with marked osteolysis. Peroperative was found a huge oval-shape hemorrhagic and firm mass associated with scalp invasion and bone destruction that was totally resected. Histopathology revealed renal cell carcinoma (RCC. Pelvic and abdominal CT scan was performed, revealing a large mass on the left kidney with irregular contours and poor definition. The patient was then transferred to urology where he underwent nephrectomy. The patient went then through adjuvant chemotherapy. Clinical and radiological follow up of 12 months did not bring to light tumor recurrence. Conclusions: Although metastases to the head and neck occur infrequently, they should be considered when evaluating any unusual subcutaneous mass in the head and neck. RCC should not be discounted when sites as unlikely as the calvaria are evaluated. Treatment of metastatic renal cell carcinoma is complex, and the optimal regimen for achieving a lasting response without severe toxicity has not yet been defined. Keywords: Renal tumor, Skull metastasis, Neurosurgery

  11. Effect of low dose irradiation on renal enlargement in children following nephrectomy for Wilms' tumor

    International Nuclear Information System (INIS)

    Cassady, J.R.; Lebowitz, R.L.; Jaffe, N.; Hoffman, A.

    1981-01-01

    The effect of low dose irradiation on subsequent compensatory renal enlargement in a group of children operated upon with unilateral nephrectomy for Wilms' tumor is reported. Comparison groups were children whose remaining kidney received either no direct irradiation or irradiation to only the upper portion of the kidney. The rate of growth of the kidney in the irradiated group was slightly less but this was not statistically significant. Thus radiation therapy in children with Wilm's tumor, so crucial to optimum survival, does not seem to cause significant renal toxicity. (Auth.)

  12. Morphometric scores for renal tumors: What does the radiologist need to know?

    Energy Technology Data Exchange (ETDEWEB)

    Millet, Ingrid; Doyon, Fernanda Curros; Pages, Emma [Department of Imaging, CHU Montpellier (France); Thuret, Rodolphe [Department of Urology, CHU Montpellier (France); Taourel, Patrice, E-mail: p-taourel@chu-montpellier.fr [Department of Imaging, CHU Montpellier (France)

    2014-08-15

    Numerous therapeutic options are possible in the treatment of renal carcinomas including radical nephrectomy, partial nephrectomy, cryoablation, radiofrequency, active follow-up and among surgical treatments, different approaches may be used such as laparotomy, laparoscopy, robotic-assisted intervention. The choice between these different procedures is partially based on the anatomic conditions of the tumors. Different anatomic scores determined from cross-sectional imaging have been built to predict the complexity of the surgical procedure. The goals of this article are to review the relevant morphologic pattern for management of patients with renal tumors, to know how to calculate these different scores and to understand the clinical applications of these scores.

  13. Diagnostic Value of Processing Cytologic Aspirates of Renal Tumors in Agar Cell (Tissue) Blocks

    DEFF Research Database (Denmark)

    Smedts, F.; Schrik, M.; Horn, T.

    2010-01-01

    Objective To adapt a method enabling utilization of most of the harvest from a fine needle aspirate in an effort to improve diagnostic accuracy in the assessment of a renal tumor in a single histologic slide. Study Design In a series of 43 renal tumors, 2 fine needle aspirations were performed, 4...... smears were prepared after each aspiration for conventional cytology and the remaining aspirate was processed for the improved agar microbiopsy (AM) method. Conventional cytology slides, AM slides and surgical specimens were diagnosed separately, after which the diagnoses were compared...

  14. Brown tumor: clinical findings of secondary hyperparathyroidism in patients with renal osteodystrophy.

    Science.gov (United States)

    Silva, Mairaira Teles Leão E; Cedraz, Juliana Silva Barros; Pontes, Caetano Guilherme Carvalho; Trento, Cleverson Luciano; Brasileiro, Bernardo Ferreira; Piva, Marta Rabello; Pereira, Fabiano Alvim

    2017-01-01

    A brown tumor, or osteoclastoma, is a nonneoplastic bony lesion associated with hyperparathyroidism and directly related to increased levels of parathyroid hormone. These tumors result from excessive osteoclastic activity. This article presents 3 cases of brown tumor localized in facial bones. The lesions were the result of secondary hyperparathyroidism associated with chronic renal failure. The patients were two 42-year-old men and a 39-year-old woman. All patients had been treated systemically by hemodialysis for more than 10 years. This article highlights the importance of proper diagnosis and management of dental patients presenting with a brown tumor.

  15. Radioimmunoassay of tumor markers in serum of patients with renal carcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Cordoni-Voutsas, M.; Glaubitt, D.; Wagner, W.; Lichtenberg, T.

    1984-01-01

    Having noted an increased serum level of TPA and CEA in patients with renal carcinoma the authors extended these studies by using a larger number of tumor markers. In 15 patients (11 men and 4 women after menopause) aged 33 to 74 years who had renal carcinoma, among them 3 with tumor metastases, the serum concentration of TPA, CA 12-5, CEA, AFP, ferritin, prolactin, ..beta..-HCG, and ..beta../sub 2/-microglobulin was measured by radioimmunoassay. Monoclonal antibodies were used in the determination of serum CA 12-5 and CEA. In all patients surgical treatment, irradiation, or cytostatic therapy had not been performed. In serum the normal range was exceeded by TPA in 7 patients, CA 12-5 in 3, CEA and AFP in one each, ferritin in 12, prolactin in 2, and ..beta../sub 2/-microglobulin in 10 patients. In one man serum prolactin was reduced. Serum ..beta..-HCG was normal in all patients. According to these results serum ferritin, TPA, and ..beta../sub 2/-microglobulin are of great value as tumor markers in patients with renal carcinoma. In several patients the increase of serum ..beta../sub 2/-microglobulin may be ascribed partly to deterioration of renal function. As no consistent patterns of tumor markers in serum were observed it is recommended to determine several tumor markers and not only one of them during the follow-up of patients. Radioimmunoassays for measuring the serum level of tumor markers, especially ferritin, TPA, and ..beta../sub 2/-microglobulin, may considerably assist in the management of patients with renal carcinoma by providing early information about tumor recurrence or metastases.

  16. Radioimmunoassay of tumor markers in serum of patients with renal carcinoma

    International Nuclear Information System (INIS)

    Cordoni-Voutsas, M.; Glaubitt, D.; Wagner, W.; Lichtenberg, T.

    1984-01-01

    Having noted an increased serum level of TPA and CEA in patients with renal carcinoma the authors extended these studies by using a larger number of tumor markers. In 15 patients (11 men and 4 women after menopause) aged 33 to 74 years who had renal carcinoma, among them 3 with tumor metastases, the serum concentration of TPA, CA 12-5, CEA, AFP, ferritin, prolactin, β-HCG, and β/sub 2/-microglobulin was measured by radioimmunoassay. Monoclonal antibodies were used in the determination of serum CA 12-5 and CEA. In all patients surgical treatment, irradiation, or cytostatic therapy had not been performed. In serum the normal range was exceeded by TPA in 7 patients, CA 12-5 in 3, CEA and AFP in one each, ferritin in 12, prolactin in 2, and β/sub 2/-microglobulin in 10 patients. In one man serum prolactin was reduced. Serum β-HCG was normal in all patients. According to these results serum ferritin, TPA, and β/sub 2/-microglobulin are of great value as tumor markers in patients with renal carcinoma. In several patients the increase of serum β/sub 2/-microglobulin may be ascribed partly to deterioration of renal function. As no consistent patterns of tumor markers in serum were observed it is recommended to determine several tumor markers and not only one of them during the follow-up of patients. Radioimmunoassays for measuring the serum level of tumor markers, especially ferritin, TPA, and β/sub 2/-microglobulin, may considerably assist in the management of patients with renal carcinoma by providing early information about tumor recurrence or metastases

  17. Expression patterns of cell cycle components in sporadic and neurofibromatosis type 1-related malignant peripheral nerve sheath tumors

    NARCIS (Netherlands)

    Agesen, Trude Holmeide; Florenes, Viva Ann; Molenaar, Willemina M.; Lind, Guro E.; Berner, Jeane-Marie; Plaat, Boudewijn E.C.; Komdeur, Rudy; Myklebost, Ola; van den Berg, Eva; Lothe, Ragnhild A.

    The molecular biology underlying the development of highly malignant peripheral nerve sheath tumors (MPNSTs) remains mostly unknown. In the present study, the expression pattern of 10 selected cell cycle components is investigated in a series of 15 MPNSTs from patients with (n = 9) or without (n =

  18. Prognostic Factors Influencing the Development of an Iatrogenic Pneumothorax for Computed Tomography-Guided Radiofrequency Ablation of Upper Renal Tumor

    International Nuclear Information System (INIS)

    Park, B.K.; Kim, C.K.

    2008-01-01

    Background: Percutaneous radiofrequency (RF) ablation of upper renal tumors is considered a minimally invasive treatment, but this technique may cause pneumothorax. Purpose: To assess retrospectively the prognostic factors influencing the development of iatrogenic pneumothorax for RF ablation of upper renal tumors. Material and Methods: Computed tomography (CT)-guided RF ablation was performed in 24 patients (21 men, three women; age range 31-77 years, mean age 53.3 years) with 28 upper renal tumors. Various factors for pneumothorax-complicated (PC) upper renal tumors and non-pneumothoracic (NP) upper renal tumors were compared during RF ablation to determine which of the factors were involved in the development of pneumothorax. Results: Among 28 upper renal tumors in 24 patients, a pneumothorax occurred accidentally in six patients with eight tumors and intentionally in two patients with two tumors. This complication was treated with conservative management, instead of tube drainage. PC upper renal tumors had shorter distance from the lung or from the costophrenic line to the tumor, a larger angle between the costophrenic line and the tumor, and a higher incidence of intervening lung tissue than NP upper renal tumors (P<0.01). Intervening lung tissue was more frequently detected on CT images obtained with the patient in the prone position than on CT images obtained with the patient in the supine position. Conclusion: The presence of intervening lung tissue and the close proximity between an upper renal tumor and the lung are high risk factors for developing an iatrogenic pneumothorax. Pre-ablation CT scan should be performed in the prone position to exactly evaluate intervening lung tissue

  19. Radiological classification of renal angiomyolipomas based on 127 tumors

    Directory of Open Access Journals (Sweden)

    Prando Adilson

    2003-01-01

    Full Text Available PURPOSE: Demonstrate radiological findings of 127 angiomyolipomas (AMLs and propose a classification based on the radiological evidence of fat. MATERIALS AND METHODS: The imaging findings of 85 consecutive patients with AMLs: isolated (n = 73, multiple without tuberous sclerosis (TS (n = 4 and multiple with TS (n = 8, were retrospectively reviewed. Eighteen AMLs (14% presented with hemorrhage. All patients were submitted to a dedicated helical CT or magnetic resonance studies. All hemorrhagic and non-hemorrhagic lesions were grouped together since our objective was to analyze the presence of detectable fat. Out of 85 patients, 53 were monitored and 32 were treated surgically due to large perirenal component (n = 13, hemorrhage (n = 11 and impossibility of an adequate preoperative characterization (n = 8. There was not a case of renal cell carcinoma (RCC with fat component in this group of patients. RESULTS: Based on the presence and amount of detectable fat within the lesion, AMLs were classified in 4 distinct radiological patterns: Pattern-I, predominantly fatty (usually less than 2 cm in diameter and intrarenal: 54%; Pattern-II, partially fatty (intrarenal or exophytic: 29%; Pattern-III, minimally fatty (most exophytic and perirenal: 11%; and Pattern-IV, without fat (most exophytic and perirenal: 6%. CONCLUSIONS: This proposed classification might be useful to understand the imaging manifestations of AMLs, their differential diagnosis and determine when further radiological evaluation would be necessary. Small (< 1.5 cm, pattern-I AMLs tend to be intra-renal, homogeneous and predominantly fatty. As they grow they tend to be partially or completely exophytic and heterogeneous (patterns II and III. The rare pattern-IV AMLs, however, can be small or large, intra-renal or exophytic but are always homogeneous and hyperdense mass. Since no renal cell carcinoma was found in our series, from an evidence-based practice, all renal mass with detectable

  20. Spontaneous rupture of renal pelvis secondary to ureteral obstruction by urothelial tumor

    Energy Technology Data Exchange (ETDEWEB)

    Fernandes, Daniel Alvarenga; Palma, Ana Laura Gatti; Kido, Ricardo Yoshio Zanetti; Barros, Ricardo Hoelz de Oliveira; Martins, Daniel Lahan; Penachim, Thiago Jose; Caserta, Nelson Marcio Gomes, E-mail: daniel_alvafer@yahoo.com.br, E-mail: daniel_alvafer@icloud.com [Universidade Estadual de Campinas (UNICAMP), SP (Brazil). Fac. de Medicina. Dept. de Radiologia

    2014-09-15

    Partial spontaneous rupture of the upper urinary tract is rare and usually associated with nephrolithiasis. Other reported causes, apart from instrumentation and trauma, involve obstructive ureteral tumor in the pelvic cavity, retroperitoneal fibrosis, fluid overload, and pregnancy. We report a case of spontaneous rupture of renal pelvis secondary to ureteral obstruction caused by urothelial tumor, clinically suspected and evaluated by CT scans and MRIs, discussing the relevant findings for diagnosis.(author)

  1. Spontaneous rupture of renal pelvis secondary to ureteral obstruction by urothelial tumor

    International Nuclear Information System (INIS)

    Fernandes, Daniel Alvarenga; Palma, Ana Laura Gatti; Kido, Ricardo Yoshio Zanetti; Barros, Ricardo Hoelz de Oliveira; Martins, Daniel Lahan; Penachim, Thiago Jose; Caserta, Nelson Marcio Gomes

    2014-01-01

    Partial spontaneous rupture of the upper urinary tract is rare and usually associated with nephrolithiasis. Other reported causes, apart from instrumentation and trauma, involve obstructive ureteral tumor in the pelvic cavity, retroperitoneal fibrosis, fluid overload, and pregnancy. We report a case of spontaneous rupture of renal pelvis secondary to ureteral obstruction caused by urothelial tumor, clinically suspected and evaluated by CT scans and MRIs, discussing the relevant findings for diagnosis.(author)

  2. Review of renal tumors associated with Birt-Hogg-Dubé syndrome with focus on clinical and pathobiological aspects.

    Science.gov (United States)

    Kuroda, N; Furuya, M; Nagashima, Y; Gotohda, H; Kawakami, F; Moritani, S; Ota, S; Hora, M; Michal, M; Hes, O; Nakatani, Y

    2014-06-01

    Birt-Hogg-Dubé syndrome (BHDS) is an autosomal dominant inherited disorder characterized by clinical features of skin lesions, pulmonary lesions and renal tumor. The gene responsible for this syndrome is located on chromosome 17p11.2 and designated as FLCN. In this article, we review renal tumors associated with BHDS with a focus on clinical and pathobiological aspects. Renal tumors often occur multifocally or bilaterally in the imaging analyses or gross examination. Histological examination of renal tumors includes a variety of subtypes such as hybrid oncocytic tumor, chromophobe renal cell carcinoma (RCC), oncocytoma, clear cell RCC and papillary RCC. The histologic discordance in multiple tumors seems to be characteristic of this syndrome. Oncocytosis is observed histologically in about half of the cases. Several investigations have elucidated that folliculin may be involved in the mammalian target of rapamycin (mTOR) pathway recently. Renal tumors composed of clear cells may behave in an aggressive fashion. However, renal tumors including hybrid oncocytic tumor, chromophobe RCC and oncocytoma behave mostly in an indolent fashion.

  3. Percutaneous Cryoablation of Renal Tumors: Is It Time for a New Paradigm Shift?

    Science.gov (United States)

    Aoun, Hussein D; Littrup, Peter J; Jaber, Mohamed; Memon, Fatima; Adam, Barbara; Krycia, Mark; Prus, Matthew; Heath, Elisabeth; Pontes, Edson

    2017-10-01

    To retrospectively assess long-term outcomes of percutaneous renal cryoablation, including factors affecting complications and local recurrence rates. A total of 357 computed tomographic (CT) fluoroscopy-guided percutaneous cryoablation procedures were performed for 382 masses in 302 outpatients; 347 were biopsy-proven renal-cell carcinoma (RCC) or Bosniak category > III masses (n = 28). Benign pathologic conditions (n = 18) or metastatic non-RCC disease (n = 17) were included to analyze procedural complication rate, but recurrence rates, tumor staging, and nephrometry score were limited to RCCs. The average tumor diameter was 2.9 cm (range, 1-10.3 cm), and median nephrometry score for RCC was 8 (mean, 7.4). Protection of adjacent vital structures was performed in 34% of procedures (n = 121), and ureteral stent placement was performed for 9.2% (n = 33). All major complications were graded per surgical Clavien-Dindo criteria. The average CT-visible cryoablation zone diameter was 5 cm (range, 2.5-10.5 cm). Grade ≥ 3 complications occurred in 2.8% of procedures (n = 10), and appeared related to only high nephrometry scores (P = .0086) and larger tumors (P = .0034). No significant changes in renal function before and after the procedure were noted (P = .18). At a mean follow-up of 31.8 months, the local tumor recurrence rate was 3.2% (11 of 347) for RCC, and no significant difference was noted between tumors larger or smaller than 3 cm (P = .15). The difference reached significance only among the small number of stage ≥ T2 RCC tumors (P = .0039). Long-term follow-up of percutaneous renal cryoablation demonstrates low recurrence rates with preserved renal function, even for patients with high nephrometry scores and body mass index, assuming thorough cytotoxic technique and protection measures. Copyright © 2017 SIR. Published by Elsevier Inc. All rights reserved.

  4. Hyperdiagnostic of renal tumor by intravenous urography in patient with adult polycystic disease

    International Nuclear Information System (INIS)

    Djerassi, R.; Lubomirova, M.; Mutafova, I.; Bogov, B.; Gavrikova, V.; Garvanska, G.

    2005-01-01

    Autosomal dominant polycystic kidney disease (ADPKD) is one of the often seen (from 1:400 to 1:1000) inherited renal diseases with serious prognosis. The exact diagnosis, earlier treatment of the urinary tract infections and hypertension were the steps for prevention of the renal disease progression. The abdominal ultrasound is method used for screening. The frequency of the renal tumors in general population was not higher compared to those in patients with ADPKD. We described and discussed the results obtained by different imaging techniques in 23 years old female with family history for ADPKD. She was admitted to the 'Alexandrovska' University Hospital Nephrology Clinic because of the recurrence of the urinary tract infection. The diagnosis of renal tumor was suspected by renal intravenous pyelography (IVP). All the others imaging techniques - Triplex sonography-B-mode, Color, Pulse, Power Doppler, Tissue Doppler as well as contrast computer tomography showed the polycystic kidney disease, without focal changes, with several small cysts based in the medulla near distal calyces. This was probably the reason for the false-positive image made by IVP. The diagnostic values of the different imaging techniques in making the exact diagnosis in patients with polycystic kidney disease were comment, as well as a peculiar ultrasound image of the polycystic kidney in young patients, aged less then 30 years. To make the correct diagnosis of ADPKD the combination of all known imaging techniques was needed. The small kidney tumors were better visualized by tissue-harmonic ultrasound

  5. Lymphatic spread of mesenchymal renal tumor to metastatic parathymic lymph nodes in rat.

    Science.gov (United States)

    Rozsa, David; Trencsenyi, Gyorgy; Kertai, Pal; Marian, Terez; Nagy, Gabor; Banfalvi, Gasper

    2009-11-01

    Rat mesenchymal renal tumor cells (NeDe) transplanted under the kidney capsule of F344 rats resulted in metastases in the parathymic lymph nodes. Tumor cells were isolated from these tumor-bearing lymph nodes and 106 cells were implanted under the kidney capsule. Tumor growth after this implantation could be traced within six days. India ink was implanted to prove that there is a connection between the lymphatic vessels of the kidney capsule and the parathymic lymph nodes. The distribution of the radioligand 18FDG in different organs also provided evidence that the parathymic lymph nodes are the primary sites of metastatic tumor growth. Tumor growth was followed after staining sections of biopsies of normal, tumorous kidneys and parathymic lymph nodes embedded in paraffin. The progression of tumor formation was seen as a frontline between the healthy and tumor bearing tissue. This demarcation line was sharp at the beginning of the invasion and at the peripheral regions of the tumor, while the central region infiltrated into the healthy kidney tissue. The initial invasion gradually turned to an infiltration resulting in the disruption of the renal tissue, especially at the periphery. Accumulation of lipids and flow of blood to the lymphatic vessels was due to the lack of angiogenesis, leading to an increased pressure of the interstitial fluid. Interstitial damage ultimately led to the appearance of blood and the growth of tumor cells in parathymic lymph nodes. The kidney capsule-parathymic lymph node complex is proposed as a suitable metastatic model for the isolated in vivo examination of tumor development and for the analysis of secondary tumors.

  6. Renal cell tumors with clear cell histology and intact VHL and chromosome 3p: a histological review of tumors from the Cancer Genome Atlas database.

    Science.gov (United States)

    Favazza, Laura; Chitale, Dhananjay A; Barod, Ravi; Rogers, Craig G; Kalyana-Sundaram, Shanker; Palanisamy, Nallasivam; Gupta, Nilesh S; Williamson, Sean R

    2017-11-01

    Clear cell renal cell carcinoma is by far the most common form of kidney cancer; however, a number of histologically similar tumors are now recognized and considered distinct entities. The Cancer Genome Atlas published data set was queried (http://cbioportal.org) for clear cell renal cell carcinoma tumors lacking VHL gene mutation and chromosome 3p loss, for which whole-slide images were reviewed. Of the 418 tumors in the published Cancer Genome Atlas clear cell renal cell carcinoma database, 387 had VHL mutation, copy number loss for chromosome 3p, or both (93%). Of the remaining, 27/31 had whole-slide images for review. One had 3p loss based on karyotype but not sequencing, and three demonstrated VHL promoter hypermethylation. Nine could be reclassified as distinct or emerging entities: translocation renal cell carcinoma (n=3), TCEB1 mutant renal cell carcinoma (n=3), papillary renal cell carcinoma (n=2), and clear cell papillary renal cell carcinoma (n=1). Of the remaining, 6 had other clear cell renal cell carcinoma-associated gene alterations (PBRM1, SMARCA4, BAP1, SETD2), leaving 11 specimens, including 2 high-grade or sarcomatoid renal cell carcinomas and 2 with prominent fibromuscular stroma (not TCEB1 mutant). One of the remaining tumors exhibited gain of chromosome 7 but lacked histological features of papillary renal cell carcinoma. Two tumors previously reported to harbor TFE3 gene fusions also exhibited VHL mutation, chromosome 3p loss, and morphology indistinguishable from clear cell renal cell carcinoma, the significance of which is uncertain. In summary, almost all clear cell renal cell carcinomas harbor VHL mutation, 3p copy number loss, or both. Of tumors with clear cell histology that lack these alterations, a subset can now be reclassified as other entities. Further study will determine whether additional entities exist, based on distinct genetic pathways that may have implications for treatment.

  7. Tumoral calcinosis in a dog with chronic renal failure : clinical communication

    Directory of Open Access Journals (Sweden)

    T.C. Spotswood

    2003-06-01

    Full Text Available A 2-year-old male German shepherd dog in poor bodily condition was evaluated for thoracic limb lameness due to a large, firm mass medial to the left cranial scapula. Radiography revealed several large cauliflower-like mineralized masses in the craniomedial left scapula musculature, pectoral region and bilaterally in the biceps tendon sheaths. Urinalysis, haematology and serum biochemistry showed that the dog was severely anaemic, hyperphosphataemic and in chronic renal failure. The dog was euthanased and a full post mortem performed. A diagnosis of chronic renal failure with secondary hyperparathyroidism was confirmed. The mineralized masses were grossly and histopathologically consistent with a diagnosis of tumoral calcinosis. Tumoral calcinosis associated with chronic renal failure that does not involve the foot pads is rarely seen.

  8. Microwave ablation of renal tumors: A narrative review of technical considerations and clinical results.

    Science.gov (United States)

    Cornelis, F H; Marcelin, C; Bernhard, J-C

    2017-04-01

    The purpose of this review was to identify the specific technical considerations to adequately perform microwave ablations (MWA) of renal tumors and analyze the currently available clinical results. Using Medline, a systematic review was performed including articles published between January 2000 and September 2016. English language original articles, reviews and editorials were selected based on their clinical relevance. MWA has several theoretical advantages over radiofrequency ablation in consistently providing higher intratumoral temperatures. MWA is less dependent of electrical conductivities of tissues and the delivered energy is less limited by desiccation of heated tissues. While there are insufficient data, especially because of a lack of studies with mid- to long-term follow-up, to determine the oncologic effectiveness of MWA, this technique appears safe and effective for the ablation of T1 renal tumors. There is evidence for using mid-level settings based on experimental and clinical data. Power set at 50-65W for 5-15min appears adequate in kidney but close clinical and imaging follow-up have to be performed. Renal MWA offers theoretical advantages by comparison with other available techniques to treat renal tumors. However, MWA suffers of less cumulative data compared to radiofrequency ablation or cryoablation. Moreover, microwaves still require further studies to identify the optimal tumor characteristics and device settings leading to predictable ablation. Copyright © 2016 Éditions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

  9. Tumor infiltrating lymphocyte therapy for ovarian cancer and renal cell carcinoma

    DEFF Research Database (Denmark)

    Andersen, Rikke; Donia, Marco; Westergaard, Marie Christine Wulff

    2015-01-01

    stimulated the interest in developing this approach for other indications. Here, we summarize the early clinical data in the field of adoptive cell transfer therapy (ACT) using tumor-infiltrating lymphocytes for patients with renal cell carcinoma (RCC) and ovarian cancer (OC). In addition we describe...

  10. Tumor-promoting phorbol esters effect alkalinization of canine renal proximal tubular cells

    Energy Technology Data Exchange (ETDEWEB)

    Mellas, J.; Hammerman, M.R.

    1986-03-01

    We have demonstrated the presence of specific receptors for tumor-promoting phorbol esters in the plasma membrane of the canine renal proximal tubular cell. These compounds affect proximal tubular metabolism in vitro. For example, we have shown that they inhibit gluconeogenesis in canine renal proximal tubular segments. Tumor-promoting phorbol esters have been shown to effect alkalinization of non-renal cells, by enhancing Na/sup +/-H/sup +/ exchange across the plasma membrane. To determine whether the actions of tumor-promoting phorbol esters in proximal tubular segments might be mediated by a similar process, we incubated suspensions of segments from dog kidney with these compounds and measured changes in intracellular pH using (/sup 14/C)-5,5-dimethoxazoladine-2-4-dione (DMO) and flow dialysis. Incubation of segments with phorbol 12,13 dibutyrate, but not inactive phorbol ester, 4 ..gamma.. phorbol, effected alkalinization of cells within the segments in a concentration-dependent manner. Alkalinization was dependent upon the presence of extracellular (Na/sup +/) > intracellular (Na/sup +/), was prevented by amiloride and was demonstrable in the presence of SITS. Our findings suggest that tumor-promoting esters stimulate the Na/sup +/-H/sup +/ exchanger known to be present in the brush border membrane of the renal proximal tubular cell. It is possible that the stimulation reflects a mechanism by which phorbol esters affect metabolic processes in these cells.

  11. Brown tumors in patients with chronic renal failure and secondary hyperparathyroidism: Report of 12 cases

    Directory of Open Access Journals (Sweden)

    Fatma Lilia

    2010-01-01

    Full Text Available Brown tumors are unusual but serious complications of renal osteodystrophy. We retrospectively studied 12 patients presenting with chronic renal failure and brown tumor related to secondary hyperparathyroidism. Eleven patients were on chronic hemodialysis. The median duration between renal failure and end stage renal failure was 36 months (range: 12-190 months and the median duration in dialysis for 11 cases: 92 months (range: 72-252 months. The bone pain was noted in all cases (100%, pathological fracture in one case (8% and a palpable bone tumor in 10 cases (83%. Elevated serum Calcium (> 2.35 mmol/L was noted in four cases (33%, elevated serum Phosphate (> 1.78 mmol/L in ten cases (80%, elevated serum Alkaline Phosphate (> 290 UI/L in all cases and intact PTH was > 300 pg/mL in all cases with a serum median rate at 1475 pg/mL (range: 682-3687 pg/L. Subtotal parathyroidectomy was performed in all cases with a resultant decrease in size of brown tumors. We report here patient with CKD with unusual frequency and variable locations. This may be attributed tothe lack of the new calcium free phosphate binders and calcimimetics.

  12. Distinct Cytoplasmic Expression of KL-6 Mucin in Chromophobe Renal Cell Carcinoma: A Comparative Immunohistochemical Study with Other Renal Epithelial Cell Tumors

    International Nuclear Information System (INIS)

    Fukushima, Mana; Higuchi, Kayoko; Shimojo, Hisashi; Uehara, Takeshi; Ota, Hiroyoshi

    2012-01-01

    The presence of cytoplasmic sialyl glycoproteins is a conspicuous feature in chromophobe renal cell carcinoma (RCC). We compared the immunohistochemical expression of sialyl glycoproteins in chromophobe RCC with that in other types of renal tumors. Formalin-fixed, paraffin-embedded tissues of surgically resected renal tumors (chromophobe RCC, 14 cases [10 cases of classic type and 4 cases of eosinophilic variant]; oncocytoma, 7 cases; and clear cell RCC, 9 cases) and kidneys from immature infants (4 cases) were immunostained with antibodies against sialyl glycoproteins (anti-KL-6 and anti-sialyl MUC1 antibodies). Cytoplasmic expression of KL-6 and sialyl MUC1 was distinctive in the chromophobe RCC and renal oncocytoma cells, and in the intercalated cells in collecting duct epithelia. Apical-surface staining of these sialyl glycoproteins was predominantly observed in clear RCC, in the epithelia of the distal tubule and collecting duct, and in the neonatal renal proximal tubule, but not in those of the adult renal proximal tubule. The above-mentioned observations provide additional evidence for similar phenotypic profiles of chromophobe RCC and renal oncocytoma, and the intercalated cells in collecting ducts and the oncofetal expression of sialyl glycoproteins in clear cell RCC. KL-6 is a potential tumor marker for renal tumors

  13. Is percutaneous microwave ablation of liver tumor safe for patients with renal dysfunction

    International Nuclear Information System (INIS)

    Liu Cun; Wang Yang; Yu Xiaoling; Dong Baowei; Zhou Pei; Ren He; Liang Ping

    2011-01-01

    Purpose: To determine the safety of percutaneous microwave ablation of primary and metastatic liver tumor for patients with renal dysfunction. Materials and methods: Fifty primary and metastatic liver tumors in 23 patients with renal dysfunction were retrospectively reviewed at our institution. Renal function was determined by measuring serum creatinine and serum urea before MWA as baseline, within 1 week and at last follow-up. The mean creatinine was 1.69 ± 0.32 mg/dL, 1.71 ± 0.33 mg/dL, and 1.71 ± 0.26 mg/dL respectively, there was not a statistically significant difference between baseline and at last follow-up (P = 0.26). The mean serum urea was 52.52 ± 6.48 mg/dL, 56.55 ± 14.72 mg/dL, and 57.90 ± 16.39 mg/dL respectively, there was not a statistically significant difference between baseline and within 1 week (P = 0.119), between within baseline and at last follow-up (P = 0.090). At the last follow-up examination, all patients had adequately functioning kidneys and did not require any form of renal replacement therapy. This is a small retrospectively study including highly selected patients treated. Therefore, further study should to determine the safety of percutaneous MWA for patients with renal dysfunction in the future. Conclusions: Percutaneous microwave ablation of primary and metastatic liver tumor is no adverse influence on renal function for patients with renal dysfunction in this preliminary series, which can be a minimally invasive alternative therapy.

  14. Is percutaneous microwave ablation of liver tumor safe for patients with renal dysfunction

    Energy Technology Data Exchange (ETDEWEB)

    Liu Cun [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853 (China); Department of Ultrasound, Jinan Central Hospital, Shandong University, 105 Jiefang Xi Road, Jinan, Shandong 250013 (China); Wang Yang; Yu Xiaoling; Dong Baowei; Zhou Pei; Ren He [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853 (China); Liang Ping, E-mail: liangping301@hotmail.com [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing 100853 (China)

    2011-08-15

    Purpose: To determine the safety of percutaneous microwave ablation of primary and metastatic liver tumor for patients with renal dysfunction. Materials and methods: Fifty primary and metastatic liver tumors in 23 patients with renal dysfunction were retrospectively reviewed at our institution. Renal function was determined by measuring serum creatinine and serum urea before MWA as baseline, within 1 week and at last follow-up. The mean creatinine was 1.69 {+-} 0.32 mg/dL, 1.71 {+-} 0.33 mg/dL, and 1.71 {+-} 0.26 mg/dL respectively, there was not a statistically significant difference between baseline and at last follow-up (P = 0.26). The mean serum urea was 52.52 {+-} 6.48 mg/dL, 56.55 {+-} 14.72 mg/dL, and 57.90 {+-} 16.39 mg/dL respectively, there was not a statistically significant difference between baseline and within 1 week (P = 0.119), between within baseline and at last follow-up (P = 0.090). At the last follow-up examination, all patients had adequately functioning kidneys and did not require any form of renal replacement therapy. This is a small retrospectively study including highly selected patients treated. Therefore, further study should to determine the safety of percutaneous MWA for patients with renal dysfunction in the future. Conclusions: Percutaneous microwave ablation of primary and metastatic liver tumor is no adverse influence on renal function for patients with renal dysfunction in this preliminary series, which can be a minimally invasive alternative therapy.

  15. The contemporary role of renal mass biopsy in the management of small renal tumors

    International Nuclear Information System (INIS)

    Lim, Amy; O'Neil, Brock; Heilbrun, Marta E.; Dechet, Christopher; Lowrance, William T.

    2012-01-01

    The selective use of percutaneous biopsy for diagnosis in renal masses is a relatively uncommon approach when compared to the management of other solid neoplasms. With recent advancements in imaging techniques and their widespread use, the incidental discovery of asymptomatic, small renal masses (SRM) is on the rise and a substantial percentage of these SRM are benign. Recent advances in diagnostics have significantly improved accuracy rates of renal mass biopsy (RMB), making it a potentially powerful tool in the management of SRM. In this review, we will discuss the current management of SRM, problems with the traditional view of RMB, improvements in the diagnostic power of RMB, cost-effectiveness of RMB, and risks associated with RMB. RMB may offer important information enabling treating clinicians to better risk-stratify patients and ultimately provide a more personalized treatment approach for SRM.

  16. Renal cell carcinoma as a second malignant neoplasm in a patient with non-syndromic hemihypertrophy and previous Wilms tumor

    Energy Technology Data Exchange (ETDEWEB)

    Kraushaar, Greg; Wiebe, Sheldon [Royal University Hospital, Department of Medical Imaging, Saskatoon (Canada)

    2005-12-01

    Survivors of childhood Wilms tumors are at an increased risk of second malignant neoplasms. Recently, it has been postulated that renal cell carcinoma is among the malignancies for which this population is at risk. We present the unique case of an adult Wilms tumor survivor with non-syndromic hemihypertrophy (NSHH) who developed renal cell carcinoma. This case highlights the need for close follow-up in two populations: adults who have survived Wilms tumor and those with NSHH. (orig.)

  17. Renal cell carcinoma as a second malignant neoplasm in a patient with non-syndromic hemihypertrophy and previous Wilms tumor.

    Science.gov (United States)

    Kraushaar, Greg; Wiebe, Sheldon

    2005-12-01

    Survivors of childhood Wilms tumors are at an increased risk of second malignant neoplasms. Recently, it has been postulated that renal cell carcinoma is among the malignancies for which this population is at risk. We present the unique case of an adult Wilms tumor survivor with non-syndromic hemihypertrophy (NSHH) who developed renal cell carcinoma. This case highlights the need for close follow-up in two populations: adults who have survived Wilms tumor and those with NSHH.

  18. Radiofrequency Ablation of Renal Tumors: Four-Year Follow-Up Results in 47 Patients

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Soo Dong; Yoon, Seong Guk; Sung, Gyung Tak [Dong-A University College of Medicine, Busan (Korea, Republic of)

    2012-09-15

    To retrospectively evaluate the intermediate results of radiofrequency ablation (RFA) of small renal masses (SRMs). Percutaneous or laparoscopic RFA was performed on 48 renal tumors in 47 patients. The follow-up studies included a physical examination, chest radiography, creatinine level, and contrast-enhanced CT or MRI. To confirm the pathologic criteria of complete ablation, 35 patients underwent a follow-up biopsy. Recurrence was defined as contrast enhancement on imaging studies after 3 months, lesion growth at subsequent imaging, or viable cancer cells on follow-up biopsy. Technical success was achieved in 43 (89.6%) of 48 renal tumors. The mean tumor size was 2.3 cm and the mean follow-up period was 49.6 months. Repeated RFA was necessary in 5 tumors due to incomplete ablation. The overall complication rate was 35.8%, of which 96.2% were mild complications. Serum creatinine levels at 12 months after RFA did not differ from those before RFA (1.28 vs. 1.36 mg/dL). Four patients were found to have recurrence at various follow-up intervals, and distant metastasis was not found in any cases. RFA appears to be a useful treatment for selected patients with SRMs. Our 4-year follow-up results disclose an excellent therapeutic outcome with RFA, while achieving effective local tumor control.

  19. Prognostic factors in urothelial renal pelvis and ureter tumors: a multicenter rare cancer network study

    International Nuclear Information System (INIS)

    Ozsahin, M.; Zouhair, A.; Villa, S.; Storme, G.; Chauvet, B.; Taussky, D.; Houtte, P. van; Ries, G.; Bontemps, P.; Coucke, P.; Mirimanoff, R.O.

    1997-01-01

    Purpose: To assess the prognostic factors and the outcome in patients with transitional-cell carcinoma of the renal pelvis and/or ureter. Materials and Methods: A series of 138 patients treated between 1971 and 1996 for transitional-cell carcinoma of the renal pelvis and/or ureter was collected in a retrospective multicenter study of the Rare Cancer Network. Twelve patients with distant metastases were excluded from the statistical evaluation. In the remaining 126 patients, median age was 66 years (range: 41-87). The male to female ratio was 2.5 ((90(36))). All but 3 patients underwent a radical surgery: nephroureterectomy (n = 71), nephroureterectomy and lymphadenectomy (n = 20), nephroureterectomy and partial bladder resection or transurethral resection (n = 20), nephrectomy (n = 8), and ureterectomy (n = 4). There were 6 stage pTa, 22 pT1, 17 pT2, 37 pT3, 37 pT4, and 7 pTx tumors. The pN-stage distribution was as follows: 69 pN0, 8 pN1, 14 pN2, 4 pN3, and 31 pNx. Sixty-one percent (n = 77) of the tumors were located in the renal pelvis, and 21% (n = 27) in the ureter. Renal pelvis and ureter localization was present together in 22 (17%) patients. There were 4 grade 1, 37 grade 2, 42 grade 3 tumors (grade was not registered in 43). Following surgery, microscopic (n = 16) or macroscopic (n = 17) tumor rest was detected in 33 patients. Postoperative radiotherapy was given in 45 (36%) patients with a median total dose of 50 Gy (range: 20-66) in median 25 fractions (range: 4-33). Adjuvant systemic chemotherapy was administered in 12 (10%) patients. The median follow-up period was 39 months (range: 5-220). Results: In a median period of 9 months (range: 1-141), 66% (n = 81) of the patients relapsed (local in 34, locoregional in 7, regional in 16, and distant in 24). The 5- and 10-year overall survival (Kaplan-Meier product-limit estimates) was respectively 29% (± 5) and 19% (± 5) in all patients. In univariate analyses (logrank test), statistically significant

  20. Role of Tumor Location in Selecting Patients for Percutaneous Versus Surgical Cryoablation of Renal Masses

    Science.gov (United States)

    Long, Christopher J; Canter, Daniel J; Smaldone, Marc C; Li, Tianyu; Simhan, Jay; Rozenfeld, Boris; Teper, Ervin; Chen, David YT; Greenberg, Richard E; Viterbo, Rosalia; Uzzo, Robert G; Kutikov, Alexander

    2013-01-01

    Purpose To characterize the relationship between tumor location and choice in selecting surgical cryoablation (SCA) vs. percutaneous cryoablation (PCA) for treatment of renal masses. Materials and Methods MEDLINE search was performed to identify studies in which cryoablation was used as therapy for renal masses. Tumor location was stratified as anterior, posterior, or lateral. Lesions were also described by endophycity (endo-, meso-, or exophytic) and polarity (upper, mid, or lower pole). Treating specialty was stratified as urology, radiology, or both. Comorbidity reporting rates were indexed for each manuscript. Results 37 manuscripts included 2344 lesions treated by SCA or PCA formed the basis for the analysis. Comparing SCA vs. PCA series, anterior/posterior designation was reported in 31 vs. 47% of series; endophycity designation was reported in 17 vs. 40% of series; and polarity designation was reported in 48 vs. 47% of series (all p-values >0.05). Amongst those lesions treated by SCA, 44% were anterior lesions and 28% were posterior, while among PCA-treated lesions 9% were anterior and 81% were posterior. Tumor location description was entirely absent in 32% (14/44) of published series. Conclusions Despite data that tumor location is integral to choice of treatment for renal mass, anatomic tumor descriptors are vastly underreported in the cryotherapy literature. Nearly one third of masses treated with SCA are on the posterior surface of the affected kidney, and may be amenable to PCA, thus avoiding risk of general anesthesia and intraabdominal dissection in comorbid cohorts. Better reporting of objective measures of tumor anatomy and location in cryosurgery literature may facilitate standardization of treatment protocols in patients with renal mass. PMID:23040619

  1. Development and Validity of a Silicone Renal Tumor Model for Robotic Partial Nephrectomy Training.

    Science.gov (United States)

    Monda, Steven M; Weese, Jonathan R; Anderson, Barrett G; Vetter, Joel M; Venkatesh, Ramakrishna; Du, Kefu; Andriole, Gerald L; Figenshau, Robert S

    2018-02-05

    Objectives To provide a training tool to address the technical challenges of robot-assisted laparoscopic partial nephrectomy, we created silicone renal tumor models using 3D printed molds of a patient's kidney with a mass. In this study, we assessed the face, content, and construct validity of these models. Materials and Methods Surgeons of different training levels completed four simulations on silicone renal tumor models. Participants were surveyed on the usefulness and realism of the model as a training tool. Performance was measured using operation specific metrics, self-reported operative demands (NASA TLX), and blinded expert assessment (GEARS). Results 24 participants included attending urologists, endourology fellows, urology residents, and medical students. Post-training surveys of expert participants yielded mean results of 79.2 on the realism of the model's overall feel and 90.2 on the model's overall usefulness for training. Renal artery clamp times and GEARS scores were significantly better in surgeons further in training (p≤0.005, p≤0.025). Renal artery clamp times, preserved renal parenchyma, positive margins, NASA TLX, and GEARS scores were all found to improve across trials (psilicone renal tumor model in a cohort of surgeons of different training levels. Expert participants deemed the model useful and realistic. Surgeons of higher training levels performed better than less experienced surgeons in various study metrics, and improvements within individuals were observed over sequential trials. Future studies should aim to assess model predictive validity, namely the association between model performance improvements and improvements in live surgery. Copyright © 2018. Published by Elsevier Inc.

  2. Perirenal Fat Promotes Renal Arterial Endothelial Dysfunction in Obese Swine through Tumor Necrosis Factor-α.

    Science.gov (United States)

    Ma, Shuangtao; Zhu, Xiang-Yang; Eirin, Alfonso; Woollard, John R; Jordan, Kyra L; Tang, Hui; Lerman, Amir; Lerman, Lilach O

    2016-04-01

    Perirenal fat is associated with poor blood pressure control and chronic kidney disease but the underlying mechanisms remain elusive. We tested the hypothesis that perirenal fat impairs renal arterial endothelial function in pigs with obesity-metabolic derangements. We studied 14 domestic pigs after 16 weeks of a high fat/high fructose diet (obesity-metabolic derangement group) or standard chow (lean group). Renal blood flow, glomerular filtration rate and visceral fat volumes were studied in vivo by computerized tomography. Renal arterial endothelial function was also studied ex vivo in organ baths. Pigs with obesity-metabolic derangements demonstrated increased body weight, blood pressure, cholesterol and intra-abdominal fat compared to lean pigs and perirenal fat volume was significantly larger. Renal blood flow and glomerular filtration rate were markedly elevated while urinary protein level was preserved. Ex vivo acetylcholine induced, endothelium dependent vasodilation of renal artery rings was substantially impaired in pigs with obesity-metabolic derangements compared to lean pigs. Endothelial function was further blunted in obesity-metabolic derangement and lean arterial rings by incubation with perirenal fat harvested from pigs with obesity-metabolic derangements but not from lean pigs. It was restored by inhibiting tumor necrosis factor-α. Perirenal fat from pigs with obesity-metabolic derangements also showed increased pro-inflammatory macrophage infiltration and tumor necrosis factor-α expression. In pigs with obesity-metabolic derangements perirenal fat directly causes renal artery endothelial dysfunction, which is partly mediated by tumor necrosis factor-α. Copyright © 2016 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

  3. Probe retraction during renal tumor cryoablation: a technique to minimize direct ureteral injury.

    Science.gov (United States)

    Froemming, Adam; Atwell, Thomas; Farrell, Michael; Callstrom, Matthew; Leibovich, Bradley; Charboneau, William

    2010-01-01

    Ureteral injury is a well-known complication of radiofrequency ablation of renal tumors. Ureteral injury with cryoablation seems to be less common, but has been reported. Herein, the authors describe a technique that can be used to avoid direct ureteral injury during cryoablation of renal masses. Initial ice ball formation serves to fix the probe in the target. The mass and kidney can then be retracted away from the ureter to avoid direct involvement with the subsequent ice ball. The authors report three successful example cases, with no significant complications.

  4. Comparison of renal function following donor nephrectomy versus radical nephrectomy for renal tumor

    Directory of Open Access Journals (Sweden)

    Mohamed Etafy

    2015-01-01

    Full Text Available In this study, we compared renal function in patients after donor nephrectomy (DN and radical nephrectomy (RN. We retrospectively reviewed 68 patients (mean follow-up 15 months, including 30 patients who had undergone DN and 38 patients who had undergone RN. The study was performed between April 2006 and July 2010 at a single institute. Patients were matched for age and co-morbidities (hypertension and diabetes mellitus. We calculated the estimated glomerular filtration rate (eGFR using the Modification of Diet in Renal Disease study group equation. Parameters studied included GFR (≥60 to 2.0 mg/dL, metabolic acidosis (serum bicarbonate 30 mg. There were no significant demographic differences between the two study groups. After a mean follow-up of 15 months, low eGFR (<60 mL/min/1.73 m 2 was seen in 28% and 6.7% of patients in the RN and DN groups, respectively (P = 0.03. Similarly, proteinuria was seen in 21% vs 0%, P = 0.007, and de novo elevated creatinine was seen in 13% vs 0%, respectively P = 0.04; thus the changes were greater in the RN group. Our study shows that undergoing RN had a significantly greater risk of developing renal insufficiency and proteinuria compared with age-and co-morbidity-matched patients undergoing DN. We concluded that patients undergoing RN show a significantly greater risk of developing renal insufficiency and proteinuria compared with the patients undergoing DN.

  5. Intratumoral peripheral small papillary tufts: a diagnostic clue of renal tumors associated with Birt-Hogg-Dubé syndrome.

    Science.gov (United States)

    Kuroda, Naoto; Furuya, Mitsuko; Nagashima, Yoji; Gotohda, Hiroko; Moritani, Suzuko; Kawakami, Fumi; Imamura, Yoshiaki; Bando, Yoshimi; Takahashi, Masayuki; Kanayama, Hiro-omi; Ota, Satoshi; Michal, Michal; Hes, Ondrej; Nakatani, Yukio

    2014-06-01

    In this article, we searched for the common histologic characteristic of renal tumors in patients with Birt-Hogg-Dubé syndrome (BHDS). We selected 6 patients with histologically confirmed renal tumor in BHDS. Germline FLCN gene mutation has been identified in 5 patients. Multifocality and bilaterality of the renal tumors were pathologically or radiologically confirmed in 5 and 2 cases, respectively. Histologic subtypes of the dominant tumor included 3 previously described hybrid oncocytic tumors, one composite chromophobe/papillary/clear cell renal cell carcinoma (RCC) and one unclassified RCC resembling hybrid chromophobe/clear cell RCC. In one case, chromophobe RCC and clear cell RCC were separately observed. Small papillary lesions located in the peripheral area of the tumor, which we designated as intratumoral peripheral small papillary tufts, were identified in all patients. In conclusion, multifocality/bilaterality of renal tumors, discordance of histologic subtypes, and the presence of intratumoral peripheral small papillary tufts may be important clues to identify BHDS-associated renal tumors. Copyright © 2014 Elsevier Inc. All rights reserved.

  6. Microwave ablation of renal tumors: state of the art and development trends.

    Science.gov (United States)

    Floridi, Chiara; De Bernardi, Irene; Fontana, Federico; Muollo, Alessandra; Ierardi, Anna Maria; Agostini, Andrea; Fonio, Paolo; Squillaci, Ettore; Brunese, Luca; Fugazzola, Carlo; Carrafiello, Gianpaolo

    2014-07-01

    In the last decades an increased incidence of new renal tumor cases has been for clinically localized, small tumors elderly patients, with medical comorbidities whom the risk of surgical complications may pose a greater risk of death than that due to the tumor itself. In these patients, unsuitable for surgical approach, thermal ablation represents a valid alternative to traditional surgery. Thermal ablation is a less invasive, less morbid treatment option thanks to reduced blood loss, lower incidence of complications during the procedure and a less long convalescence. At present, the most widely used thermal ablative techniques are cryoablation, radiofrequency ablation and microwave ablation (MWA). MWA offers many benefits of other ablation techniques and offers several other advantages: higher intratumoral temperatures, larger tumor ablation volumes, faster ablation times, the ability to use multiple applicators simultaneously, optimal heating of cystic masses and tumors close to the vessels and less procedural pain. This review aims to provide the reader with an overview about the state of the art of microwave ablation for renal tumors and to cast a glance on the new development trends of this technique.

  7. Mini-flank supra-12th rib incision for open partial nephrectomy for renal tumor with RENAL nephrometry score ≥10: an innovation of traditional open surgery.

    Science.gov (United States)

    Wang, Hang; Sun, Li-an; Wang, Yiwei; Xiang, Zhuoyi; Zhou, Lin; Guo, Jianming; Wang, Guomin

    2015-04-01

    The skill of supra-12th rib mini-flank approach for open partial nephrectomy (MI-OPN) provides an advanced operative method for renal tumor. Compared with laparoscopic and robotic surgery, it may be a feasible selection for the complex renal tumors. We describe our techniques and results of MI-OPN in complex renal tumors with high RENAL nephrometry score (RENAL nephrometry score ≥10). Fifty-five patients diagnosed with renal tumors between January 2009 and July 2013 were included in this study. Eligibility criteria comprised of patients with complex renal tumor (RENAL score ≥10) being candidates for partial nephrectomy (PN). All patients received MI-OPN and all surgeries were performed by a single urologist. The preoperative workup comprised of medical history, physical examination, and routine laboratory tests. Serum creatinine was recorded preoperatively and 2 to 3 months after operation. Operative time, ischemia time, blood loss, operative and postoperative complications, renal function, and pathology parameters were recorded. MI-OPN was successfully performed in all cases. Mean tumor size was 4.7 cm (range: 2.5-8.1). Mean warm ischemia time was 28.1 minutes (range: 21-39), mean operative time was 105 minutes (range: 70-150) and mean estimated blood loss was 68 mL (range: 10-400). Mean postoperative hospital stay was 6.5 days (range: 5-12). Postoperative complications were found in 3 patients (5.5%). The mean pre- and postoperative serum creatinine levels were 76.2 μmol/L (range: 47-132) and 87.1 μmol/L (range: 61-189) with significant difference (P = 0.004). The mean pre- and postoperative estimated glomerular filtration rate (eGFR) were 91.5 (range: 34-133) and 82.5 (range: 22-126.5), respectively with significant difference (P = 0.024). In an average follow-up of 19.9 months (range: 8-50), no local recurrence or systemic progression occurred. In conclusion, MI-OPN can combine the benefits of both minimal invasive and traditional open

  8. Efficiency and Reliability of Laparoscopic Partial Nephrectomy for Renal Tumors Larger than 4 cm

    Directory of Open Access Journals (Sweden)

    Faruk Özgör

    2015-03-01

    Full Text Available Aim: To evaluate safety and efficiency of laparoscopic partial nephrectomy for renal tumors larger than 4 cm. Methods: We retrospectivelly evaluated the medical records of 65 patients who underwent laparascopic partial nephrectomy between May 2009 and June 2013 in our clinic. The patients were divided into two groups according to tumor size. Patients with a tumor 4 cm were included in group 1 (n=45 and group 2 (n=20, respectively. Demographic, perioperative and postoperative parameters were compared between the groups. Histopathological examination and surgical margin status were also evaluated. Results: The mean age of the patients was 59.2±10.9 (range: 26- 81 years. The mean tumor size and the mean RENAL nephrometry score were significantly higher in group 2 than in group 1. The mean operation time and warm ischemia time were similar between groups but estimated blood loss and transfusion requirement were significantly higher in group 2. Convertion to open surgery was seen two patients in group 2 and one patient in group 1. Only one patient underwent radical nephrectomy for uncontrolled bleeding in group 2. There was no difference in preoperative and 3-month postoperative serum creatinine levels between the groups. The incidence of positive surgical margin was 0% and 5% in group 1 and group 2, respectively. Conclusion: Laparoscopic partial nephrectomy for renal tumors is an effective and feasible procedure with acceptable oncologic results. However, tranfusion rate and requiremet of pelvicaliceal system repair were more common in patients with tumor >4 cm. (The Medical Bulletin of Haseki 2015; 53:30-5

  9. Primary nephrectomy and intraoperative tumor spill: Report from the Children’s Oncology Group (COG) renal tumors committee

    Science.gov (United States)

    Gow, Kenneth W.; Barnhart, Douglas C.; Hamilton, Thomas E.; Kandel, Jessica J.; Chen, Mike K.S.; Ferrer, Fernando A.; Price, Mitchell R.; Mullen, Elizabeth A.; Geller, James I.; Gratias, Eric J.; Rosen, Nancy; Khanna, Geetika; Naranjo, Arlene; Ritchey, Michael L.; Grundy, Paul E.; Dome, Jeffrey S.; Ehrlich, Peter F.

    2015-01-01

    Purpose Initial Children’s Oncology Group (COG) management for Wilms’ tumor (WT) consists of primary nephroureterectomy with lymph node sampling. While this provides accurate staging to define further treatment, it may result in intraoperative spill (IOS), which is associated with higher recurrence rates and therefore requires more intensive therapy. The purpose of this study is to determine current rates and identify factors which may predispose a patient to IOS. Methods The study population was drawn from the AREN03B2 renal tumor banking and classification study of the Children’s Oncology Group. All children with a first time occurrence of a renal mass were eligible for the study. At the time of enrollment and prior to risk stratification, the institution is required to submit operative notes, pathology specimens, a chest computed tomography scan (CT), and a contrast-enhanced CT or magnetic resonance imaging (MRI) of the abdomen and pelvis for central imaging review. These data are then used to determine an initial risk classification and therapeutic protocol eligibility. Patients who had a unilateral nephroureterectomy for favorable histology WT underwent further review to assure data accuracy and to clarify details regarding the spill. Analyses were performed using chi square and logistic regression. Odd ratios (OR) are shown with 95% confidence intervals. Results There were 1,131 primary nephrectomies for unilateral WT with an IOS rate of 9.7% with an additional 1.8% having possible tumor spill during renal vein or IVC tumor thrombectomy. IOS correlated with diameter (>12cm, p<0.0001) and laterality (right, p=0.0414). Simple logistic regression indicated that IOS increased 2.7% [p=0.0240, OR 1.027 (1.004, 1.052)] with each 1 cm increase in diameter (3 –21cm) and 4.7% [p=0.0147 OR 1.047 (1.009, 1.086)] with each 100 g increase in weight (80 – 1800 g). Multiple logistic regression indicated that laterality [right p=0.048, OR 1.46 (1.004, 2.110)] and

  10. A case of a desmoid tumor with an uretertumoral fisttula detected on renal scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Yoo, Hyun Woo; Lee, Kyu Taek; Lee, Sang Mi [Soonchunhyang University Cheonan Hospital, Cheonan (Korea, Republic of)

    2016-11-15

    Desmoid tumors are rare benign tumors with aggressive fibroblastic proliferation. Although desmoid tumors do not metastasize, they have locally aggressive features and can cause a urinary fistula. Here, we report a case of a 35-year-old woman with Gardner syndrome who was diagnosed with an intra-abdominal desmoid tumor 1 year previously and who presented with a newly developed cystic mass lesion on a computed tomography scan. The cystic mass lesion was clinically diagnosed as an urinoma from the right ureterotumoral fistula; thus, surgical resection of the mass lesion was planned. However, Tc-99m diethylenetriamine pentaacetic acid renal scintigraphy revealed bilateral ureterotumoral fistulas; hence, the treatment plan was changed to conservative management.

  11. BHD Tumor Cell Line and Renal Cell Carcinoma Line | NCI Technology Transfer Center | TTC

    Science.gov (United States)

    Scientists at the National Cancer Institute  have developed a novel renal cell carcinoma (RCC) cell line designated UOK257, which was derived from the surgical kidney tissue of a patient with hereditary Birt-Hogg-Dube''''(BHD) syndrome and companion cell line UOK257-2 in which FLCN expression has been restored by lentivirus infection. The NCI Urologic Oncology Branch seeks parties interested in licensing or collaborative research to co-develop, evaluate, or commercialize kidney cancer tumor cell lines.

  12. Percutaneous Renal Tumor Ablation: Radiation Exposure During Cryoablation and Radiofrequency Ablation

    Energy Technology Data Exchange (ETDEWEB)

    McEachen, James C., E-mail: james.mceachen2@gmail.com [Mayo Clinic, Division of Preventive, Occupational, and Aerospace Medicine (United States); Leng, Shuai; Atwell, Thomas D. [Mayo Clinic, Department of Radiology (United States); Tollefson, Matthew K. [Mayo Clinic, Department of Urology (United States); Friese, Jeremy L. [Mayo Clinic, Department of Radiology (United States); Wang, Zhen; Murad, M. Hassan [Mayo Clinic, Division of Preventive, Occupational, and Aerospace Medicine (United States); Schmit, Grant D. [Mayo Clinic, Department of Radiology (United States)

    2016-02-15

    IntroductionOnce reserved solely for non-surgical cases, percutaneous ablation is becoming an increasingly popular treatment option for a wider array of patients with small renal masses and the radiation risk needs to be better defined as this transition continues.Materials and MethodsRetrospective review of our renal tumor ablation database revealed 425 patients who underwent percutaneous ablation for treatment of 455 renal tumors over a 5-year time period. Imparted radiation dose information was reviewed for each procedure and converted to effective patient dose and skin dose using established techniques. Statistical analysis was performed with each ablative technique.ResultsFor the 331 cryoablation procedures, the mean DLP was 6987 mGycm (SD = 2861) resulting in a mean effective dose of 104.7 mSv (SD = 43.5) and the mean CTDI{sub vol} was 558 mGy (SD = 439) resulting in a mean skin dose of 563.2 mGy (SD = 344.1). For the 124 RFA procedures, the mean DLP was 3485 mGycm (SD = 1630) resulting in a mean effective dose of 50.3 mSv (SD = 24.0) and the mean CTDI{sub vol} was 232 mGy (SD = 149) resulting in a mean skin dose of 233.2 mGy (SD = 117.4). The difference in patient radiation exposure between the two renal ablation techniques was statistically significant (p < 0.001).ConclusionBoth cryoablation and RFA imparted an average skin dose that was well below the 2 Gy deterministic threshold for appreciable sequela. Renal tumor cryoablation resulted in a mean skin and effective radiation dose more than twice that for RFA. The radiation exposure for both renal tumor ablation techniques was at the high end of the medical imaging radiation dose spectrum.

  13. Hemorrhagic Complications of Percutaneous Cryoablation for Renal Tumors: Results from a 7-year Prospective Study

    Energy Technology Data Exchange (ETDEWEB)

    Kakarala, Bharat, E-mail: bkakara1@jhmi.edu, E-mail: bharat.kakarala@gmail.com [Johns Hopkins University, Vascular and Interventional Radiology (United States); Frangakis, Constantine E., E-mail: cfrangak@jhsph.edu [Johns Hopkins University, Biostatistics and Epidemiology, Bloomberg School of Public Health (United States); Rodriguez, Ron, E-mail: rodriguezr32@uthscsa.edu [University of Texas Health Science Center, Urologic Surgery (United States); Georgiades, Christos S., E-mail: g-christos@hotmail.com [Johns Hopkins University (United States)

    2016-11-15

    PurposeCryoablation of renal tumors is assumed to have a higher risk of hemorrhagic complications compared to other ablative modalities. Our purpose was to establish the exact risk and to identify hemorrhagic risk factors.Materials and MethodsThis IRB approved, 7-year prospective study included 261 renal cryoablations. Procedures were under conscious sedation and CT guidance. Pre- and postablation CT was obtained, and hemorrhagic complications were CTCAE tabulated. Age, gender, tumor size, histology, and probes number were tested based on averages or proportions using their exact permutation distribution. “High-risk” subgroups (those exceeding the thresholds of all variables) were tested for each variable alone, and for all combinations of variable threshold values. We compared the subgroup with the best PPV using one variable, with the subgroup with the best PPV using all variables (McNemmar test).ResultsThe hemorrhagic complication rate was 3.5 %. Four patients required transfusions, two required emergent angiograms, one required both a transfusion and angiogram, and two required bladder irrigation for outlet obstruction. Perirenal space hemorrhage was more clinically significant than elsewhere. Univariate risks were tumor size >2 cm, number of probes >2, and malignant histology (P = 0.005, 0.002, and 0.033, respectively). Multivariate analysis showed that patients >55 years with malignant tumors >2 cm requiring 2 or more probes yielded the highest PPV (7.5 %).ConclusionsAlthough older patients (>55 years old) with larger (>2 cm), malignant tumors have an increased risk of hemorrhagic complications, the low PPV does not support the routine use of embolization. Percutaneous cryoablation has a 3.5 % risk of significant hemorrhage, similar to that reported for other types of renal ablative modalities.

  14. Hemorrhagic Complications of Percutaneous Cryoablation for Renal Tumors: Results from a 7-year Prospective Study

    International Nuclear Information System (INIS)

    Kakarala, Bharat; Frangakis, Constantine E.; Rodriguez, Ron; Georgiades, Christos S.

    2016-01-01

    PurposeCryoablation of renal tumors is assumed to have a higher risk of hemorrhagic complications compared to other ablative modalities. Our purpose was to establish the exact risk and to identify hemorrhagic risk factors.Materials and MethodsThis IRB approved, 7-year prospective study included 261 renal cryoablations. Procedures were under conscious sedation and CT guidance. Pre- and postablation CT was obtained, and hemorrhagic complications were CTCAE tabulated. Age, gender, tumor size, histology, and probes number were tested based on averages or proportions using their exact permutation distribution. “High-risk” subgroups (those exceeding the thresholds of all variables) were tested for each variable alone, and for all combinations of variable threshold values. We compared the subgroup with the best PPV using one variable, with the subgroup with the best PPV using all variables (McNemmar test).ResultsThe hemorrhagic complication rate was 3.5 %. Four patients required transfusions, two required emergent angiograms, one required both a transfusion and angiogram, and two required bladder irrigation for outlet obstruction. Perirenal space hemorrhage was more clinically significant than elsewhere. Univariate risks were tumor size >2 cm, number of probes >2, and malignant histology (P = 0.005, 0.002, and 0.033, respectively). Multivariate analysis showed that patients >55 years with malignant tumors >2 cm requiring 2 or more probes yielded the highest PPV (7.5 %).ConclusionsAlthough older patients (>55 years old) with larger (>2 cm), malignant tumors have an increased risk of hemorrhagic complications, the low PPV does not support the routine use of embolization. Percutaneous cryoablation has a 3.5 % risk of significant hemorrhage, similar to that reported for other types of renal ablative modalities.

  15. Clinical and Molecular Features of Renal and Pheochromocytoma/Paraganglioma Tumor Association Syndrome (RAPTAS): Case Series and Literature Review.

    Science.gov (United States)

    Casey, Ruth T; Warren, Anne Y; Martin, Jose Ezequiel; Challis, Benjamin G; Rattenberry, Eleanor; Whitworth, James; Andrews, Katrina A; Roberts, Thomas; Clark, Graeme R; West, Hannah; Smith, Philip S; Docquier, France M; Rodger, Fay; Murray, Vicki; Simpson, Helen L; Wallis, Yvonne; Giger, Olivier; Tran, Maxine; Tomkins, Susan; Stewart, Grant D; Park, Soo-Mi; Woodward, Emma R; Maher, Eamonn R

    2017-11-01

    The co-occurrence of pheochromocytoma (PC) and renal tumors was linked to the inherited familial cancer syndrome von Hippel-Lindau (VHL) disease more than six decades ago. Subsequently, other shared genetic causes of predisposition to renal tumors and to PC, paraganglioma (PGL), or head and neck paraganglioma (HNPGL) have been described, but case series of non-VHL-related cases of renal tumor and pheochromocytoma/paraganglioma tumor association syndrome (RAPTAS) are rare. To determine the clinical and molecular features of non-VHL RAPTAS by literature review and characterization of a case series. A review of the literature was performed and a retrospective study of referrals for investigation of genetic causes of RAPTAS. Literature review revealed evidence of an association, in addition to VHL disease, between germline mutations in SDHB, SDHC, SDHD, TMEM127, and MAX genes and RAPTAS [defined here as the co-occurrence of tumors from both classes (PC/PGL/HNPGL and renal tumors) in the same individual or in first-degree relatives]. In both the literature review and our case series of 22 probands with non-VHL RAPTAS, SDHB mutations were the most frequent cause of non-VHL RAPTAS. A genetic cause was identified in 36.3% (8/22) of kindreds. Renal tumors and PC/PGL/HNPGL tumors share common molecular features and their co-occurrence in an individual or family should prompt genetic investigations. We report a case of MAX-associated renal cell carcinoma and confirm the role of TMEM127 mutations with renal cell carcinoma predisposition. Copyright © 2017 Endocrine Society

  16. Prevalence of Baseline Chronic Kidney Disease in Patients Presenting with Solid Renal Tumors

    Science.gov (United States)

    Canter, Daniel; Kutikov, Alexander; Sirohi, Mohit; Street, Ryan; Viterbo, Rosalia; Chen, David YT; Greenberg, Richard E.; Uzzo, Robert G.

    2011-01-01

    Objectives Serum creatinine (sCr) inadequately reflects renal function. Estimated glomerular filtration rate (eGFR) and CKD stage are more clinically relevant parameters to reflect the risk of morbidity and mortality in patients following nephron loss. Comparing sCr and eGFR, we evaluated the prevalence of baseline CKD in a large cohort of patients presenting with renal masses to a tertiary care center. Methods Using the prospectively maintained Fox Chase Kidney Cancer Database, we identified patients undergoing kidney surgery between 1/2000– 5/2010. eGFR was calculated using the Modification of Diet in Renal Disease (MDRD) and the CKDEpidemiology formulas. CKD stages I–V were defined using the National Kidney Foundation definitions. Results 1114 patients had adequate data available to calculate a preoperative eGFR (ml/min). Although 88% of all patients presenting for surgery at our institution had a “normal” baseline sCr (≤1.4 mg/dl), 22% of patients had CKD Stage III or greater. Moreover, of the 282 patients 70 years and older, 40% (113/282) had CKD stage III. 23% (51/220) of patients older than 70 years had a CKD stage III with a seemingly normal sCr. Conclusions Many patients with a normal sCr have CKD III or higher, particularly patients older 70 years old. Given the high prevalence of baseline CKD in patients with a solid renal tumor, a concerted effort must be made to preserve renal function when surgically treating solid renal masses. PMID:21316090

  17. Evaluation of Renal Function in Pediatric Patients After Treatment for Wilms' Tumor.

    Science.gov (United States)

    Janeczko, Małgorzata; Niedzielska, Ewa; Pietras, Wojciech

    2015-01-01

    Wilms' tumor is the most common kidney cancer in children. Treatment consists of pre- and post-operative chemotherapy, surgery and in some cases radiotherapy. The treatment of nephroblastomas is very effective. Hence, the population of adult patients cured of this cancer in their childhood is steadily growing, generating a need for long-term health assessment, including renal function, due to the specifications of the therapy and the location of the tumor. The aim of the study was to evaluate nephrological complications after treatment for nephroblastoma. The study group consisted of 50 children treated in the Department of Pediatric Hematology, Oncology and Bone Marrow Transplantation at Wroclaw Medical University (Poland) from 2002 to 2012. An analysis of the patients' medical histories was carried out. The glomerular filtration rate estimated by the Schwartz formula (GFR by Schwartz), serum creatinine levels, urea and electrolyte concentrations; the results of urinalysis and blood pressure were assessed. Each of these analyses was performed at the time of diagnosis, at the end of therapy, as well as 6 months, one year and two years after its completion. The study showed that, in most cases, implemented therapy had no significant impact on the deterioration of renal parameters in the two-year period following treatment for Wilms' tumor. However, the group of patients treated with cyclophosphamide and carboplatin required more careful monitoring, due to a higher risk of renal function deterioration. The study shows that the problem of nephrotoxicity after treatment for Wilms' tumor is more frequent than indicated in other studies; however, the deterioration of kidney function in most cases is not serious. Additional attention should be paid to patients treated with cyclophosphamide and carboplatin. Assessment of the early and late effects of the treatment is a key element in improving the quality of the patients' life.

  18. microRNA-184 functions as tumor suppressor in renal cell carcinoma.

    Science.gov (United States)

    Su, Zhengming; Chen, Duqun; Li, Yifan; Zhang, Enpu; Yu, Zuhu; Chen, Ting; Jiang, Zhimao; Ni, Liangchao; Yang, Shangqi; Gui, Yaoting; Ye, Jiongxian; Lai, Yongqing

    2015-03-01

    microRNAs (miRNAs) are evolutionarily conserved, endogenous, small, noncoding RNA molecules of approximately 22 nucleotides in length that function as post-transcriptional gene regulators. Their aberrant expression may be involved in human diseases, including cancer. Although miRNA-184 (miR-184) has been reported in other tumors, its function in renal cell carcinoma (RCC) is still unknown. The aim of the present study was to investigate the role of miR-184 in RCC. The impacts of miR-184 on cell migration, proliferation and apoptosis were evaluated using migration scratch, 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) and flow cytometry assay. Our studies revealed that miR-184 mimic significantly inhibits cell migration, suppresses cell proliferation and induces renal cancer cell apoptosis in vitro when compared with the negative control (P184 played a significant role as a tumor suppressor in RCC. Therefore, miR-184 may be a promising therapeutic target for renal cancer treatment in the future.

  19. Bilateral renal tumors in an adult man with Smith-Magenis syndrome: The role of the FLCN gene.

    Science.gov (United States)

    Dardour, Leila; Verleyen, Pieter; Lesage, Karl; Holvoet, Maureen; Devriendt, Koen

    2016-10-01

    Smith-Magenis syndrome (SMS) is a contiguous-gene disorder most commonly caused by a deletion of chromosome 17p11.2. We report a 57 year-old man with SMS who presents bilateral renal tumors. This is most likely related to haploinsufficiency of FLCN gene, located in the deleted region, and a known tumor suppressor gene. Haploinsufficiency of FLCN causes Birt-Hogg-Dubé syndrome (BHDS), characterized by pulmonary cysts, renal and skin tumors. The present observation suggests that the follow-up of patients with SMS should also focus on possible manifestations of BHDS. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  20. Termoablación con radiofrecuencia de tumores del parenquima renal

    OpenAIRE

    Calvo González, Raúl

    2015-01-01

    Evaluar la técnica de Termoablación mediante Radiofrecuencia (RFA) como opción terapéutica para el tratamiento de masas renales de pequeño tamaño. Evaluar la técnica Termoablación mediante Radiofrecuencia en términos de eficacia oncológica. Evaluación del protocolo de seguimiento posterior a la aplicación de técnicas ablativas para el tratamiento de tumores del parénquima renal. Evaluar el perfil de seguridad de la técnica de Termoablación mediante Radiofrecuencia determinando las c...

  1. Differential diagnosis of renal tumors with tubulopapillary architecture in children and young adults: a case report and review of literature.

    Science.gov (United States)

    Chen, Longwen; Deng, Fang-Ming; Melamed, Jonathan; Zhou, Ming

    2014-01-01

    Tumors of the kidney are uncommon in children and young adults. Accurate classification is crucial for both prognostication and therapeutic intervention. However, majority of the tumors in this age group have unusual morphology that renders classification challenging. Tubulopapillary architecture is one of the most common morphological patterns observed in renal tumors of children and young adults. A patient with epithelial predominant Wilms tumor was reported. Differential diagnosis of renal tumors with tubulopapillary morphology was discussed with an emphasis on the histological and immunohistochemical features, and the literature was reviewed. A 25 year-old female patient presented with bilateral multilocular cystic masses. She underwent right radical nephrectomy and left partial nephrectomy. The pathological examination revealed a tumor with tubulopapillary architecture which was lined with low columnar epithelial cells. During the work-up of this case, several entities were considered and ruled out by careful gross, microscopic examination and prudent use of immunohistochemistry. The tumor cells were positive for WT-1, and variably positive for cytokeratin AE1/3, CD56, CD57, and negative for cytokeratin 7 and EMA. Fluorescent in-situ hybridization revealed no gain of chromosome 7 and 17. A diagnosis of epithelial predominant adult Wilms tumors was rendered for both kidneys. The patient received systemic chemotherapy and radiation to the remnant left kidney and was free of disease three years after the initial surgery. The differential diagnosis of renal tumors with tubulopapillary features in children and young adults include papillary renal cell carcinoma, metanephric adenoma, epithelial predominant Wilms tumor, translocation renal cell carcinoma and metastatic adenocarcinoma to the kidney. An accurate classification relies on careful examination of clinical and pathological features and immunohistochemical characteristics.

  2. Haploidentical hematopoietic SCT increases graft-versus-tumor effect against renal cell carcinoma.

    Science.gov (United States)

    Budak-Alpdogan, T; Sauter, C T; Bailey, C P; Biswas, C S; Panis, M M; Civriz, S; Flomenberg, N; Alpdogan, O

    2013-08-01

    Allogeneic hematopoietic SCT (HSCT) has been shown to be an effective treatment option for advanced renal cell cancer (RCC). However, tumor resistance/relapse remains as the main post transplant issue. Therefore, enhancing graft-versus-tumor (GVT) activity without increasing GVHD is critical for improving the outcome of HSCT. We explored the GVT effect of haploidentical-SCT (haplo-SCT) against RCC in murine models. Lethally irradiated CB6F1 (H2K(b/d)) recipients were transplanted with T-cell-depleted BM cells from B6CBAF1 (H2K(b/k)) mice. Haplo-SCT combined with a low-dose haploidentical (HI) T-cell infusion (1 × 10(5)) successfully provided GVT activity without incurring GVHD. This effect elicited murine RCC growth control and consequently displayed a comparative survival advantage of haplo-SCT recipients when compared with MHC-matched (B6D2F1CB6F1) and parent-F1 (B6CB6F1) transplant recipients. Recipients of haplo-SCT had an increase in donor-derived splenic T-cell numbers, T-cell proliferation and IFN-γ-secreting donor-derived T-cells, a critical aspect for anti-tumor activity. The splenocytes from B6CBAF1 mice had a higher cytotoxicity against RENCA cells than the splenocytes from B6 and B6D2F1 donors after tumor challenge. These findings suggest that haplo-SCT might be an innovative immunotherapeutic platform for solid tumors, particularly for renal cell carcinoma.

  3. Evaluation of three-dimensional printing for laparoscopic partial nephrectomy of renal tumors: a preliminary report.

    Science.gov (United States)

    Zhang, Yi; Ge, Hong-wei; Li, Ning-chen; Yu, Cheng-fan; Guo, Hong-feng; Jin, Shi-hua; Liu, Jin-shun; Na, Yan-qun

    2016-04-01

    To investigate the impact of three-dimensional (3D) printing on the surgical planning, potential of training and patients' comprehension of minimally invasive surgery for renal tumors. Patients of a T1N0M0 single renal tumor and indicated for laparoscopic partial nephrectomy were selected. CT data were sent for post-processing and output to the 3D printer to create kidney models with tumor. By presenting to experienced laparoscopic urologists and patients, respectively, the models' realism, effectiveness for surgical planning and training, and patients' comprehension of disease and procedure were evaluated with plotted questionnaires (10-point rating scales, 1-not at all useful/not at all realistic/poor, 10-very useful/very realistic/excellent). The size of resected tumors was compared with that on the models. Ten kidney models of such patients were fabricated successfully. The overall effectiveness in surgical planning and training (7.8 ± 0.7-8.0 ± 1.1), and realism (6.0 ± 0.6-7.8 ± 1.0) were reached by four invited urologists. Intraoperative correlation was advocated by the two performing urologists. Patients were fascinated with the demonstration of a tactile "diseased organ" (average ≥ 9.0). The size deviation was 3.4 ± 1.3 mm. Generating kidney models of T1N0M0 tumors with 3D printing are feasible with refinements to be performed. Face and content validity was obtained when those models were presented to experienced urologists for making practical planning and training. Understandings of the disease and procedure from patients were well appreciated with this novel technology.

  4. Association between residential proximity to environmental pollution sources and childhood renal tumors.

    Science.gov (United States)

    García-Pérez, Javier; Morales-Piga, Antonio; Gómez, José; Gómez-Barroso, Diana; Tamayo-Uria, Ibon; Pardo Romaguera, Elena; Fernández-Navarro, Pablo; López-Abente, Gonzalo; Ramis, Rebeca

    2016-05-01

    Few risk factors for childhood renal tumors are well established. While a small fraction of cases might be attributable to susceptibility genes and congenital anomalies, the role of environmental factors needs to be assessed. To explore the possible association between residential proximity to environmental pollution sources (industrial and urban areas, and agricultural crops) and childhood renal cancer, taking into account industrial groups and toxic substances released. We conducted a population-based case-control study of childhood renal cancer in Spain, including 213 incident cases gathered from the Spanish Registry of Childhood Tumors (period 1996-2011), and 1278 controls individually matched by year of birth, sex, and region of residence. Distances were computed from the respective subject's residences to the 1271 industries, the 30 urban areas with ≥75,000 inhabitants, and the agricultural crops located in the study area. Using logistic regression, odds ratios (ORs) and 95% confidence intervals (95%CIs) for categories of distance to pollution sources were calculated, with adjustment for matching variables and socioeconomic confounders. Excess risk (OR; 95%CI) of childhood renal tumors was observed for children living near (≤2.5km) industrial installations as a whole (1.97; 1.13-3.42) - particularly glass and mineral fibers (2.69; 1.19-6.08), galvanization (2.66; 1.14-6.22), hazardous waste (2.59; 1.25-5.37), ceramic (2.35; 1.06-5.21), surface treatment of metals (2.25; 1.24-4.08), organic chemical industry (2.22; 1.15-4.26), food and beverage sector (2.19; 1.18-4.07), urban and waste-water treatment plants (2.14; 1.07-4.30), and production and processing of metals (1.98; 1.03-3.82) -, and in the proximity of agricultural crops (3.16; 1.54-8.89 for children with percentage of crop surface ≥24.35% in a 1-km buffer around their residences). Our study provides some epidemiological evidence that living near certain industrial areas and agricultural crops

  5. Renal Functional Outcome of Partial Nephrectomy for Complex R.E.N.A.L. Score Tumors With or Without Neoadjuvant Sunitinib: A Multicenter Analysis.

    Science.gov (United States)

    McDonald, Michelle L; Lane, Brian R; Jimenez, Juan; Lee, Hak J; Yim, Kendrick; Bindayi, Ahmet; Hamilton, Zachary A; Field, Charles A; Bloch, Aaron S; Dey, Sumi; Noyes, Sabrina; McKay, Rana; Millard, Frederick; Rini, Brian I; Campbell, Steven C; Derweesh, Ithaar H

    2018-04-01

    Sunitinib might optimize the feasibility of partial nephrectomy (PN) for complex renal tumors with imperative indications. We compared the renal functional outcomes of patients with complex renal masses who had undergone sunitinib before PN with those of patients who had not required neoadjuvant sunitinib before PN. We performed a multicenter retrospective analysis of patients with renal cell carcinoma who had undergone PN for a complex renal mass (R.E.N.A.L. nephrometry score, 10-12) and imperative indications from January 2012 to July 2014. Neoadjuvant sunitinib was used in cases for which PN was not considered feasible. The cohort was divided into those patients who had undergone PN without neoadjuvant sunitinib and those who had undergone PN after sunitinib (no-neoadjuvant vs. neoadjuvant). The change in tumor size and R.E.N.A.L. score were assessed. The primary outcome was the change in the estimated glomerular filtration rate (ΔeGFR) from preoperatively to the last postoperative follow-up visit. The data from 125 consecutive patients were analyzed (47 neoadjuvant and 78 no-neoadjuvant; median follow-up, 21 months). The neoadjuvant plus PN patients had had a greater median tumor size preoperatively (7.2 vs. 6 cm; P = .045). Sunitinib caused a significant decrease in the median tumor size (from 7.2 to 5.8 cm [19.4%]; P = .012) and R.E.N.A.L. score (from 11 to 9; P = .001). No significant differences were found between the neoadjuvant and no-neoadjuvant groups in the ischemia time (P = .413) or incidence of complications (P = .728). The median ΔeGFR was similar (neoadjuvant, 6.4; no-neoadjuvant, 6.1; P = .534). Linear regression analysis for factors associated with an increasing ΔeGFR demonstrated increasing age (estimate, -0.074; P = .009) increasing body mass index (estimate, -0.087; P = .043), and decreasing baseline eGFR (estimate, -0.104; P = .02) as significant factors. The use of neoadjuvant sunitinib might facilitate complex PN and result

  6. Skull Base Clear Cell Carcinoma, Metastasis of Renal Primary Tumor: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Ilson Sepúlveda

    2013-08-01

    Full Text Available We report on a patient who presented with cranial nerve VI bilateral paresis, absence of pharyngeal reflex, dysarthria, right tongue deviation, and right facial paralysis. Imaging studies showed an expansive process in the cranial base with clivus and petrous apex osteolysis. A biopsy confirmed the presence of clear cell adenocarcinoma and suspicion of renal tumor metastases. Abdominal imaging studies revealed a mass in the right kidney. Consequently, radiotherapy was performed, and the patient was enrolled in a palliative care and pain control program.

  7. The metastatic potential of renal tumors: Influence of histologic subtypes on definition of small renal masses, risk stratification, and future active surveillance protocols.

    Science.gov (United States)

    Daugherty, Michael; Sedaghatpour, Dillon; Shapiro, Oleg; Vourganti, Srinivas; Kutikov, Alexander; Bratslavsky, Gennady

    2017-04-01

    The influence of histology in metastatic potential is often overlooked when discussing the management options of small renal masses (SRM), with size or growth rate often serving as the triggers for the intervention. We aim to re-examine the definition of a SRM by evaluating the metastatic potential of renal masses incorporating tumor size and histology to create metastatic risk tables. Surveillance Epidemiology and End Results (SEER)-18 registries database was queried for all cases of clear cell, papillary, and chromophobe renal cell carcinoma (RCC) diagnosed between 2004 and 2012. There were 55,478 cases identified that included 43,783, 8,587, and 3,208 cases of clear cell, papillary, and chromophobe, respectively. Tumors were stratified using 1-cm increments to determine the metastatic potential by calculating the metastatic rate at presentation for different size intervals in histologic categories. For all 3 histologies, tumors measuring 5cm or less had a rate of metastatic RCC at presentation of less than 4%. The metastatic potential was highest for clear cell, followed by papillary and then chromophobe tumors. Setting a cutoff of no more than 3% for metastatic potential to be called a SRM, makes clear cell carcinoma and papillary carcinoma a SRM up to 4cm, whereas the chromophobe RCC would be considered a SRM up to 7cm. Although clinical staging and tumor size have been the key determinants in decision-making of patients with solid renal tumors, the histology-specific risks of metastatic potential are different for each mass. The definition of a SRM should be based on the metastatic potential and not on tumor size alone. This information could be helpful for counseling and managing patients with SRMs as well as for modifying active surveillance protocols. Copyright © 2017 Elsevier Inc. All rights reserved.

  8. Modeling Renal Cell Carcinoma in Mice: Bap1 and Pbrm1 Inactivation Drive Tumor Grade.

    Science.gov (United States)

    Gu, Yi-Feng; Cohn, Shannon; Christie, Alana; McKenzie, Tiffani; Wolff, Nicholas; Do, Quyen N; Madhuranthakam, Ananth J; Pedrosa, Ivan; Wang, Tao; Dey, Anwesha; Busslinger, Meinrad; Xie, Xian-Jin; Hammer, Robert E; McKay, Renée M; Kapur, Payal; Brugarolas, James

    2017-08-01

    Clear cell renal cell carcinoma (ccRCC) is characterized by BAP1 and PBRM1 mutations, which are associated with tumors of different grade and prognosis. However, whether BAP1 and PBRM1 loss causes ccRCC and determines tumor grade is unclear. We conditionally targeted Bap1 and Pbrm1 (with Vhl ) in the mouse using several Cre drivers. Sglt2 and Villin proximal convoluted tubule drivers failed to cause tumorigenesis, challenging the conventional notion of ccRCC origins. In contrast, targeting with PAX8, a transcription factor frequently overexpressed in ccRCC, led to ccRCC of different grades. Bap1 -deficient tumors were of high grade and showed greater mTORC1 activation than Pbrm1 -deficient tumors, which exhibited longer latency. Disrupting one allele of the mTORC1 negative regulator, Tsc1 , in Pbrm1 -deficient kidneys triggered higher grade ccRCC. This study establishes Bap1 and Pbrm1 as lineage-specific drivers of ccRCC and histologic grade, implicates mTORC1 as a tumor grade rheostat, and suggests that ccRCCs arise from Bowman capsule cells. Significance: Determinants of tumor grade and aggressiveness across cancer types are poorly understood. Using ccRCC as a model, we show that Bap1 and Pbrm1 loss drives tumor grade. Furthermore, we show that the conversion from low grade to high grade can be promoted by activation of mTORC1. Cancer Discov; 7(8); 900-17. ©2017 AACR. See related commentary by Leung and Kim, p. 802 This article is highlighted in the In This Issue feature, p. 783 . ©2017 American Association for Cancer Research.

  9. Tc-MDP bone scintigraphy in a case with sporodical tumoral calcinosis

    International Nuclear Information System (INIS)

    Guveli, Tulay Kacar; Mulazimoglu, Mehmet; Tamam, Muge Oner; Tatoglu, Tarik; Ozpacaci, Tevfik; Tamam, Cueneyt

    2010-01-01

    Tumoral calcinosis is an uncommon and benign condition characterized by the presence of large calcific soft tissue deposits occurring predominantly in a periarticular location. It generally occurs as a complication of renal dialysis or trauma, and is rarely seen in familial and sporadic cases. Bone scintigraphy is a sensitive method for diagnosing tumoral calcinosis. A 28-year-old female patient with a history of operation due to tumoral calcinosis located bilateral hips, referred to our department. She had a tender palpable mass in the right knee and a fistulized incisional scar overlying the bilateral hip joints. A sporadic case of tumoral calcinosis with relapses was presented. (author)

  10. Assessing performance trends in laparoscopic nephrectomy and nephron sparing surgery for localized renal tumors

    Science.gov (United States)

    Smaldone, Marc C.; Kutikov, Alexander; Egleston, Brian; Simhan, Jay; Canter, Daniel J.; Teper, Ervin; Viterbo, Rosalia; Chen, David Y.T.; Greenberg, Richard E.; Uzzo, Robert G.

    2012-01-01

    Objective To assess the impact of laparoscopy on utilization of partial nephrectomy (PN) by comparing national utilization trends in patients undergoing surgery for localized renal tumors. Methods Using linked Surveillance, Epidemiology, and End Results (SEER)-Medicare data, we retrospectively examined trends in procedure utilization from 1995–2007 for patients undergoing surgery for localized (stage I/II) renal masses. Procedures were classified as open radical nephrectomy (ORN), laparoscopic radical nephrectomy (LRN), open partial nephrectomy (OPN), and laparoscopic partial nephrectomy (LPN). Patients were further stratified by tumor size (≤4cm, >4 to ≤7cm, >7cm). Data were primarily analyzed using logistic regressions. Results 11,689 patients (mean age 74.4±5.7 years, 56% male) with a mean tumor size of 4.7±3.3cm met inclusion criteria. From 1995–2007, ORN rates decreased while for each year successive year, patients were more likely to be treated with OPN (OR 1.17, CI 1.14–1.19), LRN (OR 1.44, CI 1.41–1.47) and LPN (OR 1.75, CI 1.68–1.83). While the increased utilization of OPN (7.5% vs. 13.6%, p<0.001) and LPN (0% vs. 14.2%, p<0.001) reached statistical significance, this was offset by a marked increase in LRN over the same time period (3.0% vs. 43.0%, p<0.001). Conclusions Despite increasing emphasis on nephron preservation, PN utilization rates remain low. Compared to a 40% increase in LRN, utilization of PN increased by only 20% from 1995–2007. As a result, 72% of identified Medicare beneficiaries with localized tumors were managed with RN in 2007. The trade-off of minimally invasive surgery for nephron preservation may have adverse long term consequences. PMID:22704174

  11. Understanding Treatment Disconnect and Mortality Trends in Renal Cell Carcinoma Using Tumor Registry Data.

    Science.gov (United States)

    Smaldone, Marc C; Egleston, Brian; Hollingsworth, John M; Hollenbeck, Brent K; Miller, David C; Morgan, Todd M; Kim, Simon P; Malhotra, Aseem; Handorf, Elizabeth; Wong, Yu-Ning; Uzzo, Robert G; Kutikov, Alexander

    2017-04-01

    The paradoxical rise in overall and cancer-specific mortality despite increased detection and treatment of renal cell carcinoma (RCC) is termed "treatment disconnect." We reassess this phenomenon by evaluating impact of missing data and rising incidence on mortality trends. Using Surveillance, Epidemiology, and End Results data, we identified patients with RCC diagnosis from 1973 to 2011. We estimated mortality rates by tumor size after accounting for lags from diagnosis to death using multiple imputations for missing data from 1983. Mortality rates were estimated irrespective of tumor size after adjustment for prior cumulative incidence using ridge regression. A total of 78,891 patients met inclusion criteria. Of 70,212 patients diagnosed since 1983, 10.4% had missing data. Significant attenuation in cancer-specific mortality was noted from 1983 to 2011 when comparing observed with imputed rates: Δobs0.05 versus Δimp0.10 (P=0.001, 7 cm tumors). Holding incidence of RCC constant to 2011 rates, temporal increase in overall mortality for all patients was attenuated (Pmortality trends. These findings were supported by assessment of mortality to incidence ratio trends. Missing data and rising incidence may contribute substantially to the "treatment disconnect" phenomenon when examining mortality rates in RCC using tumor registry data. Caution is advised when basing clinical and policy decisions on these data.

  12. Supra-Acetabular Brown Tumor due to Primary Hyperparathyroidism Associated with Chronic Renal Failure

    Directory of Open Access Journals (Sweden)

    Rosaria M. Ruggeri

    2010-01-01

    Full Text Available A 63-year-old woman presented to the Orthopedic Unit of our hospital complaining of right hip pain of 6 months'duration associated with a worsening limp. Her past medical history included chronic renal insufficiency. Physical examination revealed deep pain in the iliac region and severe restriction of the right hip's articular function in the maximum degrees of range of motion. X-rays and CT scan detected an osteolytic and expansive lesion of the right supra-acetabular region with structural reabsorption of the right iliac wing. 99mTc-MDP whole-body bone scan showed an abnormal uptake in the right iliac region. Bone biopsy revealed an osteolytic lesion with multinucleated giant cells, indicating a brown tumor. Serum intact PTH was elevated (1020 pg/ml; normal values, 12 62 pg/ml, but her serum calcium was normal (total = 9.4 mg/dl, nv 8.5–10.5; ionized = 5.0 mg/dl, nv 4.2–5.4 due to the coexistence of chronic renal failure. 99mTc-MIBI scintigraphy revealed a single focus of sestamibi accumulation in the left retrosternal location, which turned out to be an intrathoracic parathyroid adenoma at surgical exploration. After surgical removal of the parathyroid adenoma, PTH levels decreased to 212 pg/ml. Three months after parathyroidectomy, the imaging studies showed complete recovery of the osteolytic lesion, thus avoiding any orthopedic surgery. This case is noteworthy because (1 primary hyperparathyroidism was not suspected due to the normocalcemia, likely attributable to the coexistence of chronic renal failure; and (2 it was associated with a brown tumor of unusual location (right supra-acetabular region.

  13. Microsatellite alteration and immunohistochemical expression profile of chromosome 9p21 in patients with sporadic renal cell carcinoma following surgical resection

    International Nuclear Information System (INIS)

    El-Mokadem, Ismail; Lim, Alison; Kidd, Thomas; Garret, Katherine; Pratt, Norman; Batty, David; Fleming, Stewart; Nabi, Ghulam

    2016-01-01

    Long-term prognostic significance of loss of heterozygosity on chromosome 9p21 for localized renal cell carcinoma following surgery remains unreported. The study assessed the frequency of deletions of different loci of chromosome 9p along with immunohistochemical profile of proteins in surgically resected renal cancer tissue and correlated this with long-term outcomes. DNA was extracted from renal tumours and corresponding normal kidney tissues in prospectively collected samples of 108 patients who underwent surgical resection for clinically localized disease between January 2001 and December 2005, providing a minimum of 9 years follow-up for each participant. After checking quality of DNA, amplified by PCR, loss of heterozygosity (LOH) on chromosome 9p was assessed using 6 microsatellite markers in 77 clear cell carcinoma. Only 5 of the markers showed LOH (D9S1814, D9S916, D9S974, D9S942, and D9S171). Protein expression of p15(INK4b), p16(INK4a), p14(ARF), CAIX, and adipose related protein (ADFP) were demonstrated by immunostaining in normal and cancer tissues. Loss of heterozygosity for microsatellite analysis was correlated with tumour characteristics, recurrence free, cancer specific, and overall survival, including significance of immunohistochemical profile of protein expressions. The main deletion was found at loci telomeric to CDKN2A region at D9S916. There was a significant correlation between frequency of LOH stage (p = 0.005) and metastases (p = 0.006) suggesting a higher LOH for advanced and aggressive renal cell carcinoma. Most commonly observed LOH in the 3 markers: D9S916, D9S974, and D9S942 were associated with poor survival, and were statistically significant on multivariate analysis. Immunohistochemical expression of p14, p15, and p16 proteins were either low or absent in cancer tissue compared to normal. Loss of heterozygosity of p921 chromosome is associated with aggressive tumours, and predicts cancer specific or recurrence free survival on

  14. Renal Tumor Cryoablation Planning. The Efficiency of Simulation on Reconstructed 3D CT Scan

    Directory of Open Access Journals (Sweden)

    Ciprian Valerian LUCAN

    2010-12-01

    Full Text Available Introduction & Objective: Nephron-sparing surgical techniques risks are related to tumor relationships with adjacent anatomic structures. Complexity of the renal anatomy drives the interest to develop tools for 3D reconstruction and surgery simulation. The aim of the article was to assess the simulation on reconstructed 3D CT scan used for planning the cryoablation. Material & Method: A prospective randomized study was performed between Jan. 2007 and July 2009 on 27 patients who underwent retroperitoneoscopic T1a renal tumors cryoablation (RC. All patients were assessed preoperatively by CT scan, also used for 3D volume rendering. In the Gr.A, the patients underwent surgery planning by simulation on 3D CT scan. In the Gr.B., patients underwent standard RC. The two groups were compared in terms of surgical time, bleeding, postoperative drainage, analgesics requirement, hospital stay, time to socio-professional reintegration. Results: Fourteen patients underwent preoperative cryoablation planning (Gr.A and 13 patients underwent standard CR (Gr.B. All parameters analyzed were shorter in the Gr.A. On multivariate logistic regression, only shortens of the surgical time (138.79±5.51 min. in Gr.A. vs. 140.92±5.54 min in Gr.B. and bleeding (164.29±60.22 mL in Gr.A. vs. 215.38±100.80 mL in Gr.B. achieved statistical significance (p<0.05. The number of cryoneedles assessed by simulation had a 92.52% accuracy when compared with those effectively used. Conclusions: Simulation of the cryoablation using reconstructed 3D CT scan improves the surgical results. The application used for simulation was able to accurately assess the number of cryoneedles required for tumor ablation, their direction and approach.

  15. Treatment Failure After Image-Guided Percutaneous Radiofrequency Ablation (RFA) of Renal Tumors - A Systematic Review with Description of Type, Frequency, Risk Factors and Management.

    Science.gov (United States)

    Vollherbst, Dominik; Bertheau, Robert; Kauczor, Hans-Ulrich; Radeleff, Boris Alexis; Pereira, Philippe L; Sommer, Christof-Matthias

    2017-03-01

    Background  Radiofrequency ablation (RFA) is an established treatment for small renal tumors. The objective of this review is to systematically assess the type, frequency, risk factors and management of treatment failure after image-guided percutaneous RFA of renal tumors. Method  10 studies (967 patients, 1033 tumors) with a mean/median follow-up of ≥ 30 months were systematically identified and analyzed. Results and Conclusion  Image-guided percutaneous RFA of localized renal tumors is very effective. The most common type of treatment failure is residual unablated tumor (5.9 %), followed by local tumor progression (4.7 %). De novo tumors in the kidneys occur in 1.3 % of cases and extra-renal metastases in 2.0 %. Local tumor progression, de novo tumors in the kidneys and extra-renal metastases occur predominantly later than 12 months after initial RFA. Tumor size > 3 cm and central tumor location are the major risk factors for treatment failure. In the case of treatment failure, repeated RFA shows high success rates (86.3 % for residual unablated tumors and 87.5 % for local tumor progression). Key Points: · Treatment failure can be subdivided into residual unablated tumor and local tumor progression.. · Residual unablated tumor occurs in 5.9 % of cases.. · Local tumor progression occurs in 4.7 % of cases.. · Tumor size and location are the major risk factors for treatment failure.. · Repeated RFA is effective and commonly used for management.. Citation Format · Vollherbst D, Bertheau R, Kauczor H et al. Treatment Failure After Image-Guided Percutaneous Radiofrequency Ablation (RFA) of Renal Tumors - A Systematic Review with Description of Type, Frequency, Risk Factors and Management. Fortschr Röntgenstr 2017; 189: 219 - 227. © Georg Thieme Verlag KG Stuttgart · New York.

  16. Percutaneous US-guided RF thermal ablation for malignant renal tumors: preliminary results in 13 patients

    Energy Technology Data Exchange (ETDEWEB)

    Veltri, Andrea; Fazio, Giuseppina De; Malfitana, Valeria; Isolato, Giuseppe; Gandini, Giovanni [University of Turin, Institute of Diagnostic and Interventional Radiology, Turin (Italy); Fontana, Dario; Tizzani, Alessandro [University of Turin, Institute of Urology, Turin (Italy)

    2004-12-01

    Minimally invasive treatment for small renal cell carcinoma (RCC) can be necessary in selected patients and, anyway, is desirable. In situ ablation techniques, including RFA, have been developed. The aim of this study is to evaluate the feasibility, safety and short-term local effectiveness of percutaneous US-guided RFA in a small series, as well as mid-term patient outcome. Thirteen patients with a total of 18 tumors (17 small lesions, 35 mm in size or less, and a larger one, 75 mm in size) underwent 19 RFA sessions. Seven patients had a solitary kidney, and three suffered from VHL disease, too. We treated four lesions in a patient with a bilateral tumor. In another patient, three lesions were ablated. Seventeen tumors were RCC; one was a metastasis from lung cancer. Eight lesions were parenchymal, six exophytic, two parenchymal/exophytic, one parenchymal/central and one central. A monopolar RF system with multitined expandable electrode needles was used. The 35-mm lesion underwent two sessions; the 75-mm lesion was treated with transcatheter arterial embolization before RFA. Tumors with complete loss of contrast enhancement at short-term CT (or MR) were considered successfully treated. Percutaneous US-guided RFA was always feasible without major complications. The success rate after a single treatment in tumors less than 35 mm in size was 88.2% (15/17) and rose to 94.1% (16/17) after the second treatment of the largest lesion. After a mean 14-month follow-up, no successfully treated lesions recurred locally. Only the patient with metastasis from lung cancer died from disease progression in a further location, while all other patients are alive, with renal function still sufficient to avoid dialysis. US guidance allows an easy and safe percutaneous approach for RFA of small non-parahilar RCC. The treatment is locally effective and can be proposed as a minimally invasive therapy for patients with contraindications to surgery or to those expressing an informed

  17. Percutaneous US-guided RF thermal ablation for malignant renal tumors: preliminary results in 13 patients

    International Nuclear Information System (INIS)

    Veltri, Andrea; Fazio, Giuseppina De; Malfitana, Valeria; Isolato, Giuseppe; Gandini, Giovanni; Fontana, Dario; Tizzani, Alessandro

    2004-01-01

    Minimally invasive treatment for small renal cell carcinoma (RCC) can be necessary in selected patients and, anyway, is desirable. In situ ablation techniques, including RFA, have been developed. The aim of this study is to evaluate the feasibility, safety and short-term local effectiveness of percutaneous US-guided RFA in a small series, as well as mid-term patient outcome. Thirteen patients with a total of 18 tumors (17 small lesions, 35 mm in size or less, and a larger one, 75 mm in size) underwent 19 RFA sessions. Seven patients had a solitary kidney, and three suffered from VHL disease, too. We treated four lesions in a patient with a bilateral tumor. In another patient, three lesions were ablated. Seventeen tumors were RCC; one was a metastasis from lung cancer. Eight lesions were parenchymal, six exophytic, two parenchymal/exophytic, one parenchymal/central and one central. A monopolar RF system with multitined expandable electrode needles was used. The 35-mm lesion underwent two sessions; the 75-mm lesion was treated with transcatheter arterial embolization before RFA. Tumors with complete loss of contrast enhancement at short-term CT (or MR) were considered successfully treated. Percutaneous US-guided RFA was always feasible without major complications. The success rate after a single treatment in tumors less than 35 mm in size was 88.2% (15/17) and rose to 94.1% (16/17) after the second treatment of the largest lesion. After a mean 14-month follow-up, no successfully treated lesions recurred locally. Only the patient with metastasis from lung cancer died from disease progression in a further location, while all other patients are alive, with renal function still sufficient to avoid dialysis. US guidance allows an easy and safe percutaneous approach for RFA of small non-parahilar RCC. The treatment is locally effective and can be proposed as a minimally invasive therapy for patients with contraindications to surgery or to those expressing an informed

  18. PO-60 - Renal tumors with extensive vascular disease: management challenges in a pediatric series from the Hospital for Sick Children.

    Science.gov (United States)

    Zamperlini-Netto, G; Zanette, A; Wehbi, E; Williams, S; Grant, R M; Brandao, L R

    2016-04-01

    Venous thrombotic events (VTE) are becoming more and more common in children, particularly in the hospital setting. To date, 1 in 200 children admitted to tertiary pediatric hospitals are now being recognized to develop VTE. Amongst those patients with an identified thrombotic occlusion, pediatric patients diagnosed with renal tumors have long been recognized, but their ideal management in the instances of vascular invasion remains controversial. We describe the clinical behavior of patients diagnosed with renal tumors and extra renal vascular involvement at The Hospital for Sick Children in Toronto, Canada. A retrospective analysis was conducted in patients diagnosed from 1990 to 2012. Data collected included: age, gender, symptoms at presentation, staging, pathology report, radiological evidence of intravascular thrombus [i.e. renal veins (RV), inferior vena cava (IVC) and right atrium (RA)], intraoperative findings, therapeutic protocol implemented and anticoagulation; for outcomes, tumor and/or thrombus recurrence, thromboembolic phenomena [i.e. pulmonary embolism (PE)] and survival. Of 299 patients with renal tumors identified, 292 were included: Wilms (219), Renal Cell Carcinoma (RCC, 29), Clear Cell Sarcoma of the Kidney (CCSK, 12), others (32). The median age of the group was 4.53years (4days - 18 years). Extra renal vascular disease was identified in 29 patients, with a median age 7.05years (0.6-16 years; p=0.03), including Wilms tumors (22/219, 10%), RCC (2/29, 7%), CCSK (1/12, 8.3%) and others (4/32, 12.5%; p=0.01). Vascular involvement comprised exclusive evidence of RV disease (7), IVC disease (19; 15 infra-hepatic), RA disease (3) and PE (5).Treatment escalation because of vascular disease included neo-adjuvant chemotherapy (12; Wilms [11], RCC [1]), intraoperative cavectomy/ thrombectomy (1; Wilms), and cavotomies (11 Wilms [7], RCC [1], CCSK [1], PNET [1], sarcoma [1]). Four patients were placed under cardiopulmonary bypass. Anticoagulation was

  19. ANGIOMIOLIPOMA RENAL. UN TUMOR QUE CAMBIA EL PRONÓSTICO AL PACIENTE | KIDNEY ANGIOMYOLIPOMA. A TUMOR THAT CHANGES THE PATIENT PROGNOSIS

    Directory of Open Access Journals (Sweden)

    Iraida Daboin Hidalgo

    2016-08-01

    Full Text Available Angiomyolipoma is a benign mesenchymal tumor consisting of smooth muscle, adipose tissue and thinwalled blood vessels that has an aberrant hamartomatous distribution. Its most common location is renal, with an incidence in the general population of 0.1% to 0.3%. It can occur in the context of tuberous sclerosis or isolated. This tumor is often asymptomatic and it has been reported that up to 60% of cases are only accidentally diagnosed by radiological screening for some other pathology. The treatment is usually conservative leaving nephrectomy for cases of large tumor mass.

  20. Why should survivors of childhood renal tumor and others with only one kidney be denied the chance to play contact sports?

    Science.gov (United States)

    Spreafico, Filippo; Terenziani, Monica; van den Heuvel-Eibrink, Marry M; Pritchard-Jones, Kathy; Levitt, Gill; Graf, Norbert; Bergeron, Christophe; Massimino, Maura

    2014-04-01

    Over the last few decades advances in the treatment of childhood and young adult cancer have greatly improved survival. As a result most children diagnosed with Wilms' tumor (the most common renal tumor in childhood) or other renal tumors become long-term survivors, living with surgically solitary kidneys. As physical activity is gaining credibility as a therapy enhancing well-being, encouraging cancer survivors do exercise during adolescence and adulthood is advisable. Discussing current evidence-based information would help to provide a framework for the harmonization of guidelines for sport participation of childhood and young adult renal tumor survivors.

  1. Tumor neuroectodérmico primitivo renal. Reporte de un caso

    OpenAIRE

    Nestor Moisés Tineo Araque; Helen Daniela Uzcátegui Camacaro; Henrry Ramirez; Humberto Ruz

    2011-01-01

    El cáncer de riñón representa entre el 2 y 3% del total de las neoplasias malignas, encontrándose entre ellas el tumor neuroectodérmico (1,1% dentro del cáncer renal). En muchos casos el diagnóstico es tardío y se describe la triada clásica: dolor, hematuria y masa palpable. El ultrasonido y la tomografía axial computarizada son los métodos de diagnóstico más importantes y el éxito terapéutico se basa en la cirugía radical. Se presenta caso de paciente femenina de 23 años quien inicia enferm...

  2. 159. Trombectomía de Vena Cava Inferior Por Invasión de Tumor Renal

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    N. Miranda

    2012-04-01

    Conclusiones: en casos seleccionados, el carcinoma de célula renal extendiéndose a VCI puede ser resecado sin dificultad sin necesidad de esternotomía, CBP o PCHP, constituyendo una alternativa curativa en el tratamiento de dichos tumores.

  3. Paraneoplastic Erythropoietin Secreting Tumor of Renal Origin: A Forgotten Endocrine Hormone

    Directory of Open Access Journals (Sweden)

    Yaseen Ali

    2015-09-01

    Full Text Available Paraneoplastic syndromes are a group of disorders associated by the presence of cancer in the human body, but are not caused by the cancer or its metastatic potential. These syndromes are usually the result of cell mediators. For instance, the interleukins and cytokines secreted by the body’s defense system combat with neoplastic cells that inadvertently bind to cell specific receptors in the endocrine system due to similar structural morphology and produce undesired side effects. In this article, we presented an interesting case of a 44-year-old male veteran who came to our free clinic for his chronic constipation unmitigated by over the counter laxatives and sought other provider care in the past few months. During our brief clinic visit, his history and examination revealed that he had also been suffering from mild abdominal pain, decrease in appetite, inconvenient reddish plethoric facial appearance and lethargy. A careful review of the history, physical and laboratory examination revealed erythrocytosis and mild hypercalcemia. Meanwhile, a renal mass biopsy was proven to be erythropoietin secreting renal cell tumor. Subsequently, the patient underwent resection and was followed to complete resolution of his symptoms.

  4. [Diagnostic utility of S100A1, GLUT-1 and Caveolin-1 in renal tumors with oncocytic features: a comparative study].

    Science.gov (United States)

    Zhang, Wei; Wang, Kejia; Yu, Wenjuan; Liu, Yan; Chu, Jing; Jiang, Yanxia; Li, Yujun

    2015-11-01

    To study the immunohistochemical expression of S100A1, GLUT-1 and Cavolin-1 and its diagnostic significance in renal tumors with oncocytic features. Tissue microarray and immunohistochemical staining for S100A1, GLUT-1 and Cavolin-1 were carried out in 59 cases of renal tumors with oncocytic features, including 19 cases of renal oncocytoma, 15 cases of clear cell renal cell carcinoma (CCRCC) with eosinophilic cells, 11 cases of eosinophilic variant of chromophobe renal cell carcinoma, 7 cases of oncocytic papillary renal cell carcinoma and 7 cases of epithelioid angiomyolipoma. S100A1 was expressed in renal oncocytoma, with a positive propotion of 16/19 (including 14 cases showing widespread and strong positivity). On the other hand, the rate of expression of S100A1 was 2/11 in eosinophilic variant of chromophobe renal cell carcinoma, 10/15 in CCRCC with eosinophilic cells, 3/7 in oncocytic papillary renal cell carcinoma and 6/7 in epithelioid angiomyolipoma (P>0.05). The difference of S100A1 expression between renal oncocytoma and eosinophilic variant of chromophobe renal cell carcinoma was statistically significant. GLUT-1 was located in cell membrane, with a positive rate of 13/15 in CCRCC with eosinophilic cells, 7/19 in renal oncocytoma, 4/7 (weak) in oncocytic papillary renal cell carcinoma, 1/11 in eosinophilic variant of chromophobe renal cell carcinoma and 0/7 in epithelioid angiomyolipoma. The rate of expression of Cav-1 was 6/15 in CCRCC with eosinophilic cells, 2/7 in oncocytic papillary renal cell carcinoma, 5/7 in epithelioid angiomyolipoma, 2/11 (weak) in eosinophilic variant of chromophobe renal cell carcinoma and 0/19 in renal oncocytoma. S100A1 showed high sensitivity and 50% specificity in the diagnosis of renal oncocytoma. GLUT-1 and Cav-1 showed high specificity and sensitivity in the diagnosis of CCRCC and epithelioid angiomyolipoma. S100A1 is widely expressed in various oncocytic renal neoplasms and helpful in differential diagnosis of renal

  5. Phase I/II Study of Radiofrequency Ablation for Malignant Renal Tumors: Japan Interventional Radiology in Oncology Study Group 0701

    International Nuclear Information System (INIS)

    Mimura, Hidefumi; Arai, Yasuaki; Yamakado, Koichiro; Sone, Miyuki; Takeuchi, Yoshito; Miki, Tsuneharu; Gobara, Hideo; Sakuhara, Yusuke; Yamamoto, Takanobu; Sato, Yozo; Kanazawa, Susumu

    2016-01-01

    PurposeThis multicenter phase I/II study evaluated the safety, feasibility, and initial efficacy of radiofrequency ablation (RFA) for small malignant renal tumors.MethodsThirty-three patients were enrolled in the study. A single session of RFA was performed in patients with a renal tumor of 1–3 cm in greatest diameter, with the exception of lesions adjacent to the renal hilum. The primary endpoint was the safety of renal RFA, and the secondary endpoints were its feasibility and initial efficacy for local control, as well as the incidence and grade of adverse events. Clinical efficacy was evaluated by CT scans within 1 week and at a further 4 weeks after the procedure using the criteria adapted from the Response Evaluation Criteria in Solid Tumors.ResultsThe RFA procedure was completed in 100 % (95 % confidence interval [CI] 89–100 %) of all 33 patients. There were no severe adverse events (0 % [95 % CI 0–11 %]). Among the 33 patients, a complete response, partial response, progressive disease, and stable disease were seen in 28 (85 %), 0 (0 %), one (3 %), and one (3 %) patient(s), respectively, with a tumor response rate of 85 % [95 % CI 68–95 %]). Three patients (9 %), including one ineligible patient (3 %), were not evaluable. Out of 30 evaluable patients, a complete response was achieved in 28 (93 %).ConclusionThe current multicenter trial revealed that RFA is a safe, feasible, and effective treatment for small malignant renal tumors in patients who are not candidates for surgery.

  6. Phase I/II Study of Radiofrequency Ablation for Malignant Renal Tumors: Japan Interventional Radiology in Oncology Study Group 0701

    Energy Technology Data Exchange (ETDEWEB)

    Mimura, Hidefumi, E-mail: mimura@marianna-u.ac.jp [St. Marianna University School of Medicine, Department of Radiology (Japan); Arai, Yasuaki, E-mail: arai-y3111@mvh.biglobe.ne.jp [National Cancer Center Hospital, Department of Diagnostic Radiology (Japan); Yamakado, Koichiro, E-mail: yama@clin.medic.mie-u.ac.jp [Mie University School of Medicine, Department of Interventional Radiology (Japan); Sone, Miyuki, E-mail: msone@me.com; Takeuchi, Yoshito, E-mail: yotake62@qg8.so-net.ne.jp [National Cancer Center Hospital, Department of Diagnostic Radiology (Japan); Miki, Tsuneharu, E-mail: tmiki@koto.kpu-m.ac.jp [Kyoto Prefectural University of Medicine, Department of Urology (Japan); Gobara, Hideo, E-mail: gobara@cc.okayama-u.ac.jp [Okayama University Medical School, Department of Radiology (Japan); Sakuhara, Yusuke, E-mail: yusaku@med.hokudai.ac.jp [Hokkaido University School of Medicine, Department of Diagnostic and Interventional Radiology (Japan); Yamamoto, Takanobu, E-mail: tyamamot@tcc.pref.tochigi.lg.jp [Tochigi Cancer Center, Department of Radiology (Japan); Sato, Yozo, E-mail: ysato@aichi-cc.jp [Aichi Cancer Center Hospital, Department of Diagnostic and Interventional Radiology (Japan); Kanazawa, Susumu, E-mail: susumu@cc.okayama-u.ac.jp [Okayama University Medical School, Department of Radiology (Japan)

    2016-05-15

    PurposeThis multicenter phase I/II study evaluated the safety, feasibility, and initial efficacy of radiofrequency ablation (RFA) for small malignant renal tumors.MethodsThirty-three patients were enrolled in the study. A single session of RFA was performed in patients with a renal tumor of 1–3 cm in greatest diameter, with the exception of lesions adjacent to the renal hilum. The primary endpoint was the safety of renal RFA, and the secondary endpoints were its feasibility and initial efficacy for local control, as well as the incidence and grade of adverse events. Clinical efficacy was evaluated by CT scans within 1 week and at a further 4 weeks after the procedure using the criteria adapted from the Response Evaluation Criteria in Solid Tumors.ResultsThe RFA procedure was completed in 100 % (95 % confidence interval [CI] 89–100 %) of all 33 patients. There were no severe adverse events (0 % [95 % CI 0–11 %]). Among the 33 patients, a complete response, partial response, progressive disease, and stable disease were seen in 28 (85 %), 0 (0 %), one (3 %), and one (3 %) patient(s), respectively, with a tumor response rate of 85 % [95 % CI 68–95 %]). Three patients (9 %), including one ineligible patient (3 %), were not evaluable. Out of 30 evaluable patients, a complete response was achieved in 28 (93 %).ConclusionThe current multicenter trial revealed that RFA is a safe, feasible, and effective treatment for small malignant renal tumors in patients who are not candidates for surgery.

  7. Introduction to Sporadic Groups

    Directory of Open Access Journals (Sweden)

    Luis J. Boya

    2011-01-01

    Full Text Available This is an introduction to finite simple groups, in particular sporadic groups, intended for physicists. After a short review of group theory, we enumerate the 1+1+16=18 families of finite simple groups, as an introduction to the sporadic groups. These are described next, in three levels of increasing complexity, plus the six isolated ''pariah'' groups. The (old five Mathieu groups make up the first, smallest order level. The seven groups related to the Leech lattice, including the three Conway groups, constitute the second level. The third and highest level contains the Monster group M, plus seven other related groups. Next a brief mention is made of the remaining six pariah groups, thus completing the 5+7+8+6=26 sporadic groups. The review ends up with a brief discussion of a few of physical applications of finite groups in physics, including a couple of recent examples which use sporadic groups.

  8. The effect of AST/ALT (De Ritis) ratio on survival and its relation to tumor histopathological variables in patients with localized renal cell carcinoma.

    Science.gov (United States)

    Canat, Lütfi; Ataly, Hasan Anil; Agalarov, Samir; Alkan, Ilter; Alturende, Fatih

    2017-12-07

    To assess the relationship between De Ritis (aspartate aminotransaminase [AST]/Alanine aminotransaminase [ALT]) ratio and pathological variables and whether it is an independent prognostic factor. We analyzed 298 consecutive patients who underwent radical or partial nephrectomy for non-metastatic renal cell carcinoma (RCC) between 2006 and 2015. The association between De Ritis ratio and pathological variables including tumor size, presence of renal vein invasion, vena cava invasion, renal capsule infiltration, Gerota fascia invasion, renal sinus involvement, renal pelvic invasion, angiolymphatic invasion, adrenal gland involvement, lymph node involvement, tumor necrosis, and Fuhrman's grade was tested. Multivariable Cox analysis was performed to evaluate the impact of this ratio on overall survival and cancer-specific survival. An increased preoperative De Ritis ratio was significantly associated with renal vein invasion, renal capsule infiltration and renal pelvis involvement (p<0.05) in non-metastatic RCC. On multivariate analysis we found that tumor size, Fuhrman grade and lymph node involvement were independent prognostic factors for cancerspecific survival. AST/ALT ratio had no influence on the risk of overall and cancerspecific survival. An increased preoperative AST/ALT ratio had a significant association with renal vein invasion, renal capsule infiltration and renal pelvis involvement in patients with non-metastatic RCC. However, it does not appear to be an independent prognostic marker in non-metastatic RCC. Copyright® by the International Brazilian Journal of Urology.

  9. Population based analysis of survival in patients with renal cell carcinoma and venous tumor thrombus.

    Science.gov (United States)

    Whitson, Jared M; Reese, Adam C; Meng, Maxwell V

    2013-02-01

    To identify prognostic factors for renal cell carcinoma (RCC) with venous tumor thrombus (VTT) and determine the significance of thrombus level on survival. Patients within the Surveillance, Epidemiology, and End Results (SEER) database with RCC and VTT were identified and included if managed surgically. The Kaplan-Meier method and Cox regression analyses were performed to identify factors associated with disease-specific survival. A total of 1,875 patients met the inclusion criteria. One-year survival for patients undergoing surgery was 60% for patients with metastases and 90% for those without. Factors associated with worse survival included larger tumor size (HR 1.2, 95% CI 1.0-1.4), medullary, collecting duct, or sarcomatoid histology (HR 2.2, 95% CI 1.5-3.3), Fuhrman grade 3 (HR 2.2, 95% CI 1.5-3.3) or grade 4 (HR 2.9, 95% CI 1.8-4.5) tumors, positive lymph nodes (HR 1.5, 95% CI 1.0-2.0), and metastases (HR 3.5, 95% CI 2.6-4.8). Thrombus level above the diaphragm (T3c) was not significantly associated with worse survival (HR 1.4, 95% CI 0.8-2.5). In this large, population-based study of patients with RCC and VTT, we identify several disease-specific factors strongly associated with cancer-specific mortality. After controlling for adverse prognostic factors, thrombus level was not associated with worse outcome. Copyright © 2013 Elsevier Inc. All rights reserved.

  10. Uterine Didelphys with blind hemivagina, hematocolpos, ipsilateral renal agenesis (UD-BHRA) and bilateral ovarian tumors : a case report

    International Nuclear Information System (INIS)

    Lee, Young Rae; Choi, Byung Ik; Park, Hae Won; Kook, Shin Ho

    1998-01-01

    Uterine didelphys is a congenital malformation characterized by the presence of two separated hemiuteri and hemivaginas, due to lack of midfusion of the mullerian ducts. We report a case of UD-BHRA (uterine didelphys with blind hemivagina and ipsilateral renal agencies), a rare type of uterine dedelphys characterized by symptomatic unilateral hematocolpos due to blind hemivagina after menarche and ipsilateral renal agenesis. The MRI findings in 22-year-old woman with bilateral ovarian tumors demonstrated two separated uterine horns and cervical and vaginal canals, with left hematocolpos and left agenesis. (author). 7 refs., 1 fig

  11. Inter-rater reliability of surgical reviews for AREN03B2: a COG renal tumor committee study.

    Science.gov (United States)

    Hamilton, Thomas E; Barnhart, Douglas; Gow, Kenneth; Ferrer, Fernando; Kandel, Jessica; Glick, Richard; Dasgupta, Roshni; Naranjo, Arlene; He, Ying; Gratias, Eric; Geller, James; Mullen, Elizabeth; Ehrlich, Peter

    2014-01-01

    The Children's Oncology Group (COG) renal tumor study (AREN03B2) requires real-time central review of radiology, pathology, and the surgical procedure to determine appropriate risk-based therapy. The purpose of this study was to determine the inter-rater reliability of the surgical reviews. Of the first 3200 enrolled AREN03B2 patients, a sample of 100 enriched for blood vessel involvement, spill, rupture, and lymph node involvement was selected for analysis. The surgical assessment was then performed independently by two blinded surgical reviewers and compared to the original assessment, which had been completed by another of the committee surgeons. Variables assessed included surgeon-determined local tumor stage, overall disease stage, type of renal procedure performed, presence of tumor rupture, occurrence of intraoperative tumor spill, blood vessel involvement, presence of peritoneal implants, and interpretation of residual disease. Inter-rater reliability was measured using the Fleiss' Kappa statistic two-sided hypothesis tests (Kappa, p-value). Local tumor stage correlated in all 3 reviews except in one case (Kappa=0.9775, p<0.001). Similarly, overall disease stage had excellent correlation (0.9422, p<0.001). There was strong correlation for type of renal procedure (0.8357, p<0.001), presence of tumor rupture (0.6858, p<0.001), intraoperative tumor spill (0.6493, p<0.001), and blood vessel involvement (0.6470, p<0.001). Variables that had lower correlation were determination of the presence of peritoneal implants (0.2753, p<0.001) and interpretation of residual disease status (0.5310, p<0.001). The inter-rater reliability of the surgical review is high based on the great consistency in the 3 independent review results. This analysis provides validation and establishes precedent for real-time central surgical review to determine treatment assignment in a risk-based stratagem for multimodal cancer therapy. © 2014.

  12. MRI-guided percutaneous cryoablation of renal tumors: Use of external manual displacement of adjacent bowel loops

    International Nuclear Information System (INIS)

    Tuncali, Kemal; Morrison, Paul R.; Tatli, Servet; Silverman, Stuart G.

    2006-01-01

    Purpose: We sought to investigate retrospectively the safety and effectiveness of using external hand compression to displace adjacent bowel loops during MRI-guided percutaneous cryoablation of renal tumors. Materials and methods: Fourteen patients (six women, eight men; mean age: 72 years) with 15 renal tumors (mean diameter: 2.4 cm; range: 1.4-4.6 cm) adjacent to bowel were treated with MRI-guided percutaneous cryoablation during which bowel was displaced manually. Bowel loop of concern was ascending colon (n 5), descending colon (n = 8), descending colon and small bowel (n = 1), ascending colon and small bowel (n = 1). To analyze effectiveness of the maneuver, mean distance between tumor margin and bowel before and after the maneuver were compared and analyzed using paired Student's t-test. Minimum distance between iceball edge and adjacent bowel with external manual displacement during freezing was also measured. Safety was assessed by analyzing post-procedural MR imaging for adjacent bowel wall thickening and focal fluid collections as well as patients' clinical and imaging follow-up. Results: Mean distance between tumor margin and closest adjacent bowel increased from 0.8 cm (range: 0-2 cm) before external manual compression to 2.6 cm (range: 1.6-4.1 cm) with manual displacement (p < 0.01). Mean minimum distance between iceball edge and closest adjacent bowel during the procedures was 1.6 cm (range: 0.5-3.5 cm). No evidence of bowel injury was encountered. Twelve of 15 tumors had follow-up (mean: 10 months) that showed no tumor recurrence. Conclusion: MRI-guided percutaneous cryoablation of renal tumors adjacent to bowel can be done safely and effectively using external hand compression to displace bowel loops

  13. Clinicopathologic factors identify sporadic mismatch repair-defective colon cancers

    DEFF Research Database (Denmark)

    Halvarsson, Britta; Anderson, Harald; Domanska, Katarina

    2008-01-01

    Identification of sporadic mismatch repair (MMR)-defective colon cancers is increasingly demanded for decisions on adjuvant therapies. We evaluated clinicopathologic factors for the identification of these prognostically favorable tumors. Histopathologic features in 238 consecutive colon cancers...

  14. Transarterial ethanol ablation for sporadic and non-hemorrhaging angiomyolipoma in the kidney

    Energy Technology Data Exchange (ETDEWEB)

    Takebayashi, Shigeo [Department of Radiology, Yokohama City University Medical Center, 4-57, Urafune-cho, Minami-ku, Yokohama 232-0024 (Japan)], E-mail: take2922@urahp.yokohama-cu.ac.jp; Horikawa, Ayumi; Arai, Mito; Iso, Shinichiroh [Department of Radiology, Yokohama City University Medical Center, 4-57, Urafune-cho, Minami-ku, Yokohama 232-0024 (Japan); Noguchi, Kazumi [Department of Urology, Yokohama City University Medical Center, Yokohama (Japan)

    2009-10-15

    Purpose: We evaluated the efficacy and side effects of transarterial ethanol ablation in sporadic and non-hemorrhaging angiomyolipomas (AMLs) in the kidney. Material and Methods: A total of 10 patients with solitary and sporadic AMLs underwent selective transarterial absolute ethanol ablation for prophylaxis against hemorrhage. We confirmed the ratio areas of tumor vessel on angiogram, those of infraction on post-ablation computed tomography (CT) and those of tumor reduction in a 3-, 6- and 12-month follow-up CT. Results: Once or twice a single infusion of 1 or 2 ml absolute ethanol achieved in a total occlusion of 22 feeding arteries which consisted of 7 proximal interlobar arteries, 12 distal interlobar arteries and 3 renal capsular arteries. Nontarget occlusion did not occur by ethanol reflux in any cases but occurred causing spasms provoked by repeated inflation and deflation of the balloon in one case. Total occlusion of tumor vessels was observed in 7 patients and 92-95% occlusion in 3. Ethanol ablation produced 1.8-22.5% (mean 8.4 {+-} 6.8%) areas of infarctions but the outcome was not serious in all cases. Mean percentage areas of tumor reduction were 29.4 {+-} 10.6% in a 3-month follow-up, 45.7 {+-} 11.9% in a 6-month and 59.3 {+-} 11.5% in a 12-month follow-up. Conclusions: Absolute ethanol ablation for sporadic and non-hemorrhaging AML is safe and effective in reducing majority of tumor area in a 1-year follow-up.

  15. The in vitro and in vivo effects of re-expressing methylated von Hippel-Lindau tumor suppressor gene in clear cell renal carcinoma with 5-aza-2'-deoxycytidine.

    Science.gov (United States)

    Alleman, Wade G; Tabios, Ray L; Chandramouli, Gadisetti V R; Aprelikova, Olga N; Torres-Cabala, Carlos; Mendoza, Arnulfo; Rogers, Craig; Rodgers, Craig; Sopko, Nikolai A; Linehan, W Marston; Vasselli, James R

    2004-10-15

    Clear cell renal carcinoma (ccRCC) is strongly associated with loss of the von Hippel-Lindau (VHL) tumor suppressor gene. The VHL gene is functionally lost through hypermethylation in up to 19% of sporadic ccRCC cases. We theorized that re-expressing VHL silenced by methylation in ccRCC cells, using a hypo-methylating agent, may be an approach to treatment in patients with this type of cancer. We test the ability of two hypo-methylating agents to re-express VHL in cell culture and in mice bearing human ccRCC and evaluate the effects of re-expressed VHL in these models. Real-time reverse transcription-PCR was used to evaluate the ability of zebularine and 5-aza-2'-deoxycytidine (5-aza-dCyd) to re-express VHL in four ccRCC cell lines with documented VHL gene silencing through hypermethylation. We evaluated if the VHL re-expressed after hypo-methylating agent treatment could recreate similar phenotypic changes in ccRCC cells observed when the VHL gene is re-expressed via transfection in cell culture and in a xenograft mouse model. Finally we evaluate global gene expression changes occurring in our cells, using microarray analysis. 5-Aza-dCyd was able to re-express VHL in our cell lines both in culture and in xenografted murine tumors. Well described phenotypic changes of VHL expression including decreased invasiveness into Matrigel, and decreased vascular endothelial growth factor and glucose transporter-1 expression were observed in the treated lines. VHL methylated ccRCC xenografted tumors were significantly reduced in size in mice treated with 5-aza-dCyd. Mice bearing nonmethylated but VHL-mutated tumors showed no tumor shrinkage with 5-aza-dCyd treatment. Hypo-methylating agents may be useful in the treatment of patients having ccRCC tumors consisting of cells with methylated VHL.

  16. Open removal of a retained retrohepatic inferior vena cava filter with a residual primary neuroectodermal renal tumoral thrombus.

    Science.gov (United States)

    Hinojosa, Carlos A; Torres-Machorro, Adriana; Lizola, Rene; Anaya-Ayala, Javier E

    2015-10-29

    Primary neuroectodermal renal tumours (PNET) are rare and aggressive neoplasms; thrombosis of the inferior vena cava (IVC) is associated with this entity. We report here the case of a 19-year-old man who experienced a new onset of abdominal pain. A CT scan revealed a large left renal mass, perirenal haematoma and IVC thrombosis. Owing to an acute drop in haemoglobin and subsegmentary pulmonary embolism, he underwent emergency selective renal artery angiography and embolisation of bleeding vessels and IVC filter (IVCF) placement. Once stable, he underwent a left radical nephrectomy and IVC thrombectomy; the pathology report confirmed PNET. 6 months later, imaging revealed a residual tumoral thrombus in the IVCF located in the retrohepatic IVC. The patient underwent removal of this device and the thrombus via a right thoracoabdominal approach. He recovered well and at 4 months, he continues his chemotherapy cycles. 2015 BMJ Publishing Group Ltd.

  17. Renal malignant solitary fibrous tumor with single lymph node involvement: report of unusual metastasis and review of the literature

    Directory of Open Access Journals (Sweden)

    Mearini E

    2014-05-01

    Full Text Available Ettore Mearini,1 Giovanni Cochetti,1 Francesco Barillaro,1 Sonia Fatigoni,2 Fausto Roila2 1Department of Medical-Surgical Specialties and Public Health, Division of Urological Andrological Surgery and Minimally Invasive Techniques, University of Perugia, Terni, Italy; 2Medical Oncology, S Maria Hospital, Terni, Italy Abstract: Solitary fibrous tumors are rare mesenchymal spindle cell neoplasms that are usually found in the pleura. The kidneys are an uncommon site and only few cases of renal solitary fibrous tumor exhibit malignant behavior metastasizing to the liver, lung, and bone through the hematogenous pathway. Purpose: To describe the first case of lymph node metastasis from renal solitary fibrous tumor in order to increase the knowledge about the malignant behavior of these tumors. Patients and methods: A 19-year-old female patient had intermittent hematuria for several months without flank pain or other symptoms. A chest and abdomen CT scan was performed and showed a multi-lobed bulky solid mass of 170 × 98 × 120 mm in the left kidney. One day before the surgery, the left renal artery was catheterized and the kidney embolization was performed using a Haemostatic Absorbable Gelatin Sponge and polyvinyl alcohol. We then performed a radical nephrectomy with hilar, para-aortic, and inter-aortocaval lymphadenectomy. Results: Estimated intraoperative blood loss was 200 mL and the operative time was 100 minutes. No postoperative complications occurred. The hospital stay was 7 days long. The histological examination was malignant solitary fibrous tumor of the kidney. Cancerous tissue showed cellular atypia, with an increased mitotic index (up to 7 × 10 hpf. Immunohistochemical analysis showed positive results for CD34, BCL2, partial expression of HBME1, and occasionally of synaptophysin. Histological evaluation confirmed the presence of metastasis in one hilar node. The patient did not receive any other therapy. At 30-month follow-up, the

  18. Robotic partial nephrectomy for renal tumors larger than 4 cm: a systematic review and meta-analysis.

    Directory of Open Access Journals (Sweden)

    Liangkuan Bi

    Full Text Available BACKGROUND: With the establishment of minimally invasive surgery in society, the robot has been increasingly widely used in the urologic field, including in partial nephrectomy. This study aimed to comprehensively summarize the currently available evidence on the feasibility and safety of robotic partial nephrectomy for renal tumors of >4 cm. METHOD AND FINDINGS: An electronic database search of PubMed, Scopus, Web of Science, and the Cochrane Library was performed. This systematic review and meta-analysis was based on all relevant studies that assessed robotic partial nephrectomy for renal tumors of >4 cm. Five studies were included. The meta-analysis involved 3 studies from 11 institutions including 154 patients, while the narrative review involved the remaining 2 studies from 5 institutions including 64 patients. In the meta-analysis, the mean ischemic time, operation time, and console time was 28, 319, and 189 minutes, respectively. The estimated blood loss and length of stay was 317 ml and 3.8 days, respectively. The rates of conversion, positive margins, intraoperative complications, postoperative complications, hilar clamping, and collecting system repair were 7.0%, 3.5%, 7.0%, 9.8%, 93.9%, and 47.5%, respectively. The narrative review showed results similar to those of the meta-analysis. CONCLUSIONS: Robotic partial nephrectomy is feasible and safe for renal tumors of >4 cm with an acceptable warm ischemic time, positive margin rate, conversion rate, complication rate, operation time, estimated blood loss, and length of stay.

  19. Characterization of pancreatic islet cell tumors and renal tumors induced by a combined treatment of streptozotocin and nicotinamide in male SD rats.

    Science.gov (United States)

    Kato, Yuki; Masuno, Koichi; Fujisawa, Kae; Tsuchiya, Noriko; Torii, Mikinori; Hishikawa, Atsuko; Izawa, Takeshi; Kuwamura, Mitsuru; Yamate, Jyoji

    2017-09-05

    We herein investigated the histopathological features, including proliferative activity and immunoexpression, of pancreatic islet cell tumors (ICTs) in male SD rats induced by streptozotocin (STZ) and nicotinamide (NA), and discussed their relevance to biological behaviors and prognoses. A total of 70 and 43% of rats developed ICTs 37-45 weeks after the treatment with STZ (50 or 75mg/kg, i.v.) and NA (350mg/kg, twice, p.o.), respectively. Among the islet tumors observed in the STZ/NA-treated groups, 75% were adenomas, while 25% were carcinomas. Most STZ/NA-induced carcinomas were characterized by well-differentiated tumor cells with/without local invasion into the surrounding tissues, and weak proliferative activity. No outcome such as distance metastasis and death was noted. All of the ICTs strongly expressed insulin, part of which had hormone productivity; however there were no hypoglycemia-related clinical signs such as convulsion in these rats 36 weeks after the treatment. These results suggested that rat ICTs induced STZ/NA have small impact on biological activity or prognosis. STZ/NA treatment significantly increased of focal proliferative lesions in the kidney, liver and adrenal glands other than pancreatic islets. Of the STZ/NA-induced kidney tumors, more than 60% were renal cell adenomas, and many of them were basophilic type. The incidence of eosinophilic or clear cell type of tumors was less than 10%, respectively. Immunohistochemical analyses revealed that many of the STZ/NA-induced basophilic type of renal tumors were derived from proximal tubules, whereas the clear cell and eosinophilic types were derived from collecting tubules. Copyright © 2017 Elsevier GmbH. All rights reserved.

  20. Dll4 blockade potentiates the anti-tumor effects of VEGF inhibition in renal cell carcinoma patient-derived xenografts.

    Directory of Open Access Journals (Sweden)

    Kiersten Marie Miles

    Full Text Available The Notch ligand Delta-like 4 (Dll4 is highly expressed in vascular endothelium and has been shown to play a pivotal role in regulating tumor angiogenesis. Blockade of the Dll4-Notch pathway in preclinical cancer models has been associated with non-productive angiogenesis and reduced tumor growth. Given the cross-talk between the vascular endothelial growth factor (VEGF and Delta-Notch pathways in tumor angiogenesis, we examined the activity of a function-blocking Dll4 antibody, REGN1035, alone and in combination with anti-VEGF therapy in renal cell carcinoma (RCC.Severe combined immunodeficiency (SCID mice bearing patient-derived clear cell RCC xenografts were treated with REGN1035 and in combination with the multi-targeted tyrosine kinase inhibitor sunitinib or the VEGF blocker ziv-aflibercept. Immunohistochemical and immunofluorescent analyses were carried out, as well as magnetic resonance imaging (MRI examinations pre and 24 hours and 2 weeks post treatment. Single agent treatment with REGN1035 resulted in significant tumor growth inhibition (36-62% that was equivalent to or exceeded the single agent anti-tumor activity of the VEGF pathway inhibitors sunitinib (38-54% and ziv-aflibercept (46%. Importantly, combination treatments with REGN1035 plus VEGF inhibitors resulted in enhanced anti-tumor effects (72-80% growth inhibition, including some tumor regression. Magnetic resonance imaging showed a marked decrease in tumor perfusion in all treatment groups. Interestingly, anti-tumor efficacy of the combination of REGN1035 and ziv-aflibercept was also observed in a sunitinib resistant ccRCC model.Overall, these findings demonstrate the potent anti-tumor activity of Dll4 blockade in RCC patient-derived tumors and a combination benefit for the simultaneous targeting of the Dll4 and VEGF signaling pathways, highlighting the therapeutic potential of this treatment modality in RCC.

  1. The von Hippel-Lindau tumor suppressor regulates programmed cell death 5-mediated degradation of Mdm2

    NARCIS (Netherlands)

    Essers, P B; Klasson, T D; Pereboom, T C; Mans, D A; Nicastro, M; Boldt, K; Giles, R H; MacInnes, A W

    2015-01-01

    Functional loss of the von Hippel-Lindau (VHL) tumor suppressor protein (pVHL), which is part of an E3-ubiquitin ligase complex, initiates most inherited and sporadic clear-cell renal cell carcinomas (ccRCC). Genetic inactivation of the TP53 gene in ccRCC is rare, suggesting that an alternate

  2. Laparoscopic cryoablation of angiomyolipomas in adolescents and young adults: A report of four cases associated with tuberous sclerosis and 1 case of sporadic origin.

    Science.gov (United States)

    Trelborg, Karina; Nielsen, Tommy Kjærgaard; Østraat, Ernst Øyvind; Olsen, Lars Henning

    2016-12-01

    Renal angiomyolipomas (AMLs) can be of sporadic origin or associated with tuberous sclerosis (TS). TS-associated AMLs often present in childhood, tend to be bilateral and multiple, and often exhibit a faster growth rate with an increased risk of hemorrhage. Renal cryoablation is well described in adults, whereas experiences with adolescents and young adults are limited. We present here for the first time a series of renal AMLs within adolescents and young adults treated with laparoscopic assisted cryoablation (LCA). The aim was to evaluate whether LCA of AMLs in adolescents and young adults is a safe and feasible treatment modality. From October 2009 to September 2013 a total of five patients at our institution were diagnosed with AMLs requiring treatment. Four patients had TS and one had AML of sporadic origin, all five patients underwent LCA. Data were retrospectively collected by a systematic review of patient material and reassessment of renal imaging. The median age was 16 years (range 13-27 years). Eight AMLs with a median size of 3.9 cm (range 2.1-7.7 cm) were treated in five patients because of tumor size and rapid growth. Follow-up was a median 37 months (range 20-62 months), and all tumors showed a reduction in tumor size, and no regrowth was recognized (see summary table). The procedure was well tolerated, with only few perioperative complications and no postoperative complications. When considering the indication for treating AMLs, the origin (sporadic or TS associated) and size of the tumor are the decisive factors. To preserve renal function and prevent spontaneous hemorrhage caution should be intensified when tumors reach 4 cm, particularly if TS is present. After the diagnosis is established, patients should be monitored with renal imaging at regular intervals to identify rapid-growing tumors. As an alternative to current treatment modalities such as partial nephrectomy and super selective angioembolization, cryoablation is an emerging approach

  3. Dendritic-cell-based immunotherapy evokes potent anti-tumor immune responses in CD105+ human renal cancer stem cells.

    Science.gov (United States)

    Zhang, Xiao-Fei; Weng, De-Sheng; Pan, Ke; Zhou, Zi-Qi; Pan, Qiu-Zhong; Zhao, Jing-Jing; Tang, Yan; Jiang, Shan-Shan; Chen, Chang-Long; Li, Yong-Qiang; Zhang, Hong-Xia; Chang, Alfred E; Wicha, Max S; Zeng, Yi-Xin; Li, Qiao; Xia, Jian-Chuan

    2017-11-01

    Cancer stem cells (CSCs) are responsible for tumor initiation, progression, and resistance to therapeutic agents; they are usually less sensitive to conventional cancer therapies, and could cause tumor relapse. An ideal therapeutic strategy would therefore be to selectively target and destroy CSCs, thereby preventing tumor relapse. The aim of the present study was to evaluate the effectiveness of dendritic cells (DCs) pulsed with antigen derived from CD105+ human renal cell carcinoma (RCC) CSCs against renal cancer cells in vitro and in vivo. We identified "stem-like" characteristics of CD105+ cells in two human RCC cell lines: A498 and SK-RC-39. Loading with cell lysates did not change the characteristics of the DCs. However, DCs loaded with lysates derived from CD105+ CSCs induced more functionally specific active T cells and specific antibodies against CSCs, and clearly depressed the tumor growth in mice. Our results could form the basis for a novel strategy to improve the efficacy of DC-based immunotherapy for human RCC. © 2017 Wiley Periodicals, Inc.

  4. Long-term High Fat Ketogenic Diet Promotes Renal Tumor Growth in a Rat Model of Tuberous Sclerosis.

    Science.gov (United States)

    Liśkiewicz, Arkadiusz D; Kasprowska, Daniela; Wojakowska, Anna; Polański, Krzysztof; Lewin-Kowalik, Joanna; Kotulska, Katarzyna; Jędrzejowska-Szypułka, Halina

    2016-02-19

    Nutritional imbalance underlies many disease processes but can be very beneficial in certain cases; for instance, the antiepileptic action of a high fat and low carbohydrate ketogenic diet. Besides this therapeutic feature it is not clear how this abundant fat supply may affect homeostasis, leading to side effects. A ketogenic diet is used as anti-seizure therapy i.a. in tuberous sclerosis patients, but its impact on concomitant tumor growth is not known. To examine this we have evaluated the growth of renal lesions in Eker rats (Tsc2+/-) subjected to a ketogenic diet for 4, 6 and 8 months. In spite of existing opinions about the anticancer actions of a ketogenic diet, we have shown that this anti-seizure therapy, especially in its long term usage, leads to excessive tumor growth. Prolonged feeding of a ketogenic diet promotes the growth of renal tumors by recruiting ERK1/2 and mTOR which are associated with the accumulation of oleic acid and the overproduction of growth hormone. Simultaneously, we observed that Nrf2, p53 and 8-oxoguanine glycosylase α dependent antitumor mechanisms were launched by the ketogenic diet. However, the pro-cancerous mechanisms finally took the ascendency by boosting tumor growth.

  5. Laparoscopic resection of tumor recurrence after radical nephrectomy for localized renal cell carcinoma

    Directory of Open Access Journals (Sweden)

    Lessandro Curcio

    2014-06-01

    Full Text Available Introduction Local recurrence of Renal Cell Carcinoma (RCC after radical nephrectomy is a rare event. Some known risk factors are: clinical/pathological stage, locorregional disease and lyimph node positivity. Since up to 30-40% of patients can achieve a disease-free status, we show a case (video in which we performed a laparoscopic excision of a local RCC, taking advantage of all the well-known benefits of laparoscopy.Case report A 56 years old female with a history of open radical nephrectomy two years before was diagnosed with a mass at the time of surveillance CT imaging during follow-up. The suspected local recurrence was 12cm, and vascularized predominantly by tributaries originating from the iliac vessels. There was no other site of disease (i.e. brain, lung, liver, bones and laboratory tests were normal. Laparoscopic approach was approached, by inserting 4 trocars (2 of 10 and 2 of 5mm with the patient in the lateral position.Result The procedure lasted 130 minutes, with 220mL of estimated bleeding; the larger vessels were ligated with polymer clips (Hem-o-lok and the smaller handled by ultrasonic clamp. The specimen was removed by a small incision below the umbilicus in an appropriate bag. The patient was feed in the first postoperative day and discharged on the third day. Histopathology revealed sarcoma, with a high degree of mitosis, and negative surgical margins. She was referred to medical oncology for adjuvant therapy consideration.Conclusion The laparoscopic resection of recurrent tumor should be encouraged in highly selected cases. The minimally invasive method, with its known advantages, especially for more debilitated patients, can be advantageous when applied to suitable cases.

  6. Laparoscopic resection of tumor recurrence after radical nephrectomy for localized renal cell carcinoma.

    Science.gov (United States)

    Curcio, Lessandro; Cunha, Antonio Claudio; Renteria, Juan; Presto, Daniel

    2014-01-01

    Local recurrence of Renal Cell Carcinoma (RCC) after radical nephrectomy is a rare event. Some known risk factors are: clinical/pathological stage, locorregional disease and lyimph node positivity. Since up to 30-40% of patients can achieve a disease-free status, we show a case (video) in which we performed a laparoscopic excision of a local RCC, taking advantage of all the well-known benefits of laparoscopy. A 56 years old female with a history of open radical nephrectomy two years before was diagnosed with a mass at the time of surveillance CT imaging during follow-up. The suspected local recurrence was 12 cm, and vascularized predominantly by tributaries originating from the iliac vessels. There was no other site of disease (i.e. brain, lung, liver, bones) and laboratory tests were normal. Laparoscopic approach was approached, by inserting 4 trocars (2 of 10 and 2 of 5mm) with the patient in the lateral position. The procedure lasted 130 minutes, with 220 mL of estimated bleeding; the larger vessels were ligated with polymer clips (Hem-o-lok) and the smaller handled by ultrasonic clamp. The specimen was removed by a small incision below the umbilicus in an appropriate bag. The patient was feed in the first postoperative day and discharged on the third day. Histopathology revealed sarcoma, with a high degree of mitosis, and negative surgical margins. She was referred to medical oncology for adjuvant therapy consideration. The laparoscopic resection of recurrent tumor should be encouraged in highly selected cases. The minimally invasive method, with its known advantages, especially for more debilitated patients, can be advantageous when applied to suitable cases.

  7. The von Hippel-Lindau tumor suppressor gene inhibits hepatocyte growth factor/scatter factor-induced invasion and branching morphogenesis in renal carcinoma cells.

    Science.gov (United States)

    Koochekpour, S; Jeffers, M; Wang, P H; Gong, C; Taylor, G A; Roessler, L M; Stearman, R; Vasselli, J R; Stetler-Stevenson, W G; Kaelin, W G; Linehan, W M; Klausner, R D; Gnarra, J R; Vande Woude, G F

    1999-09-01

    Loss of function in the von Hippel-Lindau (VHL) tumor suppressor gene occurs in familial and most sporadic renal cell carcinomas (RCCs). VHL has been linked to the regulation of cell cycle cessation (G(0)) and to control of expression of various mRNAs such as for vascular endothelial growth factor. RCC cells express the Met receptor tyrosine kinase, and Met mediates invasion and branching morphogenesis in many cell types in response to hepatocyte growth factor/scatter factor (HGF/SF). We examined the HGF/SF responsiveness of RCC cells containing endogenous mutated (mut) forms of the VHL protein (VHL-negative RCC) with that of isogenic cells expressing exogenous wild-type (wt) VHL (VHL-positive RCC). We found that VHL-negative 786-0 and UOK-101 RCC cells were highly invasive through growth factor-reduced (GFR) Matrigel-coated filters and exhibited an extensive branching morphogenesis phenotype in response to HGF/SF in the three-dimensional (3D) GFR Matrigel cultures. In contrast, the phenotypes of A498 VHL-negative RCC cells were weaker, and isogenic RCC cells ectopically expressing wt VHL did not respond at all. We found that all VHL-negative RCC cells expressed reduced levels of tissue inhibitor of metalloproteinase 2 (TIMP-2) relative to the wt VHL-positive cells, implicating VHL in the regulation of this molecule. However, consistent with the more invasive phenotype of the 786-0 and UOK-101 VHL-negative RCC cells, the levels of TIMP-1 and TIMP-2 were reduced and levels of the matrix metalloproteinases 2 and 9 were elevated compared to the noninvasive VHL-positive RCC cells. Moreover, recombinant TIMPs completely blocked HGF/SF-mediated branching morphogenesis, while neutralizing antibodies to the TIMPs stimulated HGF/SF-mediated invasion in vitro. Thus, the loss of the VHL tumor suppressor gene is central to changes that control tissue invasiveness, and a more invasive phenotype requires additional genetic changes seen in some but not all RCC lines. These

  8. Inter-rater reliability of surgical reviews for AREN03B2: A COG renal tumor committee study✩

    Science.gov (United States)

    Hamilton, Thomas E.; Barnhart, Douglas; Gow, Kenneth; Ferrer, Fernando; Kandel, Jessica; Glick, Richard; Dasgupta, Roshni; Naranjo, Arlene; He, Ying; Gratias, Eric; Geller, James; Mullen, Elizabeth; Ehrlich, Peter

    2014-01-01

    Purpose The Children's Oncology Group (COG) renal tumor study (AREN03B2) requires real-time central review of radiology, pathology, and the surgical procedure to determine appropriate risk-based therapy. The purpose of this study was to determine the inter-rater reliability of the surgical reviews. Methods Of the first 3200 enrolled AREN03B2 patients, a sample of 100 enriched for blood vessel involvement, spill, rupture, and lymph node involvement was selected for analysis. The surgical assessment was then performed independently by two blinded surgical reviewers and compared to the original assessment, which had been completed by another of the committee surgeons. Variables assessed included surgeon-determined local tumor stage, overall disease stage, type of renal procedure performed, presence of tumor rupture, occurrence of intraoperative tumor spill, blood vessel involvement, presence of peritoneal implants, and interpretation of residual disease. Inter-rater reliability was measured using the Fleiss' Kappa statistic two-sided hypothesis tests (Kappa, p-value). Results Local tumor stage correlated in all 3 reviews except in one case (Kappa = 0.9775, p < 0.001). Similarly, overall disease stage had excellent correlation (0.9422, p < 0.001). There was strong correlation for type of renal procedure (0.8357, p < 0.001), presence of tumor rupture (0.6858, p < 0.001), intraoperative tumor spill (0.6493, p < 0.001), and blood vessel involvement (0.6470, p < 0.001). Variables that had lower correlation were determination of the presence of peritoneal implants (0.2753, p < 0.001) and interpretation of residual disease status (0.5310, p < 0.001). Conclusion The inter-rater reliability of the surgical review is high based on the great consistency in the 3 independent review results. This analysis provides validation and establishes precedent for real-time central surgical review to determine treatment assignment in a risk-based stratagem for multimodal cancer therapy

  9. Impact of renal failure on the tumor markers of mesothelioma, N-ERC/mesothelin and osteopontin.

    Science.gov (United States)

    Shiomi, Kazu; Shiomi, Satoko; Ishinaga, Yuji; Sakuraba, Motoki; Hagiwara, Yoshiaki; Miyashita, Kazuya; Maeda, Masahiro; Suzuki, Kenji; Takahashi, Kazuhisa; Hino, Okio

    2011-04-01

    Knowledge of the characteristics and effective use of tumour markers for mesothelioma is essential for early-stage diagnosis of mesothelioma. We examined whether renal dysfunction influences blood concentrations of promising new tumour markers, N-ERC/mesothelin (N-ERC) and osteopontin (OPN), to an important degree. Levels of serum N-ERC and plasma OPN in 32 patients with chronic renal dysfunction, 22 of whom were on hemodialysis (CKD group), and 102 healthy volunteers were measured. Serum concentrations of N-ERC and plasma concentrations of OPN in the CKD group were significantly higher than those in volunteers, regardless of diabetes status and age. Blood concentrations of these markers increased as renal function decreased. N-ERC and OPN concentrations are significantly influenced by renal function. Therefore, the extent of renal failure must be considered when inferring the existence of tumours and chemotherapeutic response from the values of these markers in routine practice.

  10. Synthetic Renal Capsule Tailoring: A Novel Renorrhaphy Technique After Subcapsular Dissection of Renal Tumors with Severe Adherent Perirenal Fat During Laparoscopic Partial Nephrectomy.

    Science.gov (United States)

    Vitagliano, Gonzalo; Lopez, Francisco; Guglielmi, Juan Manuel; Mieggi, Mauro; Blas, Leandro; Rico, Luis; Ameri, Carlos

    2017-07-01

    Multiple factors can account for surgical complexity during laparoscopic partial nephrectomy (LPN); severe adhesion of perirenal fat (PRF) is a crucial one. Consequent renal decapsulation can deem renorrhaphy a very challenging task. We propose a novel technique (synthetic renal capsule tailoring [SYRCT]) to facilitate renorrhaphy in decapsulated kidneys and suggest early decapsulation as a safe option in cases with severe PRF. We retrospectively analyzed perioperative results of this novel technique performed in cases with severe PRF. All cases were classified as high grade in the Mayo Adhesive Probability (MAP) score. A total of six cases were performed with the SYRCT technique. All patients were male with a mean age of 70 years (62-76 years) and mean body mass index of 30 kg/m 2 (23.66-33.86). Of the six cases, five were T1a and 1 T1b. Mean tumor size was 2.83 cm (range 1.2-6 cm). Mean operative time was 121 minutes (range 74-150 minutes); mean warm ischemia time was 17.2 minutes (range 13-25 minutes). Mean operative bleeding was 128 mL (range 50-250 mL). Mean hospital stay was 2.3 days. There were no surgical complications greater or equal to Clavien II. Performing complete renal decapsulation with subcapsular dissection and SYRCT in cases with high MAP score and severe PRF is safe and reproducible. Using this novel technique, we were able to obtain perioperative results comparable to the ones we found in LPN with normal PRF.

  11. Tumorer

    DEFF Research Database (Denmark)

    Prause, J.U.; Heegaard, S.

    2005-01-01

    oftalmologi, øjenlågstumorer, conjunctivale tumorer, malignt melanom, retinoblastom, orbitale tumorer......oftalmologi, øjenlågstumorer, conjunctivale tumorer, malignt melanom, retinoblastom, orbitale tumorer...

  12. Clinicopathologic factors identify sporadic mismatch repair-defective colon cancers

    DEFF Research Database (Denmark)

    Halvarsson, Britta; Anderson, Harald; Domanska, Katarina

    2008-01-01

    Identification of sporadic mismatch repair (MMR)-defective colon cancers is increasingly demanded for decisions on adjuvant therapies. We evaluated clinicopathologic factors for the identification of these prognostically favorable tumors. Histopathologic features in 238 consecutive colon cancers...... and excluded 61.5% of the tumors from MMR testing. This clinicopathologic index thus successfully selects MMR-defective colon cancers. Udgivelsesdato: 2008-Feb...

  13. Metastatic Renal Cell Carcinoma versus Pancreatic Neuroendocrine Tumor in von Hippel-Lindau Disease: Treatment with Interleukin-2

    Directory of Open Access Journals (Sweden)

    Christopher Williams

    2005-01-01

    Full Text Available Differentiating between clear cell neuroendocrine tumor (NET of the pancreas and renal cell carcinoma (RCC metastatic to the pancreas can be challenging in patients with von Hippel-Lindau disease (VHL. The clear cell features of both NET and RCC in VHL patients may lead to misdiagnosis, inaccurate staging, and alternative treatment. We present a patient in which this occurred. As clear cell NETs closely resembling metastatic RCC are distinctive neoplasms of VHL and metastatic RCC to the pancreas in the VHL population is rare, careful pathologic examination should be performed prior to subjecting patients to definitive surgical or medical therapies.

  14. Comprehensive Molecular Characterization of Papillary Renal-Cell Carcinoma.

    Science.gov (United States)

    Linehan, W Marston; Spellman, Paul T; Ricketts, Christopher J; Creighton, Chad J; Fei, Suzanne S; Davis, Caleb; Wheeler, David A; Murray, Bradley A; Schmidt, Laura; Vocke, Cathy D; Peto, Myron; Al Mamun, Abu Amar M; Shinbrot, Eve; Sethi, Anurag; Brooks, Samira; Rathmell, W Kimryn; Brooks, Angela N; Hoadley, Katherine A; Robertson, A Gordon; Brooks, Denise; Bowlby, Reanne; Sadeghi, Sara; Shen, Hui; Weisenberger, Daniel J; Bootwalla, Moiz; Baylin, Stephen B; Laird, Peter W; Cherniack, Andrew D; Saksena, Gordon; Haake, Scott; Li, Jun; Liang, Han; Lu, Yiling; Mills, Gordon B; Akbani, Rehan; Leiserson, Mark D M; Raphael, Benjamin J; Anur, Pavana; Bottaro, Donald; Albiges, Laurence; Barnabas, Nandita; Choueiri, Toni K; Czerniak, Bogdan; Godwin, Andrew K; Hakimi, A Ari; Ho, Thai H; Hsieh, James; Ittmann, Michael; Kim, William Y; Krishnan, Bhavani; Merino, Maria J; Mills Shaw, Kenna R; Reuter, Victor E; Reznik, Ed; Shelley, Carl S; Shuch, Brian; Signoretti, Sabina; Srinivasan, Ramaprasad; Tamboli, Pheroze; Thomas, George; Tickoo, Satish; Burnett, Kenneth; Crain, Daniel; Gardner, Johanna; Lau, Kevin; Mallery, David; Morris, Scott; Paulauskis, Joseph D; Penny, Robert J; Shelton, Candace; Shelton, W Troy; Sherman, Mark; Thompson, Eric; Yena, Peggy; Avedon, Melissa T; Bowen, Jay; Gastier-Foster, Julie M; Gerken, Mark; Leraas, Kristen M; Lichtenberg, Tara M; Ramirez, Nilsa C; Santos, Tracie; Wise, Lisa; Zmuda, Erik; Demchok, John A; Felau, Ina; Hutter, Carolyn M; Sheth, Margi; Sofia, Heidi J; Tarnuzzer, Roy; Wang, Zhining; Yang, Liming; Zenklusen, Jean C; Zhang, Jiashan; Ayala, Brenda; Baboud, Julien; Chudamani, Sudha; Liu, Jia; Lolla, Laxmi; Naresh, Rashi; Pihl, Todd; Sun, Qiang; Wan, Yunhu; Wu, Ye; Ally, Adrian; Balasundaram, Miruna; Balu, Saianand; Beroukhim, Rameen; Bodenheimer, Tom; Buhay, Christian; Butterfield, Yaron S N; Carlsen, Rebecca; Carter, Scott L; Chao, Hsu; Chuah, Eric; Clarke, Amanda; Covington, Kyle R; Dahdouli, Mahmoud; Dewal, Ninad; Dhalla, Noreen; Doddapaneni, Harsha V; Drummond, Jennifer A; Gabriel, Stacey B; Gibbs, Richard A; Guin, Ranabir; Hale, Walker; Hawes, Alicia; Hayes, D Neil; Holt, Robert A; Hoyle, Alan P; Jefferys, Stuart R; Jones, Steven J M; Jones, Corbin D; Kalra, Divya; Kovar, Christie; Lewis, Lora; Li, Jie; Ma, Yussanne; Marra, Marco A; Mayo, Michael; Meng, Shaowu; Meyerson, Matthew; Mieczkowski, Piotr A; Moore, Richard A; Morton, Donna; Mose, Lisle E; Mungall, Andrew J; Muzny, Donna; Parker, Joel S; Perou, Charles M; Roach, Jeffrey; Schein, Jacqueline E; Schumacher, Steven E; Shi, Yan; Simons, Janae V; Sipahimalani, Payal; Skelly, Tara; Soloway, Matthew G; Sougnez, Carrie; Tam, Angela; Tan, Donghui; Thiessen, Nina; Veluvolu, Umadevi; Wang, Min; Wilkerson, Matthew D; Wong, Tina; Wu, Junyuan; Xi, Liu; Zhou, Jane; Bedford, Jason; Chen, Fengju; Fu, Yao; Gerstein, Mark; Haussler, David; Kasaian, Katayoon; Lai, Phillip; Ling, Shiyun; Radenbaugh, Amie; Van Den Berg, David; Weinstein, John N; Zhu, Jingchun; Albert, Monique; Alexopoulou, Iakovina; Andersen, Jeremiah J; Auman, J Todd; Bartlett, John; Bastacky, Sheldon; Bergsten, Julie; Blute, Michael L; Boice, Lori; Bollag, Roni J; Boyd, Jeff; Castle, Erik; Chen, Ying-Bei; Cheville, John C; Curley, Erin; Davies, Benjamin; DeVolk, April; Dhir, Rajiv; Dike, Laura; Eckman, John; Engel, Jay; Harr, Jodi; Hrebinko, Ronald; Huang, Mei; Huelsenbeck-Dill, Lori; Iacocca, Mary; Jacobs, Bruce; Lobis, Michael; Maranchie, Jodi K; McMeekin, Scott; Myers, Jerome; Nelson, Joel; Parfitt, Jeremy; Parwani, Anil; Petrelli, Nicholas; Rabeno, Brenda; Roy, Somak; Salner, Andrew L; Slaton, Joel; Stanton, Melissa; Thompson, R Houston; Thorne, Leigh; Tucker, Kelinda; Weinberger, Paul M; Winemiller, Cynthia; Zach, Leigh Anne; Zuna, Rosemary

    2016-01-14

    Papillary renal-cell carcinoma, which accounts for 15 to 20% of renal-cell carcinomas, is a heterogeneous disease that consists of various types of renal cancer, including tumors with indolent, multifocal presentation and solitary tumors with an aggressive, highly lethal phenotype. Little is known about the genetic basis of sporadic papillary renal-cell carcinoma, and no effective forms of therapy for advanced disease exist. We performed comprehensive molecular characterization of 161 primary papillary renal-cell carcinomas, using whole-exome sequencing, copy-number analysis, messenger RNA and microRNA sequencing, DNA-methylation analysis, and proteomic analysis. Type 1 and type 2 papillary renal-cell carcinomas were shown to be different types of renal cancer characterized by specific genetic alterations, with type 2 further classified into three individual subgroups on the basis of molecular differences associated with patient survival. Type 1 tumors were associated with MET alterations, whereas type 2 tumors were characterized by CDKN2A silencing, SETD2 mutations, TFE3 fusions, and increased expression of the NRF2-antioxidant response element (ARE) pathway. A CpG island methylator phenotype (CIMP) was observed in a distinct subgroup of type 2 papillary renal-cell carcinomas that was characterized by poor survival and mutation of the gene encoding fumarate hydratase (FH). Type 1 and type 2 papillary renal-cell carcinomas were shown to be clinically and biologically distinct. Alterations in the MET pathway were associated with type 1, and activation of the NRF2-ARE pathway was associated with type 2; CDKN2A loss and CIMP in type 2 conveyed a poor prognosis. Furthermore, type 2 papillary renal-cell carcinoma consisted of at least three subtypes based on molecular and phenotypic features. (Funded by the National Institutes of Health.).

  15. Comprehensive Molecular Characterization of Papillary Renal Cell Carcinoma

    Science.gov (United States)

    Linehan, W. Marston; Spellman, Paul T.; Ricketts, Christopher J.; Creighton, Chad J.; Fei, Suzanne S.; Davis, Caleb; Wheeler, David A.; Murray, Bradley A.; Schmidt, Laura; Vocke, Cathy D.; Peto, Myron; Al Mamun, Abu Amar M.; Shinbrot, Eve; Sethi, Anurag; Brooks, Samira; Rathmell, W. Kimryn; Brooks, Angela N.; Hoadley, Katherine A.; Robertson, A. Gordon; Brooks, Denise; Bowlby, Reanne; Sadeghi, Sara; Shen, Hui; Weisenberger, Daniel J.; Bootwalla, Moiz; Baylin, Stephen B.; Laird, Peter W.; Cherniack, Andrew D.; Saksena, Gordon; Haake, Scott; Li, Jun; Liang, Han; Lu, Yiling; Mills, Gordon B.; Akbani, Rehan; Leiserson, Mark D.M.; Raphael, Benjamin J.; Anur, Pavana; Bottaro, Donald; Albiges, Laurence; Barnabas, Nandita; Choueiri, Toni K.; Czerniak, Bogdan; Godwin, Andrew K.; Hakimi, A. Ari; Ho, Thai; Hsieh, James; Ittmann, Michael; Kim, William Y.; Krishnan, Bhavani; Merino, Maria J.; Mills Shaw, Kenna R.; Reuter, Victor E.; Reznik, Ed; Shelley, Carl Simon; Shuch, Brian; Signoretti, Sabina; Srinivasan, Ramaprasad; Tamboli, Pheroze; Thomas, George; Tickoo, Satish; Burnett, Kenneth; Crain, Daniel; Gardner, Johanna; Lau, Kevin; Mallery, David; Morris, Scott; Paulauskis, Joseph D.; Penny, Robert J.; Shelton, Candace; Shelton, W. Troy; Sherman, Mark; Thompson, Eric; Yena, Peggy; Avedon, Melissa T.; Bowen, Jay; Gastier-Foster, Julie M.; Gerken, Mark; Leraas, Kristen M.; Lichtenberg, Tara M.; Ramirez, Nilsa C.; Santos, Tracie; Wise, Lisa; Zmuda, Erik; Demchok, John A.; Felau, Ina; Hutter, Carolyn M.; Sheth, Margi; Sofia, Heidi J.; Tarnuzzer, Roy; Wang, Zhining; Yang, Liming; Zenklusen, Jean C.; Zhang, Jiashan (Julia); Ayala, Brenda; Baboud, Julien; Chudamani, Sudha; Liu, Jia; Lolla, Laxmi; Naresh, Rashi; Pihl, Todd; Sun, Qiang; Wan, Yunhu; Wu, Ye; Ally, Adrian; Balasundaram, Miruna; Balu, Saianand; Beroukhim, Rameen; Bodenheimer, Tom; Buhay, Christian; Butterfield, Yaron S.N.; Carlsen, Rebecca; Carter, Scott L.; Chao, Hsu; Chuah, Eric; Clarke, Amanda; Covington, Kyle R.; Dahdouli, Mahmoud; Dewal, Ninad; Dhalla, Noreen; Doddapaneni, HarshaVardhan; Drummond, Jennifer; Gabriel, Stacey B.; Gibbs, Richard A.; Guin, Ranabir; Hale, Walker; Hawes, Alicia; Hayes, D. Neil; Holt, Robert A.; Hoyle, Alan P.; Jefferys, Stuart R.; Jones, Steven J.M.; Jones, Corbin D.; Kalra, Divya; Kovar, Christie; Lewis, Lora; Li, Jie; Ma, Yussanne; Marra, Marco A.; Mayo, Michael; Meng, Shaowu; Meyerson, Matthew; Mieczkowski, Piotr A.; Moore, Richard A.; Morton, Donna; Mose, Lisle E.; Mungall, Andrew J.; Muzny, Donna; Parker, Joel S.; Perou, Charles M.; Roach, Jeffrey; Schein, Jacqueline E.; Schumacher, Steven E.; Shi, Yan; Simons, Janae V.; Sipahimalani, Payal; Skelly, Tara; Soloway, Matthew G.; Sougnez, Carrie; Tam, Angela; Tan, Donghui; Thiessen, Nina; Veluvolu, Umadevi; Wang, Min; Wilkerson, Matthew D.; Wong, Tina; Wu, Junyuan; Xi, Liu; Zhou, Jane; Bedford, Jason; Chen, Fengju; Fu, Yao; Gerstein, Mark; Haussler, David; Kasaian, Katayoon; Lai, Phillip; Ling, Shiyun; Radenbaugh, Amie; Van Den Berg, David; Weinstein, John N.; Zhu, Jingchun; Albert, Monique; Alexopoulou, Iakovina; Andersen, Jeremiah J; Auman, J. Todd; Bartlett, John; Bastacky, Sheldon; Bergsten, Julie; Blute, Michael L.; Boice, Lori; Bollag, Roni J.; Boyd, Jeff; Castle, Erik; Chen, Ying-Bei; Cheville, John C.; Curley, Erin; Davies, Benjamin; DeVolk, April; Dhir, Rajiv; Dike, Laura; Eckman, John; Engel, Jay; Harr, Jodi; Hrebinko, Ronald; Huang, Mei; Huelsenbeck-Dill, Lori; Iacocca, Mary; Jacobs, Bruce; Lobis, Michael; Maranchie, Jodi K.; McMeekin, Scott; Myers, Jerome; Nelson, Joel; Parfitt, Jeremy; Parwani, Anil; Petrelli, Nicholas; Rabeno, Brenda; Roy, Somak; Salner, Andrew L.; Slaton, Joel; Stanton, Melissa; Thompson, R. Houston; Thorne, Leigh; Tucker, Kelinda; Weinberger, Paul M.; Winemiller, Cythnia; Zach, Leigh Anne; Zuna, Rosemary

    2016-01-01

    Background Papillary renal cell carcinoma, accounting for 15% of renal cell carcinoma, is a heterogeneous disease consisting of different types of renal cancer, including tumors with indolent, multifocal presentation and solitary tumors with an aggressive, highly lethal phenotype. Little is known about the genetic basis of sporadic papillary renal cell carcinoma; no effective forms of therapy for advanced disease exist. Methods We performed comprehensive molecular characterization utilizing whole-exome sequencing, copy number, mRNA, microRNA, methylation and proteomic analyses of 161 primary papillary renal cell carcinomas. Results Type 1 and Type 2 papillary renal cell carcinomas were found to be different types of renal cancer characterized by specific genetic alterations, with Type 2 further classified into three individual subgroups based on molecular differences that influenced patient survival. MET alterations were associated with Type 1 tumors, whereas Type 2 tumors were characterized by CDKN2A silencing, SETD2 mutations, TFE3 fusions, and increased expression of the NRF2-ARE pathway. A CpG island methylator phenotype (CIMP) was found in a distinct subset of Type 2 papillary renal cell carcinoma characterized by poor survival and mutation of the fumarate hydratase (FH) gene. Conclusions Type 1 and Type 2 papillary renal cell carcinomas are clinically and biologically distinct. Alterations in the MET pathway are associated with Type 1 and activation of the NRF2-ARE pathway with Type 2; CDKN2A loss and CIMP in Type 2 convey a poor prognosis. Furthermore, Type 2 papillary renal cell carcinoma consists of at least 3 subtypes based upon molecular and phenotypic features. PMID:26536169

  16. A Rare Renal Epithelial Tumor: Mucinous Cystadenocarcinoma Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Abdulkadir Tepeler

    2011-01-01

    Full Text Available Primary renal mucinous cystadenocarcinoma is a very rare lesion of kidney which originates from the metaplasia of the renal pelvic uroepithelium. Only one case with primary mucinous cystadenocarcinoma has been reported in the English literature. We report second case of mucinous cystadenocarcinoma which was radiologically classified as type-IIF Bosniak cyst in peripheral localization. We aimed to present this extreme and unusual entity with its radiological, surgical, and pathologic aspects under the light of literature.

  17. Novel multiple endocrine neoplasia type 1 variations in patients with sporadic primary hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    S Birla

    2016-01-01

    Full Text Available Background and Objectives: Primary hyperparathyroidism (PHPT can occur either as a sporadic case or in association with syndromes such as multiple endocrine neoplasia. Multiple endocrine neoplasia type 1 (MEN1 is a rare autosomal-dominant disease resulting from mutations in MEN1 gene encoding a 621 amino acid long tumor suppressor protein “menin.” We report here the results of MEN1 screening in 31 patients diagnosed with sporadic PHPT. Materials and Methods: Diagnosis of sporadic PHPT was made when blood urea and serum creatinine were normal, serum parathyroid hormone was high, and parathyroid enlargement could be localized on ultrasound and/or parathyroid scan. A total of 31 patients and 50 healthy volunteers were recruited for molecular analysis after taking informed consent. Results: Major symptoms at presentation were bone pain, fatigue, muscle weakness, and renal stones. Molecular genetic analysis revealed the presence of two novel intronic variations, c. 913-79T>A and c. 784-129T>A which by human splicing finder are predicted to cause potential alteration of splicing by either activating an intronic cryptic acceptor site or converting a conserved exonic splicing silencer sequence to an exonic splicing enhancer site. Apart from these, two reported polymorphisms rs144677807 and rs669976 were seen only in patients and none of the controls. Other reported polymorphisms rs2071313 and rs654440 were identified both in controls and patients. Conclusions: This is the first study of MEN1 gene screening in sporadic PHPT in India reporting on the clinical and genetic findings, wherein two novel intronic variations c. 913-79T>A and c. 784-129T>A were identified showing their possible role in disease causation.

  18. Expression and clinical value of the soluble major histocompatibility complex class I-related chain A molecule in the serum of patients with renal tumors.

    Science.gov (United States)

    Zhao, Y-K; Jia, C-M; Yuan, G-J; Liu, W; Qiu, Y; Zhu, Q-G

    2015-06-29

    We investigated the expression and clinical value of the soluble major histocompatibility complex class I-related chain A (sMICA) molecule in the serum of patients with renal tumors. Sixty patients diagnosed with renal tumors were enrolled in the experimental group, whereas 20 healthy volunteers served as the control group. The sMICA levels were measured via enzyme-linked immunosorbent assay, and the results were analyzed in combination with data from pathol-ogy examination. The experimental group had a statistically significant higher sMICA level (P < 0.05) than the control group. The sMICA level was higher in patients with malignant tumors than in those with be-nign tumors. We also observed a positive relationship among different tumor-node-metastasis (TNM) pathological stages with more advanced diseases exhibiting higher sMICA levels. As a tumor-associated antigen, MICA has a close relationship with renal tumorigenesis and immune es-cape. Our results indicated that sMICA levels were related to tumor pathol-ogy, TNM stage, and metastasis. Therefore, sMICA might be a potential marker for tumor characteristics, prognosis, and recurrence prediction.

  19. ERK5/BMK1 Is a Novel Target of the Tumor Suppressor VHL: Implication in Clear Cell Renal Carcinoma

    Directory of Open Access Journals (Sweden)

    Laura Arias-González

    2013-06-01

    Full Text Available Extracellular signal-regulated kinase 5 (ERK5, also known as big mitogen-activated protein kinase (MAPK 1, is implicated in a wide range of biologic processes, which include proliferation or vascularization. Here, we show that ERK5 is degraded through the ubiquitin-proteasome system, in a process mediated by the tumor suppressor von Hippel-Lindau (VHL gene, through a prolyl hydroxylation-dependent mechanism. Our conclusions derive from transient transfection assays in Cos7 cells, as well as the study of endogenous ERK5 in different experimental systems such as MCF7, HMEC, or Caki-2 cell lines. In fact, the specific knockdown of ERK5 in pVHL-negative cell lines promotes a decrease in proliferation and migration, supporting the role of this MAPK in cellular transformation. Furthermore, in a short series of fresh samples from human clear cell renal cell carcinoma, high levels of ERK5 correlate with more aggressive and metastatic stages of the disease. Therefore, our results provide new biochemical data suggesting that ERK5 is a novel target of the tumor suppressor VHL, opening a new field of research on the role of ERK5 in renal carcinomas.

  20. siRNA-mediated Erc gene silencing suppresses tumor growth in Tsc2 mutant renal carcinoma model.

    Science.gov (United States)

    Imamura, Osamu; Okada, Hiroaki; Takashima, Yuuki; Zhang, Danqing; Kobayashi, Toshiyuki; Hino, Okio

    2008-09-18

    Silencing of gene expression by small interfering RNAs (siRNAs) is rapidly becoming a powerful tool for genetic analysis and represents a potential strategy for therapeutic product development. However, there are no reports of systemic delivery of siRNAs for stable treatment except short hairpin RNAs (shRNAs). On the other hand, there are many reports of systemic delivery of siRNAs for transient treatment using liposome carriers and others. With regard to shRNAs, a report showed fatality in mice due to oversaturation of cellular microRNA/short hairpin RNA pathways. Therefore, we decided to use original siRNA microspheres instead of shRNA for stable treatment of disease. In this study, we designed rat-specific siRNA sequences for Erc/mesothelin, which is a tumor-specific gene expressed in the Eker (Tsc2 mutant) rat model of hereditary renal cancer and confirmed the efficacy of gene silencing in vitro. Then, by using siRNA microspheres, we found that the suppression of Erc/mesothelin caused growth inhibition of Tsc2 mutant renal carcinoma cells in tumor implantation experiments in mice.

  1. Small renal masses: The molecular markers associated with outcome of patients with kidney tumors 7 cm or less

    Science.gov (United States)

    Spirina, L. V.; Usynin, Y. A.; Kondakova, I. V.; Yurmazov, Z. A.; Slonimskaya, E. M.; Pikalova, L. V.

    2016-08-01

    The investigation of molecular mechanisms of tumor cell behavior in small renal masses is required to achieve the better cancer survival. The aim of the study is to find molecular markers associated with outcome of patients with kidney tumors 7 cm or less. A homogenous group of 20 patients T1N0M0-1 (mean age 57.6 ± 2.2 years) with kidney cancer was selected for the present analysis. The content of transcription and growth factors was determined by ELISA. The levels of AKT-mTOR signaling pathway components were measured by Western blotting analysis. The molecular markers associated with unfavorable outcome of patients with kidney tumors 7 cm or less were high levels of NF-kB p50, NF-kB p65, HIF-1, HIF-2, VEGF and CAIX. AKT activation with PTEN loss also correlated with the unfavorable outcome of kidney cancer patients with tumor size 7 cm or less. It is observed that the biological features of kidney cancer could predict the outcome of patients.

  2. Contrast-Enhanced Ultrasound-Guided Radiofrequency Ablation of Renal Tumors

    Science.gov (United States)

    O’Neal, Dan; Cohen, Tal; Peterson, Cynthia

    2018-01-01

    Although only limited long-term studies evaluating thermal ablation of renal masses have been performed, it appears that thermal ablation has a comparable 5-year success rate to that of partial or total nephrectomy. This technique is often used in patients who are not good candidates for partial or total nephrectomy. Contrast-enhanced ultrasound (CEUS) has been recently approved by the Food and Drug Administration for characterization of focal liver lesions in adults and pediatric patients. CEUS can be used off label for renal applications and has been used for years in Europe and Asia. It has several advantages over contrast-enhanced computed tomography for use as the technique to guide and evaluate efficacy of thermal ablation of renal masses. These include the ability to visualize small amounts of enhancement, repeat dosing to evaluate efficacy of an ablation during a procedure, thin slice thickness, and real-time visualization. Ultrasound contrast is also non-nephrotoxic and non-hepatotoxic, allowing evaluation of patients with renal insufficiency. This article reviews the use of CEUS for the guidance and follow-up of thermal ablative procedures of renal masses. PMID:29468107

  3. Combination Chemotherapy and Surgery in Treating Young Patients With Wilms Tumor

    Science.gov (United States)

    2017-11-30

    Adult Renal Wilms Tumor; Beckwith-Wiedemann Syndrome; Childhood Renal Wilms Tumor; Diffuse Hyperplastic Perilobar Nephroblastomatosis; Hemihypertrophy; Stage I Renal Wilms Tumor; Stage II Renal Wilms Tumor; Stage III Renal Wilms Tumor; Stage IV Renal Wilms Tumor; Stage V Renal Wilms Tumor

  4. Solid renal tumor severity in von Hippel Lindau disease is related to germline deletion length and location.

    Science.gov (United States)

    Maranchie, Jodi K; Afonso, Anoushka; Albert, Paul S; Kalyandrug, Sivaram; Phillips, John L; Zhou, Shubo; Peterson, James; Ghadimi, Bijan M; Hurley, Katheen; Riss, Joseph; Vasselli, James R; Ried, Thomas; Zbar, Berton; Choyke, Peter; Walther, McClellan M; Klausner, Richard D; Linehan, W Marston

    2004-01-01

    von Hippel Lindau disease (VHL) is an autosomal dominant familial cancer syndrome linked to alteration of the VHL tumor suppressor gene. Affected patients are predisposed to develop pheochromocytomas and cystic and solid tumors of the kidney, CNS, pancreas, retina, and epididymis. However, organ involvement varies considerably among families and has been shown to correlate with the underlying germline alteration. Clinically, we observed a paradoxically lower prevalence of renal cell carcinoma (RCC) in patients with complete germline deletion of VHL. To determine if a relationship existed between the type of VHL deletion and disease, we retrospectively evaluated 123 patients from 55 families with large germline VHL deletions, including 42 intragenic partial deletions and 13 complete VHL deletions, by history and radiographic imaging. Each individual and family was scored for cystic or solid involvement of CNS, pancreas, and kidney, and for pheochromocytoma. Germline deletions were mapped using a combination of fluorescent in situ hybridization (FISH) and quantitative Southern and Southern blot analysis. An age-adjusted comparison demonstrated a higher prevalence of RCC in patients with partial germline VHL deletions relative to complete deletions (48.9 vs. 22.6%, p=0.007). This striking phenotypic dichotomy was not seen for cystic renal lesions or for CNS (p=0.22), pancreas (p=0.72), or pheochromocytoma (p=0.34). Deletion mapping revealed that development of RCC had an even greater correlation with retention of HSPC300 (C3orf10), located within the 30-kb region of chromosome 3p, immediately telomeric to VHL (52.3 vs. 18.9%, p <0.001), suggesting the presence of a neighboring gene or genes critical to the development and maintenance of RCC. Careful correlation of genotypic data with objective phenotypic measures will provide further insight into the mechanisms of tumor formation. Copyright 2003 Wiley-Liss, Inc.

  5. Comparison of diagnostic performance of CT and MRI for abdominal staging of pediatric renal tumors: a report from the Children's Oncology Group

    Energy Technology Data Exchange (ETDEWEB)

    Servaes, Sabah [Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Khanna, Geetika [Washington University School of Medicine, Pediatric Radiology, St. Louis Children' s Hospital, Mallinckrodt Institute for Radiology, 510 S. Kingshighway, Campus Box 8131-MIR, St. Louis, MO (United States); Naranjo, Arlene [University of Florida, Department of Biostatistics, Gainesville, FL (United States); Geller, James I. [Cincinnati Children' s Hospital Medical Center, Division of Oncology, Cincinnati, OH (United States); Ehrlich, Peter F. [University of Michigan, Department of Surgery, C.S. Mott Children' s Hospital, Ann Arbor, MI (United States); Gow, Kenneth W. [Seattle Children' s Hospital, Pediatric Surgery, Seattle, WA (United States); Perlman, Elizabeth J. [Ann and Robert H. Lurie Children' s Hospital of Chicago, Department of Pathology, Chicago, IL (United States); Dome, Jeffrey S. [Children' s National Medical Center, Center for Cancer and Blood Disorders, Washington, DC (United States); Gratias, Eric; Mullen, Elizabeth A. [Harvard University, Dana Farber Cancer Institute and Boston Children' s Hospital, Boston, MA (United States)

    2014-08-19

    CT and MRI are both used for abdominal staging of pediatric renal tumors. The diagnostic performance of the two modalities for local and regional staging of renal tumors has not been systematically evaluated. To compare the diagnostic performance of CT and MRI for local staging of pediatric renal tumors. The study population was derived from the AREN03B2 study of the Children's Oncology Group. Baseline abdominal imaging performed with both CT and MRI within 30 days of nephrectomy was available for retrospective review in 82 renal tumor cases. Each case was evaluated for capsular penetration, lymph node metastasis, tumor thrombus, preoperative tumor rupture, and synchronous contralateral lesions. The surgical and pathological findings at central review were the reference standard. The sensitivity of CT and MRI for detecting capsular penetration was 68.6% and 62.9%, respectively (P = 0.73), while specificity was 86.5% and 83.8% (P = 1.0). The sensitivity of CT and MRI for detecting lymph node metastasis was 76.5% and 52.9% (P = 0.22), and specificity was 90.4% and 92.3% (P = 1.0). Synchronous contralateral lesions were identified by CT in 4/9 cases and by MRI in 7/9 cases. CT and MRI have similar diagnostic performance for detection of lymph node metastasis and capsular penetration. MR detected more contralateral synchronous lesions; however these were present in a very small number of cases. Either modality can be used for initial loco-regional staging of pediatric renal tumors. (orig.)

  6. Comparison of diagnostic performance of CT and MRI for abdominal staging of pediatric renal tumors: a report from the Children's Oncology Group

    International Nuclear Information System (INIS)

    Servaes, Sabah; Khanna, Geetika; Naranjo, Arlene; Geller, James I.; Ehrlich, Peter F.; Gow, Kenneth W.; Perlman, Elizabeth J.; Dome, Jeffrey S.; Gratias, Eric; Mullen, Elizabeth A.

    2015-01-01

    CT and MRI are both used for abdominal staging of pediatric renal tumors. The diagnostic performance of the two modalities for local and regional staging of renal tumors has not been systematically evaluated. To compare the diagnostic performance of CT and MRI for local staging of pediatric renal tumors. The study population was derived from the AREN03B2 study of the Children's Oncology Group. Baseline abdominal imaging performed with both CT and MRI within 30 days of nephrectomy was available for retrospective review in 82 renal tumor cases. Each case was evaluated for capsular penetration, lymph node metastasis, tumor thrombus, preoperative tumor rupture, and synchronous contralateral lesions. The surgical and pathological findings at central review were the reference standard. The sensitivity of CT and MRI for detecting capsular penetration was 68.6% and 62.9%, respectively (P = 0.73), while specificity was 86.5% and 83.8% (P = 1.0). The sensitivity of CT and MRI for detecting lymph node metastasis was 76.5% and 52.9% (P = 0.22), and specificity was 90.4% and 92.3% (P = 1.0). Synchronous contralateral lesions were identified by CT in 4/9 cases and by MRI in 7/9 cases. CT and MRI have similar diagnostic performance for detection of lymph node metastasis and capsular penetration. MR detected more contralateral synchronous lesions; however these were present in a very small number of cases. Either modality can be used for initial loco-regional staging of pediatric renal tumors. (orig.)

  7. Critical appraisal of first-generation renal tumor complexity scoring systems: Creation of a second-generation model of tumor complexity.

    Science.gov (United States)

    Tobert, Conrad M; Shoemaker, Allen; Kahnoski, Richard J; Lane, Brian R

    2015-04-01

    To investigate whether a combination of variables from each nephrometry system improves performance. There are 3 first-generation systems that quantify tumor complexity: R.E.N.A.L. nephrometry score (RNS), preoperative aspects and dimensions used for an anatomical (PADUA) classification (PC), and centrality index (CI). Although each has been subjected to validation and comparative analysis, to our knowledge, no work has been done to combine variables from each method to optimize their performance. Scores were assigned to each of 276 patients undergoing partial nephrectomy (PN) or radical nephrectomy (RN). Individual components of all 3 systems were evaluated in multivariable logistic regression analysis of surgery type (PN vs. RN) and combined into a "second-generation model." In multivariable analysis, each scoring system was a significant predictor of PN vs. RN (Psystems, CI was most highly correlated with surgery type (area under the curve [AUC] = 0.91), followed by RNS (AUC = 0.90) and PC (AUC = 0.88). Each individual component of these scoring systems was also a predictor of surgery type (Psystem (RNS), location along the lateral rim (PC), and centrality (CI). A novel model in which these 4 variables were rescaled outperformed each first-generation system (AUC = 0.91). Optimization of first-generation models of renal tumor complexity results in a novel scoring system, which strongly predicts surgery type. This second-generation model should aid comprehension, but future work is still needed to establish the most clinically useful model. Copyright © 2015 Elsevier Inc. All rights reserved.

  8. Renal cell neoplasias: reversion-inducing cysteine-rich protein with Kazal motifs discriminates tumor subtypes, while extracellular matrix metalloproteinase inducer indicates prognosis.

    Science.gov (United States)

    Rabien, Anja; Stephan, Carsten; Kilic, Ergin; Weichert, Wilko; Kristiansen, Glen; Miller, Kurt; Jung, Klaus; Erbersdobler, Andreas

    2013-10-16

    Matrix metalloproteinases can promote invasion and metastasis, which are very frequent in renal cell carcinoma even at the time of diagnosis. Knowing the reversion-inducing cysteine-rich protein with Kazal motifs (RECK) as an inhibitor of matrix metalloproteinases and the extracellular matrix metalloproteinase inducer (EMMPRIN) protein as inducer, we aimed to determine their expression, localization and possible antagonistic action in the pathogenesis and progression of renal cell tumors in a retrospective study. Tumor and adjacent normal tissues of 395 nephrectomized patients were immunostained for RECK and EMMPRIN on a tissue microarray. RECK strongly decreased in renal cell carcinoma compared to normal counterparts (Wilcoxon signed rank test, P<0.001), and it discriminated tumor entities showing the highest expression in oncocytomas. EMMPRIN, however, could be significantly correlated to pT stage and Fuhrman grading (Spearman's correlation coefficient rs=0.289 and rs=0.382, respectively). Higher expression of EMMPRIN was associated with decreased overall survival in Kaplan-Meier analysis (P<0.001), and the EMMPRIN level could independently predict survival for cases without metastasis and involvement of lymph nodes. Decreased RECK expression was confirmed by Western blotting in tissue of eight normal/tumor matches of patients after radical nephrectomy, whereas the EMMPRIN pattern appeared to be heterogeneous. We propose RECK down regulation in renal cell carcinoma to be an early event that facilitates tumor formation and progression. EMMPRIN, however, as a prognostic tumor marker, increases only when aggressiveness is proceeding and could add an additional step to invasive properties of renal cell carcinoma.

  9. Tumores renais e adrenais com invasão cardíaca: resultados cirúrgicos imediatos em 14 pacientes Tumores renales y adrenales con invasión cardiaca: resultados quirúrgicos inmediatos en 14 pacientes Renal and adrenal tumors with cardiac invasion: immediate surgical results in 14 patients

    Directory of Open Access Journals (Sweden)

    Rafael Fagionato Locali

    2009-03-01

    Full Text Available FUNDAMENTO: A ressecção do trombo tumoral em veia cava inferior (VCI e átrio direito (AD aumenta a sobrevida do paciente com câncer renal/supra-renal. OBJETIVO: Avaliar a conduta cirúrgica do trombo da VCI e AD no tratamento dos tumores renais e supra-renais. MÉTODOS:De janeiro de 1997 a junho de 2007 foram avaliados, retrospectivamente, 14 pacientes tratados cirurgicamente para retirada de trombo em VCI e/ou AD decorrente de tumor renal ou supra-renal. Desses, 64,2% eram do sexo masculino, e havia 42,8% de casos de tumor de Wilms (TW, 28,5% de adenocarcinoma de supra-renal (AS e 28,5% de carcinoma de células claras (CC, com idades médias de 4,5, 60,5 e 2,5 anos, respectivamente. Aspectos epidemiológicos e parâmetros intra e pós-operatórios hospitalar foram avaliados. RESULTADOS: Em todos os casos encontrou-se trombo tumoral em VCI supra-hepática, e em 62,4% o trombo invadiu o AD. A trombectomia foi realizada com o emprego da circulação extracorpórea associada à hipotermia profunda e parada circulatória total em 85,7% dos casos e moderada no restante. Ligou-se a VCI em 7,1% dos pacientes, e reconstruiu-se por rafia em 92,9%. Os tempos de intubação orotraqueal e internação variaram conforme o tipo de tumor. Ocorreram dois óbitos hospitalares no grupo de AS, por parada cardiorrespiratória intra-operatória. CONCLUSÃO: Existe maior número de casos de trombo tumoral em VCI e AD decorrente de TW. Os casos de AS evoluem com mais complicações no pós-operatório, e o prognóstico no pós-operatório hospitalar dos pacientes com TW é melhor.FUNDAMENTO: La resección del trombo tumoral en vena cava inferior (VCI y atrio derecho (AD aumenta la sobrevida del paciente con cáncer renal/ suprarrenal. OBJETIVO: Evaluar la conducta quirúrgica frente al trombo de la VCI y AD en el tratamiento de los tumores renales y suprarrenales. MÉTODOS: De enero de 1997 a junio de 2007, se evaluaron, retrospectivamente, a 14 pacientes tratados

  10. Renal angiomyolipoma

    DEFF Research Database (Denmark)

    Holm-Nielsen, P; Sørensen, Flemming Brandt

    1988-01-01

    Renal angiomyolipoma is a rare lesion composed of smooth muscle cells, adipose tissue and abnormal vessels. It is currently classified as a benign, non-epithelial renal tumor. It has a high incidence in patients suffering from tuberous sclerosis but is more frequently found as an isolated renal...... lesion. Three cases of renal angiomyolipoma, 2 of which underwent perfusion-fixation, were studied by electron microscopy to clarify the cellular composition of this lesion. In the smooth muscle cells abundant accumulation of glycogen was found, whereas the lipocytes disclosed normal ultrastructural......-specific vesicular structures. These findings suggest a secondary vascular damage, i.e. the thickened vessels may not be a primary, integral part of renal angiomyolipoma. Evidence of a common precursor cell of renal angiomyolipoma was not disclosed. It is concluded that renal angiomyolipoma is a hamartoma composed...

  11. Implications of Von Hippel-Lindau Syndrome and Renal Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Kenan Ashouri

    2015-09-01

    Full Text Available Von Hippel-Lindau syndrome (VHLS is a rare hereditary neoplastic disorder caused by mutations in the vhl gene leading to the development of tumors in several organs including the central nervous system, pancreas, kidneys, and reproductive organs. Manifestations of VHLS can present at different ages based on the affected organ and subclass of disease. In the subclasses of VHLS that cause renal disease, renal involvement typically begins closer to the end of the second decade of life and can present in different ways ranging from simple cystic lesions to solid tumors. Mutations in vhl are most often associated with clear cell renal carcinoma, the most common type of renal cancer, and also play a major role in sporadic cases of clear cell renal carcinoma. The recurrent, multifocal nature of this disease presents difficult challenges in the long-term management of patients with VHLS. Optimization of renal function warrants the use of several different approaches common to the management of renal carcinoma such as nephron sparing surgery, enucleation, ablation, and targeted therapies. In VHLS, renal lesions of 3 cm or bigger are considered to have metastatic potential and even small lesions often harbor malignancy. Many of the aspects of management revolve around optimizing both oncologic outcome and long-term renal function. As new surgical strategies and targeted therapies develop, the management of this complex disease evolves.  This review will discuss the key aspects of the current management of VHLS.

  12. [Differences in sports participation for children and adolescents with solitary kidney due to renal tumors across Europe. Time for harmonization].

    Science.gov (United States)

    Spreafico, F; Terenziani, M; Ardissino, G; Calegari, M; Catania, S; Massimino, M

    2015-02-01

    As a result of advances in treatment, almost 90% of children diagnosed with Wilms tumor became long-term survivors, and have a sustainable quality of life. These patients' involvement in sports during their childhood is hopefully increasing too. The cornerstone of renal tumor cure remains radical nephrectomy, however, so survivors live with a solitary kidney. In most European countries and the USA, the involvement in sports of children with a solitary kidney depends on a responsible physician saying a "qualified yes", pending individual assessment. Unlike the case in the rest of Europe, in Italy having only one kidney automatically disqualifies an individual wishing to participate in any organized "competitive" sports carrying some risk of renal trauma, including basketball, soccer and sometime volleyball. This absolute restriction is based on ad hoc Ministerial rulings concerning "Health protection in sport activities". But available data do not seem to support such an absolute limitation on participation in sports based exclusively on the fact of having a single kidney. The sport-specific incidence of kidney injuries has been estimated at 2.3 injuries per million male athlete/exposures for basketball (2.5 for females), and 2.6 for soccer (6.0 for girls). Kidney injuries are significantly more rare than head or spine injuries. This article aims to provide Italian sport medicine specialists and policy-makers with the necessary background so that the current, over-protective "unquestionably no" response can be reconsidered, and converted into a still well-founded, more permissive attitude to the sports activities suitable for any children with a solitary normal kidney.

  13. Early Significant Tumor Volume Reduction After Radiosurgery in Brain Metastases From Renal Cell Carcinoma Results in Long-Term Survival

    International Nuclear Information System (INIS)

    Kim, Wook Ha; Kim, Dong Gyu; Han, Jung Ho; Paek, Sun Ha; Chung, Hyun-Tai; Park, Chul-Kee; Kim, Chae-Yong; Kim, Yong Hwy; Kim, Jin Wook; Jung, Hee-Won

    2012-01-01

    Purpose: To retrospectively evaluate survival of patients with brain metastasis from renal cell carcinoma (RCC) after radiosurgery. Patients and Methods: Between 1998 and 2010, 46 patients were treated with radiosurgery, and the total number of lesions was 99. The mean age was 58.9 years (range, 33–78 years). Twenty-six patients (56.5%) had a single brain metastasis. The mean tumor volume was 3.0 cm 3 (range, 0.01–35.1 cm 3 ), and the mean marginal dose prescribed was 20.8 Gy (range, 12–25 Gy) at the 50% isodose line. A patient was classified into the good-response group when the sum of the volume of the brain metastases decreased to less than 75% of the original volume at a 1-month follow-up evaluation using MRI. Results: As of December 28, 2010, 39 patients (84.8%) had died, and 7 (15.2%) survived. The overall median survival time was 10.0 ± 0.4 months (95% confidence interval, 9.1–10.8). After treatment, local tumor control was achieved in 72 (84.7%) of the 85 tumors assessed using MRI after radiosurgery. The good-response group survived significantly longer than the poor-response group (median survival times of 18.0 and 9.0 months, respectively; p = 0.025). In a multivariate analysis, classification in the good-response group was the only independent prognostic factor for longer survival (p = 0.037; hazard ratio = 0.447; 95% confidence interval, 0.209–0.953). Conclusions: Radiosurgery seems to be an effective treatment modality for patients with brain metastases from RCC. The early significant tumor volume reduction observed after radiosurgery seems to result in long-term survival in RCC patients with brain metastases.

  14. Síndrome de lisis tumoral en un paciente con cáncer de riñón tratado con sunitinib Tumor lysis syndrome in a patient with a renal carcinoma treated with sunitinib

    Directory of Open Access Journals (Sweden)

    Ezequiel Rodríguez-Reimúndes

    2011-04-01

    Full Text Available El síndrome de lisis tumoral (SLT es un trastorno metabólico que ocurre como consecuencia de una destrucción celular masiva. Se caracteriza por la presencia de hiperuricemia, hiperfosfatemia, hipocalcemia e hiperkalemia, y predispone al desarrollo de insuficiencia renal aguda. En la mayoría de los casos el SLT ocurre luego de instaurarse un tratamiento antitumoral y es más frecuente en tumores de alto grado de malignidad y alta sensibilidad a la quimioterapia. Presentamos el caso de un paciente con diagnóstico de cáncer de riñón recidivado que presenta un SLT e insuficiencia renal aguda luego de iniciar tratamiento con sunitinib.The tumor mor lysis syndrome (TLS is a metabolic disorder resulting from a massive tumor breakdown. It is characterized by hyperuricemia, hyperphosphatemia, hypocalcemia and hyperkalemia and predisposes to acute renal failure. TLS usually occurs after the initiation of cytotoxic therapy and is more frequent in the case of neoplasias with a high proliferative rate or that are highly chemo-sensitive. We report the case of a man with a recurrent kidney cancer who presented with a TLS and acute renal failure after initiation of sunitinib.

  15. Hypermethylation of the 16q23.1 Tumor Suppressor Gene ADAMTS18 in Clear Cell Renal Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Ben Xu

    2015-01-01

    Full Text Available To identify tumor suppressor genes (TSGs silenced by hypermethylation and discover new epigenetic biomarkers for early cancer detection. ADAMTS18, located at 16q23.1, has been reported to be a critical TSG in multiple primary tumors; however, this has not yet been verified in clear cell renal cell carcinoma (ccRCC. We explored epigenetic alterations in this gene in ccRCC and analyzed possible clinicopathological associations. We examined ADAMTS18 gene expression and methylation by semi-quantitative reverse transcription PCR (RT-PCR and methylation-specific polymerase chain reaction (MSP in 5 ccRCC-derived cell lines before and after treatment with 5-aza-2'-deoxycytidine (5-AzaC. MSP was further performed for 101 ccRCC primary tumors and 20 adjacent normal tissues. Some cell lines and specimens were examined by subsequent bisulfite genomic sequencing (BGS and real-time PCR. Further, we analyzed the relationship between the ADAMTS18 gene methylation and clinicopathological features, including short-term disease-free survival (DFS, in patients with ccRCC. ADAMTS18 down-regulation and hypermethylation were detected in the ccRCC-derived cell lines using RT-PCR and MSP. Treatment with 5-AzaC reversed the hypermethylation of the ADAMTS18 gene and restored its expression. Hypermethylation was further detected in 44 of 101 (43.6% primary tumors and 3 of 20 (15.0% adjacent normal tissues. However, a significant difference between both groups was observed (p = 0.02. BGS analysis and real-time PCR were subsequently performed to confirm the results of RT-PCR and MSP. Furthermore, the methylation status of ADAMTS18 was not significantly associated with gender, age, location, tumor diameter, pathological stage, nuclear grade or short-term DFS in patients with ccRCC (p > 0.05. The ADAMTS18 gene is often down-regulated by hypermethylation in ccRCC-derived cell lines and primary tumors, indicating its critical role as a TSG in ccRCC. We conclude that ADAMTS18

  16. RENAL CRYOABLATION

    Directory of Open Access Journals (Sweden)

    A. V. Govorov

    2012-01-01

    Full Text Available Renal cryoablation is an alternative minimally-invasive method of treatment for localized renal cell carcinoma. The main advantages of this methodology include visualization of the tumor and the forming of "ice ball" in real time, fewer complications compared with other methods of treatment of renal cell carcinoma, as well as the possibility of conducting cryotherapy in patients with concomitant pathology. Compared with other ablative technologies cryoablation has a low rate of repeat sessions and good intermediate oncological results. The studies of long-term oncological and functional results of renal cryoablation are presently under way.

  17. Renal tumors: evaluation of prognostic factors in 98 cases from a reference hospital in Porto Alegre, Brazil

    Directory of Open Access Journals (Sweden)

    Alexandra Medeiros Souza de Freitas

    2014-02-01

    Full Text Available Introduction: Renal cell carcinoma (RCC is an aggressive disease worldwide. Objective: Study traditional prognostic factors associated with pathological reports and the novel markers survivin and B7-H1 by immunohistochemistry. Methods: In a reference hospital of Porto Alegre, Brazil, we conducted a cross-sectional study of RCC in patients who underwent radical nephrectomy between 2006 and 2009. We selected those who were diagnosed with the most common histologic subtypes: clear cell and papillary RCC. We retrospectively reviewed pathological data to determine traditional prognostic factors, like size, presence of coagulative necrosis, Fuhrman grade and tumor-node metastasis (TNM system. Besides, we performed an immunohistochemistry (IHC study with survivin and B7-H1. Results: Our sample had 98 cases, 90% of the cases were composed by clear cell histologic subtype, 73% were tumors classified as T1 and T2 in the TNM system, most were Fuhrman nuclear grade 2 or 3, and 70% were positive for necrosis. In relation to the new prognostic markers, we found 50 cases positive to survivin and 38 to B7-H1. In this investigation of traditional prognostic markers and new markers we observed that only necrosis was associated with positive results of biomarkers. < 0.001. Conclusion: This finding confirms previous studies that necrosis is an important factor to consider in the prognosis of RCC.

  18. Cyclin D1 genotype and expression in sporadic hemangioblastomas.

    NARCIS (Netherlands)

    Gijtenbeek, J.M.M.; Sprenger, S.H.E.; Franke, B.; Wesseling, P.; Jeuken, J.W.M.

    2005-01-01

    Central nervous system (CNS) hemangioblastomas are highly-vascularized tumors occurring in sporadic form or as a manifestation of von Hippel-Lindau disease (VHL). The VHL protein (pVHL) regulates various target genes, one of which is the CCND1 gene, encoding cyclin D1, a protein that plays a

  19. Single minimum incision endoscopic radical nephrectomy for renal tumors with preoperative virtual navigation using 3D-CT volume-rendering

    Directory of Open Access Journals (Sweden)

    Shioyama Yasukazu

    2010-04-01

    Full Text Available Abstract Background Single minimum incision endoscopic surgery (MIES involves the use of a flexible high-definition laparoscope to facilitate open surgery. We reviewed our method of radical nephrectomy for renal tumors, which is single MIES combined with preoperative virtual surgery employing three-dimensional CT images reconstructed by the volume rendering method (3D-CT images in order to safely and appropriately approach the renal hilar vessels. We also assessed the usefulness of 3D-CT images. Methods Radical nephrectomy was done by single MIES via the translumbar approach in 80 consecutive patients. We performed the initial 20 MIES nephrectomies without preoperative 3D-CT images and the subsequent 60 MIES nephrectomies with preoperative 3D-CT images for evaluation of the renal hilar vessels and the relation of each tumor to the surrounding structures. On the basis of the 3D information, preoperative virtual surgery was performed with a computer. Results Single MIES nephrectomy was successful in all patients. In the 60 patients who underwent 3D-CT, the number of renal arteries and veins corresponded exactly with the preoperative 3D-CT data (100% sensitivity and 100% specificity. These 60 nephrectomies were completed with a shorter operating time and smaller blood loss than the initial 20 nephrectomies. Conclusions Single MIES radical nephrectomy combined with 3D-CT and virtual surgery achieved a shorter operating time and less blood loss, possibly due to safer and easier handling of the renal hilar vessels.

  20. Laparoscopic partial nephrectomy for multiple (four tumors

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    Lessandro Curcio

    Full Text Available ABSTRACT Background Nephron sparing surgery (NSS is well established as the standard of care for most surgical small renal tumors when technically feasible. While the majority of sporadic renal tumors are solitary, multifocal tumors have been reported in 5.4% to 25% of patients with tumors smaller than 5cm. We present a video where we approach, through laparoscopy, four tumors on the same kidney. Case study Male, 58y, went through a routine abdominal ultrasound which showed a 5cm left kidney nodule. His MRI pointed a total of 4 nodules on his left kidney. The aspect suggested a papillary cancer due to high cellularity and low vascularization. The patient was submitted to partial nephrectomy under ischemia to remove the two largest tumors (inferior pole and a resection without clamping of the other two ipsilateral tumors. Result We performed the surgery in 2 stages. In the first one, we approached the 2 tumors located on the inferior pole inducing warm ischemia, whereas in the second stage we resected the 2 remaining tumors using the technique without clamping. The surgery lasted 220 minutes, with 800mL of blood loss, not requiring blood transfusion. Ischemia time was 35 minutes. The histopathological analysis confirmed that the 4 tumors were papillary cancer, with free margins. Conclusion NSS can be performed and should be tried in patients with multiple kidney tumors, preferably through laparoscopy or assisted by robot. It can be made either using or not clamping of the pedicle, depending on the RENAL score.

  1. Mitochondrial ASncmtRNA-1 and ASncmtRNA-2 as potent targets to inhibit tumor growth and metastasis in the RenCa murine renal adenocarcinoma model.

    Science.gov (United States)

    Borgna, Vincenzo; Villegas, Jaime; Burzio, Verónica A; Belmar, Sebastián; Araya, Mariela; Jeldes, Emanuel; Lobos-González, Lorena; Silva, Verónica; Villota, Claudio; Oliveira-Cruz, Luciana; Lopez, Constanza; Socias, Teresa; Castillo, Octavio; Burzio, Luis O

    2017-07-04

    Knockdown of antisense noncoding mitochondrial RNAs (ASncmtRNAs) induces apoptosis in several human and mouse tumor cell lines, but not normal cells, suggesting this approach for a selective therapy against different types of cancer. Here we show that in vitro knockdown of murine ASncmtRNAs induces apoptotic death of mouse renal adenocarcinoma RenCa cells, but not normal murine kidney epithelial cells. In a syngeneic subcutaneous RenCa model, treatment delayed and even reversed tumor growth. Since the subcutaneous model does not reflect the natural microenviroment of renal cancer, we used an orthotopic model of RenCa cells inoculated under the renal capsule. These studies showed inhibition of tumor growth and metastasis. Direct metastasis assessment by tail vein injection of RenCa cells also showed a drastic reduction in lung metastatic nodules. In vivo treatment reduces survivin, N-cadherin and P-cadherin levels, providing a molecular basis for metastasis inhibition. In consequence, the treatment significantly enhanced mouse survival in these models. Our results suggest that the ASncmtRNAs could be potent and selective targets for therapy against human renal cell carcinoma.

  2. Tumor signatures of PTHLH overexpression, high serum calcium, and poor prognosis were observed exclusively in clear cell but not non clear cell renal carcinomas

    International Nuclear Information System (INIS)

    Yao, Masahiro; Murakami, Takayuki; Shioi, Koichi; Mizuno, Nobuhiko; Ito, Hiroki; Kondo, Keiichi; Hasumi, Hisashi; Sano, Futoshi; Makiyama, Kazuhide; Nakaigawa, Noboru; Kishida, Takeshi; Nagashima, Yoji; Yamanaka, Shoji; Kubota, Yoshinobu

    2014-01-01

    High serum calcium (Ca) due to aberrant secretion of tumor parathyroid hormone-like hormone (PTHLH) is a well-known paraneoplastic sign and is associated with poor prognosis in patients with renal cell carcinoma (RCC). However, the status of serum Ca and tumor PTHLH expression have not been verified using the 2004 World Health Organization (WHO) renal tumor classification. We retrospectively reviewed corrected serum Ca levels at initial onset (n = 683) and/or as of recurrence (n = 71) in patients with RCC. We also examined a total of 623 renal parenchymal tumor samples for PTHLH mRNA expressions by quantitative real-time PCR. High serum Ca concomitant with PTHLH overexpression in tumors was observed exclusively in clear cell RCC but not in other non clear cell subtype tumors, including papillary, chromophobe, collecting-duct, unclassified, and other rare subtype RCCs or in benign oncocytomas and angiomyolipomas. In clear cell RCC, PTHLH expression was significantly high in male patients, and was associated with a symptomatic presentation, higher grade, and higher stage cases, whereas it was not associated with VHL gene status. Univariate analyses demonstrated that high PTHLH expression was strongly associated with poor outcome both in overall survival (OS) and disease-free survival (DFS) for patients who underwent standard nephrectomy. Further multivariate Cox analyses revealed that the PTHLH expressions remained as independent prognostic parameters for OS but not for DFS. These data suggest that the previously characterized tumor signatures of high serum Ca due to high PTHLH expression and poor prognosis are clear cell RCC-specific features, whereas these characteristics are rare in non clear cell RCCs

  3. Identification of Molecular Tumor Markers in Renal Cell Carcinomas with TFE3 Protein Expression by RNA Sequencing

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    Dorothee Pflueger

    2013-11-01

    Full Text Available TFE3 translocation renal cell carcinoma (tRCC is defined by chromosomal translocations involving the TFE3 transcription factor at chromosome Xp11.2. Genetically proven TFE3 tRCCs have a broad histologic spectrum with overlapping features to other renal tumor subtypes. In this study,we aimed for characterizing RCC with TFE3 protein expression. Using next-generation whole transcriptome sequencing (RNA-Seq as a discovery tool, we analyzed fusion transcripts, gene expression profile, and somatic mutations in frozen tissue of one TFE3 tRCC. By applying a computational analysis developed to call chimeric RNA molecules from paired-end RNA-Seq data, we confirmed the known TFE3 translocation. Its fusion partner SFPQ has already been described as fusion partner in tRCCs. In addition, an RNAread-through chimera between TMED6 and COG8 as well as MET and KDR (VEGFR2 point mutations were identified. An EGFR mutation, but no chromosomal rearrangements, was identified in a control group of five clear cell RCCs (ccRCCs. The TFE3 tRCC could be clearly distinguished from the ccRCCs by RNA-Seq gene expression measurements using a previously reported tRCC gene signature. In validation experiments using reverse transcription-PCR, TMED6-COG8 chimera expression was significantly higher in nine TFE3 translocated and six TFE3-expressing/non-translocated RCCs than in 24 ccRCCs (P<.001 and 22 papillaryRCCs (P<.05-.07. Immunohistochemical analysis of selected genes from the tRCC gene signature showed significantly higher eukaryotic translation elongation factor 1 alpha 2 (EEF1A2 and Contactin 3 (CNTN3 expression in 16 TFE3 translocated and six TFE3-expressing/non-translocated RCCs than in over 200 ccRCCs (P < .0001, both.

  4. Other paradigms: growth rate constants and tumor burden determined using computed tomography data correlate strongly with the overall survival of patients with renal cell carcinoma.

    Science.gov (United States)

    Stein, Wilfred D; Huang, Hui; Menefee, Michael; Edgerly, Maureen; Kotz, Herb; Dwyer, Andrew; Yang, James; Bates, Susan E

    2009-01-01

    In solid tumors, where curative therapies still elude oncologists, novel paradigms are needed to assess the efficacy of new therapies and those already approved. We used radiologic measurements obtained in patients with metastatic renal cell carcinoma enrolled in a phase II study of the epothilone B analog, ixabepilone (Ixempra), to address this issue. Using a novel 2-phase mathematical equation, we used the radiologic measurements to estimate the concomitant rates of tumor regression and growth (regression and growth rate constants). Eighty-one patients were enrolled on the ixabepilone trial at the time of this analysis. Growth rate constants were determined using computed tomography measurements obtained exclusively while a patient was enrolled on study. The growth rate constants of renal cell carcinomas treated with ixabepilone were significantly reduced compared with those of tumors in patients who received placebo in a previous trial. Furthermore, a correlation with overall survival was found for both the growth rate constant and the initial tumor burden; and this correlation was even stronger when both the growth rate constant and the initial tumor burden were combined. The readily amenable mathematical model described herein has potential applications to many tumor types that can be assessed with imaging modalities. Because the growth rate constant seems to be a surrogate for survival, assessment could aid in the evaluation of relative efficacies of different therapies and perhaps in assessing the potential individual benefit of an experimental therapy.

  5. Synchronous clear cell renal cell carcinoma and tubulocystic carcinoma: genetic evidence of independent ontogenesis and implications of chromosomal imbalances in tumor progression

    Directory of Open Access Journals (Sweden)

    Quiroga-Garza Gabriela

    2012-02-01

    Full Text Available Abstract Seven percent of renal cell carcinoma (RCC cases are diagnosed as "unclassified" RCC by morphology. Genetic profiling of RCCs helps define renal tumor subtypes, especially in cases where morphologic diagnosis is inconclusive. This report describes a patient with synchronous clear cell RCC (ccRCC and a tubulocystic renal carcinoma (TCRC in the same kidney, and discusses the pathologic features and genetic profile of both tumors. A 67 year-old male underwent CT scans for an unrelated medical event. Two incidental renal lesions were found and ultimately removed by radical nephrectomy. The smaller lesion had multiple small cystic spaces lined by hobnail cells with high nuclear grade separated by fibrous stroma. This morphology and the expression of proximal (CD10, AMACR and distal tubule cell (CK19 markers by immunohistochemistry supported the diagnosis of TCRC. The larger lesion was a typical ccRCC, with Fuhrman's nuclear grade 3 and confined to the kidney. Molecular characterization of both neoplasms using virtual karyotyping was performed to assess relatedness of these tumors. Low grade areas (Fuhrman grade 2 of the ccRCC showed loss of 3p and gains in chromosomes 5 and 7, whereas oncocytic areas displayed additional gain of 2p and loss of 10q; the high grade areas (Fuhrman grade 3 showed several additional imbalances. In contrast, the TCRC demonstrated a distinct profile with gains of chromosomes 8 and 17 and loss of 9. In conclusion, ccRCC and TCRC show distinct genomic copy number profiles and chromosomal imbalances in TCRC might be implicated in the pathogenesis of this tumor. Second, the presence of a ccRCC with varying degrees of differentiation exemplifies the sequence of chromosomal imbalances acquired during tumor progression. Virtual Slides The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1790525735655283

  6. Wilms' tumor 1-associating protein promotes renal cell carcinoma proliferation by regulating CDK2 mRNA stability.

    Science.gov (United States)

    Tang, Jingyuan; Wang, Feng; Cheng, Gong; Si, Shuhui; Sun, Xi; Han, Jie; Yu, Hao; Zhang, Wei; Lv, Qiang; Wei, Ji-Fu; Yang, Haiwei

    2018-02-27

    Wilms' tumor 1-associating protein (WTAP) plays an important role in physiological processes and the development of tumor such as cell cycle regulation. The regulation of cell cycle is mainly dependent on cyclins and cyclin-dependent protein kinases (CDKs). Recent studies have shown that CDKs are closely related to the tumor diagnosis, progression and response to treatment. However, their specific biological roles and related mechanism in renal cell carcinoma (RCC) remain unknown. Quantitative real-time PCR, western blotting and immunohistochemistry were used to detect the expression of WTAP and CDK2. The survival analysis was adopted to explore the association between WTAP expression and the prognosis of RCC. Cells were stably transfected with lentivirus approach and cell proliferation and cell cycle, as well as tumorigenesis in nude mice were performed to assess the effect of WTAP in RCC. RNA immunoprecipitation, Luciferase reporter assay and siRNA were employed to identify the direct binding sites of WTAP with CDK2 transcript. Colony formation assay was conducted to confirm the function of CDK2 in WTAP-induced growth promoting. In RCC cell lines and tissues, WTAP was significantly over-expressed. Compared with patients with low expression of WTAP, patients with high expression of WTAP had lower overall survival rate. Additionally, cell function test indicated that cell proliferation abilities in WTAP over-expressed group were enhanced, while WTAP knockdown showed the opposite results. Subcutaneous xenograft tumor model displayed that knockdown of WTAP could impede tumorigenesis in vivo. Mechanism study exhibited that CDK2 expression was positively associated with the expression of WTAP. Moreover, WTAP stabilized CDK2 transcript to enhance CDK2 expression via binding to 3'-UTR of CDK2 transcript. Additionally, specific inhibitors of CDK2 activity and small interfering RNA (siRNA) of CDK2 expression inhibited WTAP-mediated promotion of proliferation. These

  7. Tumor suppressor FLCN inhibits tumorigenesis of a FLCN-null renal cancer cell line and regulates expression of key molecules in TGF-β signaling

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    Linehan W Marston

    2010-06-01

    Full Text Available Abstract Background Germline mutations in the FLCN gene are responsible for the development of fibrofolliculomas, lung cysts and renal neoplasia in Birt-Hogg-Dube' (BHD syndrome. The encoded protein folliculin (FLCN is conserved across species but contains no classic motifs or domains and its function remains unknown. Somatic mutations or loss of heterozygosity in the remaining wild type copy of the FLCN gene have been found in renal tumors from BHD patients suggesting that FLCN is a classic tumor suppressor gene. Results To examine the tumor suppressor function of FLCN, wild-type or mutant FLCN (H255R was stably expressed in a FLCN-null renal tumor cell line, UOK257, derived from a BHD patient. When these cells were injected into nude mice, tumor development was inversely dependent upon the level of wild-type FLCN expression. We identified genes that were differentially expressed in the cell lines with or without wild-type FLCN, many of which are involved in TGF-β signaling, including TGF-β2 (TGFB2, inhibin β A chain (INHBA, thrombospondin 1 (THBS1, gremlin (GREM1, and SMAD3. In support of the in vitro data, TGFB2, INHBA, THBS1 and SMAD3 expression levels were significantly lower in BHD-associated renal tumors compared with normal kidney tissue. Although receptor mediated SMAD phosphorylation was not affected, basal and maximal TGF-β-induced levels of TGFB2, INHBA and SMAD7 were dramatically reduced in FLCN-null cells compared with FLCN-restored cells. Secreted TGF-β2 and activin A (homo-dimer of INHBA protein levels were also lower in FLCN-null cells compared with FLCN-restored cells. Consistent with a growth suppressive function, activin A (but not TGF-β2 completely suppressed anchorage-independent growth of FLCN-null UOK257 cells. Conclusions Our data demonstrate a role for FLCN in the regulation of key molecules in TGF-β signaling and confirm deregulation of their expression in BHD-associated renal tumors. Thus, deregulation of genes

  8. [Comparison of operative and oncologic results between partial nephrectomy and radiofrequency ablation for treatment of renal tumors in patients older than 75].

    Science.gov (United States)

    Cholley, I; Correas, J M; Masson-Lecomte, A; Sanchez, S; Champy, C; Le Guilchet, T; Ariane, M; Hurel, S; Audenet, F; Thiounn, N; Fontaine, E; Mejean, A; Timsit, M O

    2018-01-01

    Elderly patients represent a growing part of our society for who treatment strategy for localized renal tumors has to be chosen knowing iatrogen effects and renal function morbidity. The aim was to analyze oncological and functional results of nephron sparing surgery (PN) versus radiofrequency ablation (RFA). All patients aged more than 75 treated by partial nephrectomy or radiofrequency ablation between 2007 and 2014 in our centre were included. Patient and tumors data were compared and these criteria were analyzed: survival (overall and without recurrence) and loss of renal function (pre- and postoperative MDRD). In total, 100 patients were included (26 partial nephrectomies, group 1 and 74 radiofrequency ablation, group 2) with a 32-months medium follow-up. Medium age and tumor size were significantly different (respectively, 78 versus 81 years old, P=0.001, 38mm versus 29mm, P=0.003). Perioperative results showed no differences in complications. Transfusion rate and duration of hospital stay were significantly higher in the PN group. Median overall survival were 45 vs. 27 months (P=0.23) for PN and RFA and median recurrence-free survival were 28 vs. 10 months (P=0.34). On a multivariate analysis, operative technique (PN or RFA) were not significantly linked to survival (HR 2.37 [95% CI: 0.66-8.5]), P=0.19. Loss of renal function were 1.5±14mL/min/1.73m 2 for PN and 3±14mL/min/1.73m 2 for RFA (P=0.69). Our study showed better perioperative results for RFA than for PN, without significant different survival. Loss of renal function were little and similar. 4. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  9. Incidental renal neoplasms

    DEFF Research Database (Denmark)

    Rabjerg, Maj; Mikkelsen, Minne Nedergaard; Walter, Steen

    2014-01-01

    On the basis of associations between tumor size, pathological stage, histological subtype and tumor grade in incidentally detected renal cell carcinoma vs symptomatic renal cell carcinoma, we discussed the need for a screening program of renal cell carcinoma in Denmark. We analyzed a consecutive...... series of 204 patients with renal tumors in 2011 and 2012. The tumors were classified according to detection mode: symptomatic and incidental and compared to pathological parameters. Eighty-nine patients (44%) were symptomatic, 113 (55%) were incidental. Information was not available in two patients...

  10. Immunohistochemistry for annexin A10 can distinguish sporadic from Lynch syndrome-associated microsatellite-unstable colorectal carcinoma.

    Science.gov (United States)

    Pai, Reetesh K; Shadrach, Bonnie L; Carver, Paula; Heald, Brandie; Moline, Jessica; Church, James; Kalady, Matthew F; Burke, Carol A; Plesec, Thomas P; Lai, Keith K; Gonzalo, David H; Pai, Rish K

    2014-04-01

    Differentiating sporadic microsatellite-unstable colorectal carcinoma due to MLH1 promoter hypermethylation from Lynch syndrome (LS)-associated tumors due to mutations in mismatch-repair proteins is time consuming, cost intensive, and requires advanced laboratory testing. A mutation in BRAF has been shown to be highly specific for sporadic tumors; however, a significant proportion of sporadic microsatellite-unstable tumors lack BRAF mutations. MLH1 promoter methylation analysis is subsequently used to differentiate LS and sporadic tumors, but both tests require specialized laboratories and are costly. Through previous gene expression profiling of serrated polyps, we identified annexin A10 as a protein highly expressed in sessile serrated adenomas/polyps. As these polyps give rise to the majority of sporadic microsatellite-unstable tumors, we evaluated the ability of annexin A10 expression to discriminate between LS and sporadic tumors. A marked increase in annexin A10 mRNA was observed in sporadic microsatellite-unstable tumors compared with LS tumors (378-fold increase, Pimmunohistochemistry, annexin A10 was expressed in 23/53 (43%) BRAF-mutated and 9/22 (41%) BRAF wild-type sporadic tumors. In contrast, only 3/56 (5%) LS tumors were positive for annexin A10 (Pimmunohistochemistry. Only 1/28 (4%) LS tumors with loss of MLH1 was positive for annexin A10. This patient did not have a deleterious MLH1 mutation but rather germline promoter hypermethylation of MLH1. On the basis of these results, immunohistochemistry for annexin A10 may be a useful marker to distinguish sporadic from LS-associated microsatellite-unstable colon cancer.

  11. Role of presurgical targeted molecular therapy in renal cell carcinoma with an inferior vena cava tumor thrombus

    Directory of Open Access Journals (Sweden)

    Peng C

    2018-04-01

    Full Text Available Cheng Peng,1,* Liangyou Gu,1,* Lei Wang,2 Qingbo Huang,1 Baojun Wang,1 Gang Guo,1 Yang Fan,1 Yu Gao,1 Xin Ma,1 Xu Zhang1 1Department of Urology, State Key Laboratory of Kidney Diseases, Chinese PLA General Hospital, Chinese PLA Medical Academy, Beijing, People’s Republic of China; 2Department of Urology, Chinese PLA 534 Hospital, Luoyang, People’s Republic of China *These authors contributed equally to this work Purpose: The clinical benefit of targeted molecular therapy (TMT in renal cell carcinoma (RCC with an inferior vena cava (IVC tumor thrombus remains controversial. The aim of this study was to investigate the effects of presurgical TMT on the heights and levels of IVC thrombi, and to assess its impact on surgical strategy. Patients and methods: We retrospectively reviewed data from 18 patients with RCC involving IVC tumor thrombi who were treated at our hospital with presurgical TMT followed by an IVC thrombectomy. The changes in heights and levels of the IVC thrombi were compared using computed tomography or magnetic resonance imaging. Clinicopathological factors were also evaluated to assess their association with TMT efficacy. Results: The tumor thrombus levels before TMT were stage I in 1 patient (5.6%, II in 12 patients (66.7%, III in 4 patients (22.2%, and IV in 1 patient (5.6%. After a median of two treatment cycles (range: 1–3, the thrombus height decreased measurably in 11 patients (61.1% with an average shrinkage of 17.7%. The thrombus height remained stable in five patients (27.8% and was enlarged in two (11.1%. Downstaging of the thrombus level occurred in four patients (22.2%; the surgical strategy was modified in three patients (16.7% to avoid cardiopulmonary bypass and complicated liver mobilization under robot-assisted laparoscopy. Furthermore, a higher neutrophil count tended to be associated with a worse clinical TMT-associated outcome (P=0.056. Conclusion: Our data suggest a limited influence of presurgical TMT

  12. Tumor-specific hypermethylation of epigenetic biomarkers, including SFRP1, predicts for poorer survival in patients from the TCGA Kidney Renal Clear Cell Carcinoma (KIRC project.

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    Christopher J Ricketts

    Full Text Available The recent publication of the TCGA Kidney Renal Clear Cell Carcinoma (KIRC project has provided an immense wealth and breadth of data providing an invaluable tool for confirmation and expansion upon previous observations in a large data set containing multiple data types including DNA methylation, somatic mutation, and clinical information. In clear cell renal cell carcinoma (CCRCC many genes have been demonstrated to be epigenetically inactivated by promoter hypermethylated and in a small number of cases to be associated with clinical outcome. This study created two cohorts based on the Illumina BeadChip array used to confirm the frequency of tumor-specific hypermethylation of these published hypermethylated genes, assess the impact of somatic mutation or chromosomal loss and provide the most comprehensive assessment to date of the association of this hypermethylation with patient survival. Hypermethylation of the Fibrillin 2 (FBN2 gene was the most consistent epigenetic biomarker for CCRCC across both cohorts in 40.2% or 52.5% of tumors respectively. Hypermethylation of the secreted frizzled-related protein 1 (SFRP1 gene and the basonuclin 1 (BNC1 gene were both statistically associated with poorer survival in both cohorts (SFRP1 - p = <0.0001 or 0.0010 and BNC1 - p = <0.0001 or 0.0380 and represented better independent markers of survival than tumor stage, grade or dimension in one cohort and tumor stage or dimension in the other cohort. Loss of the SFRP1 protein can potentially activate the WNT pathway and this analysis highlighted hypermethylation of several other WNT pathway regulating genes and demonstrated a poorer survival outcome for patients with somatic mutation of these genes. The success of demethylating drugs in hematological malignances and the current trials in solid tumors suggest that the identification of clinically relevant hypermethylated genes combined with therapeutic advances may improve the effectiveness and

  13. Activation of the PI3K/AKT pathway induces urothelial carcinoma of the renal pelvis: identification in human tumors and confirmation in animal models.

    Science.gov (United States)

    Qian, Chao-Nan; Furge, Kyle A; Knol, Jared; Huang, Dan; Chen, Jindong; Dykema, Karl J; Kort, Eric J; Massie, Aaron; Khoo, Sok Kean; Vanden Beldt, Kristin; Resau, James H; Anema, John; Kahnoski, Richard J; Morreau, Hans; Camparo, Philippe; Comperat, Eva; Sibony, Mathilde; Denoux, Yves; Molinie, Vincent; Vieillefond, Annick; Eng, Charis; Williams, Bart O; Teh, Bin Tean

    2009-11-01

    Urothelial carcinoma of the renal pelvis is a deadly disease with an unclear tumorigenic mechanism. We conducted gene expression profiling on a set of human tumors of this type and identified a phosphatidylinositol 3-kinase (PI3K)/AKT activation expression signature in 76.9% (n = 13) of our samples. Sequence analysis found both activating mutations of PIK3CA (13.6%, n = 22) and loss of heterozygosity at the PTEN locus (25%, n = 8). In contrast, none of the other subtypes of kidney neoplasms (e.g., clear-cell renal cell carcinoma) harbored PIK3CA mutations (n = 87; P elevation of phosphorylated mammalian target of rapamycin (mTOR; 63.6%, n = 11). To confirm the role of the PI3K/AKT pathway in urothelial carcinoma, we generated mice containing biallelic inactivation of Pten in the urogenital epithelia. These mice developed typical renal pelvic urothelial carcinomas, with an incidence of 57.1% in mice older than 1 year. Laser capture microdissection followed by PCR confirmed the deletion of Pten exons 4 and 5 in the animal tumor cells. Immunohistochemical analyses showed increased phospho-mTOR and phospho-S6K levels in the animal tumors. Renal lymph node metastases were found in 15.8% of the animals with urothelial carcinoma. In conclusion, we identified and confirmed an important role for the PI3K/AKT pathway in the development of urothelial carcinoma and suggested that inhibitors of this pathway (e.g., mTOR inhibitor) may serve as effective therapeutic agents.

  14. PLGA nanoparticles for peptide receptor radionuclide therapy of neuroendocrine tumors: a novel approach towards reduction of renal radiation dose.

    Directory of Open Access Journals (Sweden)

    Geetanjali Arora

    Full Text Available BACKGROUND: Peptide receptor radionuclide therapy (PRRT, employed for treatment of neuroendocrine tumors (NETs is based on over-expression of Somatostatin Receptors (SSTRs on NETs. It is, however, limited by high uptake and retention of radiolabeled peptide in kidneys resulting in unnecessary radiation exposure thus causing nephrotoxicity. Employing a nanocarrier to deliver PRRT drugs specifically to the tumor can reduce the associated nephrotoxicity. Based on this, (177Lu-DOTATATE loaded PLGA nanoparticles (NPs were formulated in the present study, as a potential therapeutic model for NETs. METHODOLOGY AND FINDINGS: DOTATATE was labeled with Lutetium-177 ((177Lu (labeling efficiency 98%; R(f∼0.8. Polyethylene Glycol (PEG coated (177Lu-DOTATATE-PLGA NPs (50:50 and 75:25 formulated, were spherical with mean size of 304.5±80.8 and 733.4±101.3 nm (uncoated and 303.8±67.2 and 494.3±71.8 nm (coated for PLGA(50:50 and PLGA(75:25 respectively. Encapsulation efficiency (EE and In-vitro release kinetics for uncoated and coated NPs of PLGA (50:50 & 75:25 were assessed and compared. Mean EE was 77.375±4.98% & 67.885±5.12% (uncoated and 65.385±5.67% & 58.495±5.35% (coated. NPs showed initial burst release between 16.64-21.65% with total 42.83-44.79% over 21 days. The release increased with coating to 20.4-23.95% initially and 60.97-69.12% over 21 days. In-vivo studies were done in rats injected with (177Lu-DOTATATE and (177Lu-DOTATATE-NP (uncoated and PEG-coated by imaging and organ counting after sacrificing rats at different time points over 24 hr post-injection. With (177Lu-DOTATATE, renal uptake of 37.89±10.2%ID/g was observed, which reduced to 4.6±1.97% and 5.27±1.66%ID/g with uncoated and coated (177Lu-DOTATATE-NP. The high liver uptake with uncoated (177Lu-DOTATATE-NP (13.68±3.08% ID/g, reduced to 7.20±2.04%ID/g (p = 0.02 with PEG coating. CONCLUSION: PLGA NPs were easily formulated and modified for desired release properties. PLGA

  15. Individual Bromodomains of Polybromo-1 Contribute to Chromatin Association and Tumor Suppression in Clear Cell Renal Carcinoma.

    Science.gov (United States)

    Porter, Elizabeth G; Dykhuizen, Emily C

    2017-02-17

    The architecture of chromatin is governed, in part, by ATP-dependent chromatin remodelers. These multiprotein complexes contain targeting domains that recognize post-translational marks on histones. One such targeting domain is the bromodomain (BD), which recognizes acetyl-lysines and recruits proteins to sites of acetylation across the genome. Polybromo1 (PBRM1), a subunit of the Polybromo-associated BRG1- or hBRM-associated factors (PBAF) chromatin remodeler, contains six tandem BDs and is frequently mutated in clear cell renal cell carcinoma (ccRCC). Mutations in the PBRM1 gene often lead to the loss of protein expression; however, missense mutations in PBRM1 have been identified and tend to cluster in the BDs, particularly BD2 and BD4, suggesting that individual BDs are critical for PBRM1 function. To study the role of these six BDs, we inactivated each of the six BDs of PBRM1 and re-expressed these mutants in Caki2 cells (ccRCC cells with the loss of function mutation in PBRM1 ). Four of the six BDs abrogated PBRM1 tumor suppressor function, gene regulation, and chromatin affinity with the degree of importance correlating strongly to the rate of missense mutations in patients. Furthermore, we identified BD2 as the most critical for PBRM1 and confirmed BD2-mediated association to histone H3 peptides acetylated at lysine 14 (H3K14Ac), validating the importance of this specific acetylation mark for PBRM1 binding. From these data, we conclude that four of the BDs act together to target PBRM1 to sites on chromatin; when a single BD is mutated, PBRM1 no longer controls gene expression properly, leading to increased cell proliferation. © 2017 by The American Society for Biochemistry and Molecular Biology, Inc.

  16. A new method using multiphoton imaging and morphometric analysis for differentiating chromophobe renal cell carcinoma and oncocytoma kidney tumors

    Science.gov (United States)

    Wu, Binlin; Mukherjee, Sushmita; Jain, Manu

    2016-03-01

    Distinguishing chromophobe renal cell carcinoma (chRCC) from oncocytoma on hematoxylin and eosin images may be difficult and require time-consuming ancillary procedures. Multiphoton microscopy (MPM), an optical imaging modality, was used to rapidly generate sub-cellular histological resolution images from formalin-fixed unstained tissue sections from chRCC and oncocytoma.Tissues were excited using 780nm wavelength and emission signals (including second harmonic generation and autofluorescence) were collected in different channels between 390 nm and 650 nm. Granular structure in the cell cytoplasm was observed in both chRCC and oncocytoma. Quantitative morphometric analysis was conducted to distinguish chRCC and oncocytoma. To perform the analysis, cytoplasm and granules in tumor cells were segmented from the images. Their area and fluorescence intensity were found in different channels. Multiple features were measured to quantify the morphological and fluorescence properties. Linear support vector machine (SVM) was used for classification. Re-substitution validation, cross validation and receiver operating characteristic (ROC) curve were implemented to evaluate the efficacy of the SVM classifier. A wrapper feature algorithm was used to select the optimal features which provided the best predictive performance in separating the two tissue types (classes). Statistical measures such as sensitivity, specificity, accuracy and area under curve (AUC) of ROC were calculated to evaluate the efficacy of the classification. Over 80% accuracy was achieved as the predictive performance. This method, if validated on a larger and more diverse sample set, may serve as an automated rapid diagnostic tool to differentiate between chRCC and oncocytoma. An advantage of such automated methods are that they are free from investigator bias and variability.

  17. An immunophenotypic comparison of metanephric metaplasia of Bowman capsular epithelium with metanephric adenoma, Wilms tumor, and renal development: a case report and review of the literature.

    Science.gov (United States)

    Fischer, Edgar G; Carney, J Aidan; Anderson, Scott R; Klatt, Edward C; Lager, Donna J

    2004-06-01

    Metanephric metaplasia of the parietal epithelium of the Bowman capsule is a rare pathologic finding of unknown pathogenesis that has occurred in patients with widespread malignant neoplasms of various types. We report this finding in a 25-year-old woman with partial expression of the Carney triad who died of a disseminated gastrointestinal stromal tumor, specifically a gastric stromal sarcoma. The metaplasia involved both kidneys diffusely. It originated in the parietal epithelium of the Bowman capsule, extended into the proximal tubules, and focally surrounded the glomeruli in a semicircular manner Immunohistochemical analysis revealed that the cells of metanephric metaplasia expressed the Wilms tumor gene product, bcl-2 protein, and CD57 and cytokeratin 7 and keratin AE1/AE3 focally, but not CD56. This immunophenotype parallels that of metanephric adenoma, Wilms tumor, and nephrogenic rests and overlaps with antigen expression in certain periods of renal development.

  18. MAJOR MOLECULAR GENETIC DRIVERS IN SPORADIC PRIMARY HYPERPARATHYROIDISM.

    Science.gov (United States)

    Arnold, Andrew

    2016-01-01

    Primary hyperparathyroidism is primarily due to a solitary parathyroid adenoma but multi-gland disease, parathyroid carcinoma, and ectopic parathyroid hormone production can occur. Although primary hyperparathyroidism mostly presents sporadically, strong familial predispositions also exist. Much is known about heritable genetic mutations responsible for these syndromes, including multiple endocrine neoplasia types 1 and 2A, hyperparathyroidism-jaw tumor syndrome, and familial hypocalciuric hypercalcemia. Acquired mutations in common sporadic hyperparathyroidism have also been discovered. Here we focus on the most common and well-established genetic drivers: 1) involvement of the oncogene cyclin D1 in human neoplasia was first established in parathyroid adenomas, followed by recognition of its importance in other tumor types including breast cancer and B-lymphoid malignancy; and 2) somatic mutation of the MEN1 gene, first identified as the source of pathogenic germline mutations in patients with familial endocrinopathies, is found in a substantial fraction of non-familial parathyroid adenomas.

  19. MicroRNA-200a-3p suppresses tumor proliferation and induces apoptosis by targeting SPAG9 in renal cell carcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Xinsheng; Jiang, Fuquan; Song, Haitao; Li, Xu; Xian, Jiantao; Gu, Xinquan, E-mail: guxqprofessor@163.com

    2016-02-12

    Sperm-associated antigen 9(SPAG9), as a well-recognized oncogene protein, has a critical effect on renal cell carcinoma (RCC) progression. Our study tried to explore the mediator of miR-200a-3p, a tumor suppressing miRNA on SPAG9 expression and renal cell proliferation and apoptosis. We found the expression of miR-200a-3p was significantly lower in RCC specimens. Based on in vitro assays, we found miR-200a-3p significantly inhibit cancer cell proliferation by inducing apoptosis. In addition, our study uncovered that miR-200a-3p directly regulates oncogenic SPAG9 in 786-O and ACHN cells. Silencing of SPAG9 resulted in significantly decreased in the growth and the cell cycle of the renal cancer cell lines. Understanding of oncogenic SPAG9 regulated by miR-200a-3p might be beneficial to reveal new therapeutic targets for RCC. - Highlights: • MiR-200a-3p is downregulated in renal cell carcinoma. • MiR-200a-3p regulates cell proliferation through inducing apoptosis. • MiR-200a-3p is involved in cell cycle regulation. • SPAG9 is a potential target of miR-200a-3p.

  20. Aberrant gene promoter methylation associated with sporadic multiple colorectal cancer.

    Directory of Open Access Journals (Sweden)

    Victoria Gonzalo

    Full Text Available BACKGROUND: Colorectal cancer (CRC multiplicity has been mainly related to polyposis and non-polyposis hereditary syndromes. In sporadic CRC, aberrant gene promoter methylation has been shown to play a key role in carcinogenesis, although little is known about its involvement in multiplicity. To assess the effect of methylation in tumor multiplicity in sporadic CRC, hypermethylation of key tumor suppressor genes was evaluated in patients with both multiple and solitary tumors, as a proof-of-concept of an underlying epigenetic defect. METHODOLOGY/PRINCIPAL FINDINGS: We examined a total of 47 synchronous/metachronous primary CRC from 41 patients, and 41 gender, age (5-year intervals and tumor location-paired patients with solitary tumors. Exclusion criteria were polyposis syndromes, Lynch syndrome and inflammatory bowel disease. DNA methylation at the promoter region of the MGMT, CDKN2A, SFRP1, TMEFF2, HS3ST2 (3OST2, RASSF1A and GATA4 genes was evaluated by quantitative methylation specific PCR in both tumor and corresponding normal appearing colorectal mucosa samples. Overall, patients with multiple lesions exhibited a higher degree of methylation in tumor samples than those with solitary tumors regarding all evaluated genes. After adjusting for age and gender, binomial logistic regression analysis identified methylation of MGMT2 (OR, 1.48; 95% CI, 1.10 to 1.97; p = 0.008 and RASSF1A (OR, 2.04; 95% CI, 1.01 to 4.13; p = 0.047 as variables independently associated with tumor multiplicity, being the risk related to methylation of any of these two genes 4.57 (95% CI, 1.53 to 13.61; p = 0.006. Moreover, in six patients in whom both tumors were available, we found a correlation in the methylation levels of MGMT2 (r = 0.64, p = 0.17, SFRP1 (r = 0.83, 0.06, HPP1 (r = 0.64, p = 0.17, 3OST2 (r = 0.83, p = 0.06 and GATA4 (r = 0.6, p = 0.24. Methylation in normal appearing colorectal mucosa from patients with multiple and solitary CRC showed no relevant

  1. Computed tomography on renal masses in dogs and cats

    International Nuclear Information System (INIS)

    Yamazoe, Kazuaki; Ohashi, Fumihito; Kadosawa, Tsuyoshi; Nishimura, Ryohei; Sasaki, Nobuo; Takeuchi, Akira.

    1994-01-01

    Computed tomography (CT) was performed on renal tumors (Wilms' tumor and renal cell carcinoma) and renal cysts in dogs and cats. CT images in renal tumors were well correlated with macroscopic findings, and contrast CT images were quite useful in differentiating tumoral regions from non-tumoral ones. On renal cysts, intravenous pyelography and ultrasonography were as effective as CT images in morphological diagnosis, but CT was considered to be superior for evaluating three-dimensional (3-D) relationships in complicated lesions. (author)

  2. Hereditary Kidney Cancer Syndromes and Surgical Management of the Small Renal Mass.

    Science.gov (United States)

    Nguyen, Kevin A; Syed, Jamil S; Shuch, Brian

    2017-05-01

    The management of patients with hereditary kidney cancers presents unique challenges to clinicians. In addition to an earlier age of onset compared with patients with sporadic kidney cancer, those with hereditary kidney cancer syndromes often present with bilateral and/or multifocal renal tumors and are at risk for multiple de novo lesions. This population of patients may also present with extrarenal manifestations, which adds an additional layer of complexity. Physicians who manage these patients should be familiar with the underlying clinical characteristics of each hereditary kidney cancer syndrome and the suggested surgical approaches and recommendations of genetic testing for at-risk individuals. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. Using 18F FDG PET/CT to Detect an occult Mesenchymal Tumor Causing Oncogenic Osteomalacia

    International Nuclear Information System (INIS)

    Seo, Hyo Jung; Choi, Yun Jung; Kim, Hyun Jeong; Jeong, Yong Hyu; Cho, Arthur; Lee, Jae Hoon; Yun, Mijin; Lee, Jong Doo; Kang, Won Jun

    2011-01-01

    Oncogenic osteomalacia is a rare paraneoplastic syndrome characterized by renal phosphate excretion, hypophosphatemia, and osteomalacia. This syndrome is often caused by tumors of mesenchymal origin. Patients with oncogenic osteomalacia have abnormal bone mineralization, resulting in a high frequency of fractures. Tumor resection is the treatment of choice, as it will often correct the metabolic imbalance. Although oncogenic osteomalacia is a potentially curable disease, diagnosis is difficult and often delayed because of the small size and sporadic location of the tumor. Bone scintigraphy and radiography best characterize osteoma lacia; magnetic resonance imaging findings are nonspecific. Here, we report a case of oncogenic osteomalacia secondary to a phosphaturic mesenchymal tumor that was successfully detected by 18F fluorodeoxyglucose positron emission tomography/computed tomography ( 18F FDG PET/CT). This case illustrates the advantages of 18F FDG PET/CT in detecting the occult mesenchymal tumor that causes oncogenic osteomalacia.

  4. Sodium butyrate induces cell death by autophagy and reactivates a tumor suppressor gene DIRAS1 in renal cell carcinoma cell line UOK146.

    Science.gov (United States)

    Verma, Shiv Prakash; Agarwal, Ayushi; Das, Parimal

    2018-04-01

    Sodium butyrate (SB), a histone deacetylase inhibitor, is emerging as a potent anti-cancer drug for different types of cancers. In the present study, anti-cancer activity of SB in Xp11.2 (TFE3) translocated renal cell carcinoma cell line UOK146 was studied. Anti-proliferative effect of SB in renal cell carcinoma (RCC) cell line UOK146 was evaluated by MTT assay and morphological characteristics were observed by phase contrast microscopy which displayed the cell death after SB treatment. SB induces DNA fragmentation and change in nuclear morphology observed by increased sub-G1 region cell population and nuclear blebbings. Cell cycle arrest at G2/M phase was found after SB treatment. UOK146 cell line shows autophagy mode of cell death as displayed by acridine orange staining and flow cytometry analysis. LC3-II, a protein marker of autophagy, was also found to be upregulated after SB treatment. A tumor suppressor gene DIRAS1 was upregulated after SB treatment, displaying its anti-cancer potential at molecular level. These findings suggest that SB could serve as a novel regulator of tumor suppressors and lead to the discovery of novel therapeutics with better and enhanced anti-cancer activity.

  5. Surgical Protocol Violations in Children with Renal Tumors Provides an Opportunity to Improve Pediatric Cancer Care: A Report from the Children's Oncology Group

    Science.gov (United States)

    Ehrlich, Peter F.; Hamilton, Thomas E.; Gow, Kenneth; Barnhart, Douglas; Ferrer, Fernando; Kandel, Jessica; Glick, Richard; Dasgupta, Roshni; Naranjo, Arlene; He, Ying; Perlman, Elizabeth J.; Kalapurakal, John A.; Khanna, Geetika; Dome, Jeffrey S.; Geller, James; Mullen, Elizabeth

    2016-01-01

    Background The purpose of this study was to evaluate the frequency and characteristics of surgical protocol violations (SPV) among children undergoing surgery for renal tumors who were enrolled on the Children's Oncology Group (COG) renal tumor biology and classification study AREN03B2. Methods AREN03B2 opened in February 2006 and as of March 31, 2013, there were 3664 eligible patients. The surgical review forms for 3536 patients with unilateral disease were centrally reviewed for SPVs. The frequency, type, number of violations, institutional prevalence, and quartiles for SPVs were assessed. Results Of the 3536 patients, there were a total of 505 with at least one SPV (564 total SPVs reported), for an overall incidence of 14.28%. The types of SPVs included a lack of lymph node sampling in 365 (64.7%), avoidable spill in 61 (10.8%), biopsy immediately before nephrectomy in 89 (15.8%), an incorrect abdominal incision in 32 (5.7%), and unnecessary resection of organs in 17 (3.0%). The SPVs occurred in 163/215 participating institutions (75.8%). For centers with at least 1 SPV, the mean number of SPVs reported was 3.10 ± 2.39 (mean ± standard deviation). The incidence of protocol violation per institution ranged from 0 to 67%. Centers with an average of ≤1 case/year had an incidence of SPVs of 12.2 ± 3.8%, those with an average of >1 to 0.05). Conclusion SPVs that potentially result in additional exposure to chemotherapy and radiation therapy are not uncommon in children undergoing resection of renal malignancies. PMID:27229358

  6. Adult onset sporadic ataxias: a diagnostic challenge

    Directory of Open Access Journals (Sweden)

    Orlando Graziani Povoas Barsottini

    2014-03-01

    Full Text Available Patients with adult onset non-familial progressive ataxia are classified in sporadic ataxia group. There are several disease categories that may manifest with sporadic ataxia: toxic causes, immune-mediated ataxias, vitamin deficiency, infectious diseases, degenerative disorders and even genetic conditions. Considering heterogeneity in the clinical spectrum of sporadic ataxias, the correct diagnosis remains a clinical challenge. In this review, the different disease categories that lead to sporadic ataxia with adult onset are discussed with special emphasis on their clinical and neuroimaging features, and diagnostic criteria.

  7. Hsa-let-7a functions as a tumor suppressor in renal cell carcinoma cell lines by targeting c-myc

    Energy Technology Data Exchange (ETDEWEB)

    Liu, Yongchao; Yin, Bingde; Zhang, Changcun; Zhou, Libin [Department of Urology, Shanghai First People' s Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai 200080 (China); Fan, Jie, E-mail: jief67@sina.com [Department of Urology, Shanghai First People' s Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai 200080 (China)

    2012-01-06

    Highlights: Black-Right-Pointing-Pointer This study is the first to test the let-7a/c-myc loop in renal cell carcinoma cell lines. Black-Right-Pointing-Pointer Let-7a down-regulated c-myc in three renal cell carcinoma cell lines. Black-Right-Pointing-Pointer c-myc target genes were down-regulated because of the let-7a-mediated down-regulation of c-myc. Black-Right-Pointing-Pointer The let-7a/c-myc loop has a significant function in renal cell carcinoma cell lines. -- Abstract: Widespread functions of the c-myc pathway play a crucial role in renal cell carcinoma (RCC) carcinogenesis. Thus, we evaluated the connection between proto-oncogenic c-myc and anti-neoplastic hsa-let-7a (let-7a) in RCC cell lines. The levels of c-myc and let-7a in 3 RCC cell lines (769P, Caki-1 and 786O) were measured after transfecting the cells with let-7a mimics or a negative control. The change in c-myc protein level was confirmed by Western blot. The anti-neoplastic function of let-7a was evaluated using cell counting kit-8 (CCK-8) for proliferation analysis and cell flow cytometry for cell cycle analysis. The changes of downstream targets of c-myc were measured using reverse transcription quantitative real-time PCR (qRT-PCR). Our results suggest for the first time that let-7a acts as a tumor suppressor in RCC cell lines by down-regulating c-myc and c-myc target genes such as proliferating cell nuclear antigen (PCNA), cyclin D1 (CCND1) and the miR17-92 cluster, which is accompanied by proliferation inhibition and cell cycle arrest.

  8. [Immunohistochemical expression of microvascular density and carbonic anhidrase IX in renal carcinoma. Relation to histological type and tumoral progression].

    Science.gov (United States)

    Rubio-Briones, J; Calatrava, A; Fernández-Serra, A; Ramos Ruiz, R; Iborra, I; García-Casado, Z; Rubio, L; Trassierra, M; Collado, A; Casanova, J; Gómez-Ferrer, A; Solsona, E; López-Guerrero, J A

    2011-02-01

    to correlate the immunohistochemical expression of microvascular density (MVD) and the carbonic anhydrase IX (CAIX) with the different histological subtypes of renal carcinoma and its progression. we studied 93 patients with renal cell carcinoma operated between 1990 and 2008. Antibodies employed for immunohistochemistry (IHC); CD31 (1: 40, Dako) and CD34 (1: 50, Dako) for MVD and CAIX (1: 100, Santa Cruz). CAIX was validated semiquantitatively as: strongly positive (>85%); weakly positive (10% -85%); and negative (quick and easy approximation to MVD. More research should be done to use it as marker for targeted therapy. Copyright © 2010 AEU. Published by Elsevier Espana. All rights reserved.

  9. Are there parameters that predict a nondiagnostic biopsy outcome taken during laparoscopic-assisted cryoablation of small renal tumors?

    NARCIS (Netherlands)

    Barwari, Kurdo; Beemster, Patricia W. T.; Hew, Miki N.; Wijkstra, Hessel; de la Rosette, Jean; Laguna, M. Pilar

    2011-01-01

    The histopathologic diagnosis of a small renal mass (SRM) that is managed with cryoablation relies on preoperative or intraoperative biopsies. Because a considerable number of these SRMs are benign, accurate diagnosis has prognostic and follow-up implications. The main problem in SRMs is the high

  10. Renal myxoma: a case report

    Directory of Open Access Journals (Sweden)

    Carlos Henrique C Souza

    2015-04-01

    Full Text Available Myxomas are rare tumors that can appear in many anatomical locations. There are only 14 cases of renal involvement documented in the literature. This article reports a case of renal myxoma in an elderly woman with recurrent cystitis. After five years of follow-up, the computed tomography (CT revealed a large solid tumor mass in the left kidney. Tumor resection was performed preserving the affected kidney with histopathological diagnosis of renal myxoma. The objective of this study is to report a rare case of renal myxoma, emphasizing the importance of the differential diagnosis from other benign and malignant mesenchymal tumors.

  11. Primary Tumor Characteristics Are Important Prognostic Factors for Sorafenib-Treated Patients with Metastatic Renal Cell Carcinoma: A Retrospective Multicenter Study

    Directory of Open Access Journals (Sweden)

    Sung Han Kim

    2017-01-01

    Full Text Available We aimed to identify prognostic factors associated with progression-free survival (PFS and overall survival (OS in metastatic renal cell carcinoma (mRCC patients treated with sorafenib. We investigated 177 patients, including 116 who received sorafenib as first-line therapy, using the Cox regression model. During a median follow-up period of 19.2 months, the PFS and OS were 6.4 and 32.6 months among all patients and 7.4 months and undetermined for first-line sorafenib-treated patients, respectively. Clinical T3-4 stage (hazard ratio [HR] 2.56 and a primary tumor size >7 cm (HR 0.34 were significant prognostic factors for PFS among all patients, as were tumor size >7 cm (HR 0.12, collecting system invasion (HR 5.67, and tumor necrosis (HR 4.11 for OS (p<0.05. In first-line sorafenib-treated patients, ≥4 metastatic lesions (HR 28.57, clinical T3-4 stage (HR 4.34, collecting system invasion (univariate analysis HR 2.11; multivariate analysis HR 0.07, lymphovascular invasion (HR 13.35, and tumor necrosis (HR 6.69 were significant prognosticators of PFS, as were bone metastasis (HR 5.49 and clinical T3-4 stages (HR 4.1 for OS (p<0.05. Our study thus identified a number of primary tumor-related characteristics as important prognostic factors in sorafenib-treated mRCC patients.

  12. The human relevance of the renal tumor-inducing potential of d-limonene in male rats: implications for risk assessment.

    Science.gov (United States)

    Flamm, W G; Lehman-McKeeman, L D

    1991-02-01

    The monoterpene d-limonene is a naturally occurring chemical which is the major component in oil of orange. Currently, d-limonene is widely used as a flavor and fragrance and is listed to be generally recognized as safe (GRAS) in food by the Food and Drug Administration (21 CFR 182.60 in the Code of Federal Regulations). Recently, however, d-limonene has been shown to cause a male rat-specific kidney toxicity referred to as hyaline droplet nephropathy. Furthermore, chronic exposure to d-limonene causes a significant incidence of renal tubular tumors exclusively in male rats. Although d-limonene is not carcinogenic in female rats or male and female mice given much higher dosages, the male rat-specific nephrocarcinogenicity of d-limonene may raise some concern regarding the safety of d-limonene for human consumption. A considerable body of scientific data has indicated that the renal toxicity of d-limonene results from the accumulation of a protein, alpha 2u-globulin, in male rat kidney proximal tuble lysosomes. This protein is synthesized exclusively by adult male rats. Other species, including humans, synthesize proteins that share significant homology with alpha 2u-globulin. However, none of these proteins, including the mouse equivalent of alpha 2u-globulin, can produce this toxicity, indicating a unique specificity for alpha 2u-globulin. With chronic exposure to d-limonene, the hyaline droplet nephropathy progresses and the kidney shows tubular cell necrosis, granular cast formation at the corticomedullary junction, and compensatory cell proliferation. Both d-limonene and cis-d-limonene-1,2-oxide (the major metabolite involved in this toxicity) are negative in in vitro mutagenicity screens. Therefore, the toxicity-related renal cell proliferation is believed to be integrally involved in the carcinogenicity of d-limonene as persistent elevations in renal cell proliferation may increase fixation of spontaneously altered DNA or serve to promote spontaneously

  13. Serum levels of CA15-3, AFP, CA19-9 and CEA tumor markers in cancer care and treatment of patients with impaired renal function on hemodialysis.

    Science.gov (United States)

    Estakhri, Rasoul; Ghahramanzade, Ali; Vahedi, Amir; Nourazarian, Alireza

    2013-01-01

    Since renal failure causes decrease in tumor marker excretion, use of these markers in cancer care and treatment in patients with renal insufficiency or hemodialysis is controversial. The aim of this study was to investigate differences of serum levels of tumor markers CA15-3, AFP, CA19-9 and CEA in patients with impaired renal function. A total of 100 patients referred to the Tabriz Immam Reza and Amiralmomenin hospital from June 2010 to November 2011 were selected for study. Subjects were divided to 3 groups of healthy, dialysis and renal failure but non hemodialysis cases, the last category being re-grouped based on creatinine clearance. No significant relationship between different groups in serum levels of CEA (P=0.99) and CA19-9 (P=0.29) tumor markers was found. A significant correlation was observed between serum levels of AFP (PCEA (r=0.05, P=0.625). Creatinine clearance significantly correlated with AFP (Ptumor markers in patients with impaired renal function should be performed with special precautions.

  14. Mutations in the RET proto-oncogene in sporadic pheochromocytomas

    Energy Technology Data Exchange (ETDEWEB)

    Thibodeau, S.N.; Lindor, N.M.; Honchel, R. [Mayo Clinic and Foundation, Rochester, MN (United States)] [and others

    1994-09-01

    Mutations in the RET proto-oncogene have recently been demonstrated in kindreds with Multiple Endocrine Neoplasia (MEN) types 2A and 2B. Both of these autosomal dominant disorders are characterized by the development of neoplasia in cell lines of neural crest origin, such as medullary throid carcinomas and pheochromocytomas. Individuals with MEN 2B have, in addition, ganglioneuromas of the lips, tongue and colon, a marfanoid habitus, and corneal nerve thickening. Approximately 90% of patients with MEN 2A have a germline mutation in exons 10 or 11, while 95% of patients with MEN 2B have a T{yields}C transition in codon 918 of exon 16. In this study, pheochromocytomas from 29 individuals who had no clinical evidence of MEN 2A or 2B (sporadic) were examined for the presence of either germline or somatic mutations in exons 10, 11, and 16 of the RET proto-oncogene. Of the 29 tumors examined, 3 (10%) were found to have a mutation in one of the three exons. One tumor had a G{yields}A transition in codon 609 (exon 10), another had a 6 bp deletion encompassing codons 632 & 633 (exon 11), and the final tumor had a T{yields}C transition in codon 918 (exon 16). These mutations were not found in the corresponding normal DNA from these individuals, indicating that the mutation were somatic in origin. Although we cannot exclude the possibility of mutations in other regions of the RET proto-oncogene, our data suggests that: (1) individuals presenting with apparently sporadic pheochromocytomas are not likely to have undiagnosed MEN 2A or 2B; and (2) somatic mutations in the RET proto-oncogene contribute to the process of tumorigenesis in a small percentage of sporadic pheochromocytomas.

  15. Tumor thrombus of inferior vena cava in patients with renal cell carcinoma – clinical and oncological outcome of 50 patients after surgery

    Directory of Open Access Journals (Sweden)

    Kocot Arkadius

    2012-06-01

    Full Text Available Abstract Background To evaluate oncological and clinical outcome in patients with renal cell carcinoma (RCC and tumor thrombus involving inferior vena cava (IVC treated with nephrectomy and thrombectomy. Methods We identified 50 patients with a median age of 65 years, who underwent radical surgical treatment for RCC and tumor thrombus of the IVC between 1997 and 2010. The charts were reviewed for pathological and surgical parameters, as well as complications and oncological outcome. Results The median follow-up was 26 months. In 21 patients (42% distant metastases were already present at the time of surgery. All patients underwent radical nephrectomy, thrombectomy and lymph node dissection through a flank (15 patients/30%, thoracoabdominal (14 patients/28% or midline abdominal approach (21 patients/42%, depending upon surgeon preference and upon the characteristics of tumor and associated thrombus. Extracorporal circulation with cardiopulmonary bypass (CPB was performed in 10 patients (20% with supradiaphragmal thrombus of IVC. Cancer-specific survival for the whole cohort at 5 years was 33.1%. Survival for the patients without distant metastasis at 5 years was 50.7%, whereas survival rate in the metastatic group at 5 years was 7.4%. Median survival of patients with metastatic disease was 16.4 months. On multivariate analysis lymph node invasion, distant metastasis and grading were independent prognostic factors. There was no statistically significant influence of level of the tumor thrombus on survival rate. Indeed, patients with supradiaphragmal tumor thrombus (n = 10 even had a better outcome (overall survival at 5 years of 58.33% than the entire cohort. Conclusions An aggressive surgical approach is the most effective therapeutic option in patients with RCC and any level of tumor thrombus and offers a reasonable longterm survival. Due to good clinical and oncological outcome we prefer the use of CPB with extracorporal

  16. Bap1 and Pbrm1: Determinants of Tumor Grade and mTOR Activation in VHL-Deficient Mouse Models of Renal Cell Carcinoma.

    Science.gov (United States)

    Leung, Janet Y; Kim, William Y

    2017-08-01

    Large genome sequencing efforts have identified frequent mutations in the histone-modifying and chromatin-remodeling genes BAP1 and PBRM1 in clear cell renal cell carcinoma (ccRCC). In this issue of Cancer Discovery , Gu and colleagues model these genetic events in mice and report that dual inactivation of Vhl with either Bap1 or Pbrm1 results in faithful genetically engineered murine models of ccRCC. Moreover, their work establishes that Bap1 and Pbrm1 are determinants of tumor grade and mTORC1 activation and provocatively suggests that the cell of origin of ccRCC may lie in PAX8-expressing Bowman capsule cells. Cancer Discov; 7(8); 802-4. ©2017 AACR See related article by Gu et al., p. 900 . ©2017 American Association for Cancer Research.

  17. An Independent Validation of 2010 Tumor-Node-Metastasis Classification for Renal Cell Carcinoma: A Multi-center Study by the Urooncology Association of Turkey Renal Cancer-Study Group

    Directory of Open Access Journals (Sweden)

    Tayyar Alp Özkan

    2017-06-01

    Full Text Available Objective: The American Joint Committee on Cancer tumor-node-metastasis (TNM classification has been updated by the 7th edition in 2010. The objective of the study was to evaluate cancer-specific survival (CSS in patients with renal cell carcinoma (RCC and assess the concordance of 2002 and novel 2010 TNM primary tumor classifications. Materials and Methods: A retrospective analysis of RCC registries from 25 institutions of the Urooncology Association of Turkey Renal Cancer-Study Group was performed. Patients with RCC had a radical or partial nephrectomy. The database consisted of 1889 patients. Results: Median follow-up time was 25 months (interquartile range: 11.2-47.8. The 5-year CSS rate for pT1a, pT1b, pT2a, pT2b, pT3a and pT4 tumors were 97% [95% confidence interval (CI: 0.93-0.99], 94% (95% CI: 0.91-0.97, 88% (95% CI: 0.81-0.93, 77% (95% CI: 0.64-0.86 74% (95% CI: 0.65-0.81 and 66% (95% CI: 0.51-0.77, respectively according to the 2010 TNM classification (p<0.001. CSS comparisons between pT1a-pT1b (p=0.022, pT1b-pT2a (p=0.030, pT3a-pT3b (p<0.001 and pT3b-pT4 (p=0.020 were statistically significant. Conversely, pT2a-pT2b (p=0.070 and pT2b-pT3a (p=0.314 were not statistically significant. Multivariable analyses revealed the pT stage in the 2010 TNM classification as an independent prognostic factor for CSS (p for trend=0.002. C-indexes for 2002 and 2010 TNM classifications were 0.8683 and 0.8706, respectively. Conclusion: Subdividing pT2 does not have a CSS advantage. Moving adrenal involvement to pT4 yielded a more accurate prognosis prediction. T stage and LNI are independent prognostic factors for CSS in RCC. Overall, the novel 2010 TNM classification is slightly improved over the former one. However, shown by C-index values, this improvement is not sufficient to state that 2010 TNM outperforms the 2002 TNM.

  18. Using {sup 18F} FDG PET/CT to Detect an occult Mesenchymal Tumor Causing Oncogenic Osteomalacia

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Hyo Jung; Choi, Yun Jung; Kim, Hyun Jeong; Jeong, Yong Hyu; Cho, Arthur; Lee, Jae Hoon; Yun, Mijin; Lee, Jong Doo; Kang, Won Jun [Yonsei Univ. College of Medicine, Seoul (Korea, Republic of)

    2011-09-15

    Oncogenic osteomalacia is a rare paraneoplastic syndrome characterized by renal phosphate excretion, hypophosphatemia, and osteomalacia. This syndrome is often caused by tumors of mesenchymal origin. Patients with oncogenic osteomalacia have abnormal bone mineralization, resulting in a high frequency of fractures. Tumor resection is the treatment of choice, as it will often correct the metabolic imbalance. Although oncogenic osteomalacia is a potentially curable disease, diagnosis is difficult and often delayed because of the small size and sporadic location of the tumor. Bone scintigraphy and radiography best characterize osteoma lacia; magnetic resonance imaging findings are nonspecific. Here, we report a case of oncogenic osteomalacia secondary to a phosphaturic mesenchymal tumor that was successfully detected by {sup 18F} fluorodeoxyglucose positron emission tomography/computed tomography ({sup 18F} FDG PET/CT). This case illustrates the advantages of {sup 18F} FDG PET/CT in detecting the occult mesenchymal tumor that causes oncogenic osteomalacia.

  19. The putative tumor suppressor, miR-199a, regulated by Snail, modulates clear cell renal cell carcinoma aggressiveness by repressing ROCK1.

    Science.gov (United States)

    Zhang, Xiao; Li, Peng; Ding, Zhen; Wang, Huili; Wang, Junye; Han, Lei; Ding, Shangwei

    2018-01-01

    Aberrant expression of miR-199a has been frequently reported in cancer studies; however, its role in renal cell carcinoma (RCC) has not been examined in detail. Here, we showed that miR-199a was downregulated in RCC and associated with poor prognostic phenotype. Using luciferase and western blot assays we identified that Rho-associated coiled coil-containing protein kinases 1 (ROCK1) was a direct target gene for miR-199a. miR-199a regulated proliferation, invasion, and apoptosis of clear cell renal cell carcinoma (ccRCC) cells by modulating ROCK1 expression. Interestingly, we also found that miR-199a was modulated by snail in ccRCC cells. Snail elevated ROCK1 expression by repressing miR-199a activity. Altogether, our results identify a crucial tumor suppressive role of miR-199a in the progression of ccRCC and suggest that miR-199a might be an anticancer therapeutic target for ccRCC patients.

  20. Functional Studies on Primary Tubular Epithelial Cells Indicate a Tumor Suppressor Role of SETD2 in Clear Cell Renal Cell Carcinoma

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    Jun Li

    2016-06-01

    Full Text Available SET domain-containing 2 (SETD2 is responsible for the trimethylation of histone H3 lysine36 (H3K36me3 and is one of the genes most frequently mutated in clear cell renal cell carcinoma (ccRCC. It is located at 3p21, one copy of which is lost in the majority of ccRCC tumors, suggesting that SETD2 might function as a tumor suppressor gene. However, the manner in which loss of SETD2 contributes to ccRCC development has not been studied in renal primary tubular epithelial cells (PTECs. Therefore, we studied the consequences of SETD2 knockdown through lentiviral shRNA in human PTECs. Consistent with its known function, SETD2 knockdown (SETD-KD led to loss of H3K36me3 in PTECs. In contrast to SETD2 wild-type PTECs, which have a limited proliferation capacity; the SETD2-KD PTECs continued to proliferate. The expression profiles of SETD2-KD PTECs showed a large overlap with the expression profile of early-passage, proliferating PTECs, whereas nonproliferating PTECs showed a significantly different expression profile. Gene set enrichment analysis revealed a significant enrichment of E2F targets in SETD2-KD and proliferating PTECs as compared with nonproliferating PTECs and in proliferating PTEC compared with SETD2-KD. The SETD2-KD PTECs maintained low expression of CDKN2A and high expression of E2F1, whereas their levels changed with continuing passages in untreated PTECs. In contrast to the nonproliferating PTECs, SETD2-KD PTECs showed no β-galactosidase staining, confirming the protection against senescence. Our results indicate that SETD2 inactivation enables PTECs to bypass the senescence barrier, facilitating a malignant transformation toward ccRCC.

  1. Von Hippel-Lindau tumor suppressor gene loss in renal cell carcinoma promotes oncogenic epidermal growth factor receptor signaling via Akt-1 and MEK-1.

    Science.gov (United States)

    Lee, S Justin; Lattouf, Jean-Baptiste; Xanthopoulos, Julie; Linehan, W Marston; Bottaro, Donald P; Vasselli, James R

    2008-10-01

    Clear-cell renal cell carcinoma (RCC) is the most prevalent form of kidney cancer and is frequently associated with loss of von Hippel-Lindau (VHL) gene function, resulting in the aberrant transcriptional activation of genes that contribute to tumor growth and metastasis, including transforming growth factor-alpha (TGF-alpha), a ligand of the epidermal growth factor receptor (EGFR) tyrosine kinase. To determine the functional impact of EGFR activation on RCC, we suppressed critical components of this pathway: EGFR, Akt-1, and MEK-1. Stable transfection of RCC cells with plasmids bearing shRNA directed against each of these genes was used to individually suppress their expression. Transfectants were characterized for growth and invasiveness in vitro and tumorigenesis in vivo. RCC cell transfectants displayed significantly reduced growth rate and matrix invasion in vitro and RCC tumor xenograft growth rate in vivo. Analysis of tumor cells that emerged after extended periods in each model showed that significant EGFR suppression was sustained, whereas Akt-1 and MEK-1 knock-down cells had escaped shRNA suppression. EGFR, Akt-1, and MEK-1 are individually critical for RCC cell invasiveness in vitro and tumorigenicity in vivo, and even partial suppression of each can have a significant impact on tumor progression. The emergence of transfectants that had escaped Akt-1 and MEK-1 suppression during tumorigenicity experiments suggests that these effectors may each be more critical than EGFR for RCC tumorigenesis, consistent with results from clinical trials of EGFR inhibitors for RCC, where durable clinical responses have not been seen.

  2. Von Hippel-Lindau Tumor Suppressor Gene Loss in Renal Cell Carcinoma Promotes Oncogenic Epidermal Growth Factor Receptor Signaling via Akt-1 and MEK1

    Science.gov (United States)

    Lee, S. Justin; Lattouf, Jean-Baptiste; Xanthopoulos, Julie; Linehan, W. Marston; Bottaro, Donald P.; Vasselli, James R.

    2008-01-01

    Objectives Clear-cell renal cell carcinoma (RCC) is the most prevalent form of kidney cancer and is frequently associated with loss of von Hippel-Lindau (VHL) gene function, resulting in the aberrant transcriptional activation of genes that contribute to tumor growth and metastasis, including transforming growth factor-α (TGF-α), a ligand of the epidermal growth factor receptor (EGFR) tyrosine kinase. To determine the functional impact of EGFR activation on RCC, we suppressed critical components of this pathway: EGFR, Akt-1, and MEK-1. Methods Stable transfection of RCC cells with plasmids bearing shRNA directed against each of these genes was used to individually suppress their expression. Transfectants were characterized for growth and invasiveness in vitro and tumorigenesis in vivo. Results RCC cell transfectants displayed significantly reduced growth rate and matrix invasion in vitro and RCC tumor xenograft growth rate in vivo. Analysis of tumor cells that emerged after extended periods in each model showed that significant EGFR suppression was sustained, whereas Akt-1 and MEK-1 knockdown cells had escaped shRNA suppression. Conclusions EGFR, Akt-1, and MEK-1 are individually critical for RCC cell invasiveness in vitro and tumorigenicity in vivo, and even partial suppression of each can have a significant impact on tumor progression. The emergence of transfectants that had escaped Akt-1 and MEK-1 suppression during tumorigenicity experiments suggests that these effectors may each be more critical than EGFR for RCC tumorigenesis, consistent with results from clinical trials of EGFR inhibitors for RCC, where durable clinical responses have not been seen. PMID:18243508

  3. Comparação dos métodos de imagem no diagnóstico dos tumores renais e calcificações nestas neoplasias Comparison of imaging methods for diagnosis of renal tumors and their calcifications

    Directory of Open Access Journals (Sweden)

    Sergio Marrone Ribeiro

    2004-12-01

    Full Text Available OBJETIVO: Tentar estabelecer uma metodologia no diagnóstico e conduta dos pacientes com massas renais sólidas e complexas, comparando os custos e benefícios dos diferentes métodos de diagnóstico por imagem. Procuramos avançar no diagnóstico diferencial entre lesões benignas e malignas, particularmente através da investigação das calcificações tumorais. MÉTODOS: Realizamos um estudo prospectivo em 31 pacientes portadores de massas renais sólidas ou complexas, todos eles submetidos à ultra-sonografia abdominal (US, ultra-sonografia doppler da massa renal (US Dop, tomografia computadorizada (TC e ressonância magnética (RM. RESULTADOS: Encontramos 28 pacientes com massas malignas e três com massas benignas. Entre os 28 pacientes com lesões malignas, 17 mostraram calcificações pela TC; 16 deles calcificações do tipo central e um calcificação do tipo curvilinear periférica pura (casca de ovo. A urografia excretora (UGE mostrou uma taxa de detecção para calcificações significantemente menor que a US e a TC. Massas benignas e malignas apareceram como descrito na literatura, com o US, TC e RM mostrando alta sensibilidade e especificidade no diagnóstico dos tumores renais. A exceção foi na US Dop, onde nós obtivemos menor sensibilidade para a caracterização de fluxo tumoral maligno. CONCLUSÕES: Foi surpreendente verificar que a TC revelou calcificações centrais em 51,6% dos pacientes desta série, todas elas em lesões malignas, quando a literatura refere uma freqüência de calcificações entre 8% e 22% dos carcinomas de células renais, em estudos utilizando radiografias simples do abdômen e UGE. Este achado é de grande importância quando consideramos que estas calcificações ocorrem particularmente em neoplasias malignas. Como resultado da comparação dos diferentes métodos de diagnóstico por imagem, nós propomos uma metodologia para adequada investigação dos tumores renais.BACKGROUND: To establish the

  4. Renal Cell Carcinoma Metastatic to the Liver: Early Response Assessment after Intraarterial Therapy Using 3D Quantitative Tumor Enhancement Analysis

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    Florian Nima Fleckenstein

    2016-10-01

    CONCLUSION: Three-dimensional (3D quantitative tumor analysis is a reliable predictor of OS when assessing treatment response after IAT in patients with RCC metastatic to the liver. qEASL outperforms conventional non-3D methods and can be used as a surrogate marker for OS early after therapy.

  5. Dynamic tumor modeling of the dose–response relationship for everolimus in metastatic renal cell carcinoma using data from the phase 3 RECORD-1 trial

    International Nuclear Information System (INIS)

    Stein, Andrew; Wang, Wenping; Carter, Alison A; Chiparus, Ovidiu; Hollaender, Norbert; Kim, Hyewon; Motzer, Robert J; Sarr, Celine

    2012-01-01

    The phase 3 RECORD-1 trial (NCT00410124) established the efficacy and safety of everolimus in patients with metastatic renal cell carcinoma (mRCC) who progress on sunitinib or sorafenib. In RECORD-1, patients received 10 mg everolimus daily, with dose reduction to 5 mg daily allowed for toxicity. We have developed a model of tumor growth dynamics utilizing serial measurements of the sum of the longest tumor diameters (SLD) from individual RECORD-1 patients to define the dose–response relationship of everolimus. The model predicts that after 1 year of continuous dosing, the change in SLD of target lesions will be +142.1% ± 98.3%, +22.4% ± 17.2%, and –15.7% ± 11.5% in the average patient treated with placebo, 5 mg everolimus, and 10 mg everolimus, respectively. This nonlinear, mixed-effects modeling approach can be used to describe the dynamics of each individual patient, as well as the overall population. This allows evaluation of how an actual dosing history and individual covariates impact on the observed drug effect, and offers the possibility of predicting clinical observations as a function of time. In this pharmacodynamic model of tumor response, everolimus more effectively shrinks target lesions in mRCC when dosed 10 mg daily versus 5 mg daily, although a 5-mg dose still shows an antitumor effect. These data support earlier studies that established 10 mg daily as the preferred clinical dose of everolimus, and improve our understanding of the everolimus dose–response relationship

  6. Immune responses in patients with metastatic renal cell carcinoma treated with dendritic cells pulsed with tumor lysate

    DEFF Research Database (Denmark)

    Soleimani, A; Berntsen, A; Svane, I M

    2009-01-01

    Patients with metastatic renal cell carcinoma (mRCC) have a limited life expectancy but still a subset of these patients develop immune and clinical responses after immunotherapy including dendritic cell (DC) vaccination. In a recently published phase I/II trials, fourteen HLA-A2 negative patients...... with progressive mRCC were vaccinated with autologous DC pulsed with allogeneic tumour lysate. Low-dose IL-2 administered subcutaneously was given concomitantly. In this study, we analysed lysate specific proliferation of PBMCs from these patients together with the TH1/TH2 balance of the responding T cells. Also......, serum concentrations of IL-10, IL-12, IL-15, IL-17 and IL-18 from these patients and additional thirteen HLA-A2 positive mRCC patients treated with autologous DC pulsed with survivin and telomerase peptides were analysed during vaccination to identify systemic immune responses and potential response...

  7. Stages of Wilms Tumor

    Science.gov (United States)

    ... and interleukin-2 (IL-2) are types of biologic therapy used to treat childhood renal cell cancer . Interferon affects the division of cancer cells and ... Tumors Renal Cell Cancer (RCC) Treatment of renal cell cancer usually ... nodes. Biologic therapy ( interferon and interleukin-2 ) for cancer that ...

  8. Expresión de marcadores de proliferación y apoptosis, necrosis tumoral e infiltrado inflamatorio y su significado pronóstico en el carcinoma de células renales

    OpenAIRE

    Blasco Martínez, Ana

    2010-01-01

    Texto en español y resumen en inglés El carcinoma de células renales (CCR) se define como un conjunto de neoplasias que tienen su origen en los distintos tramos del epitelio de los túbulos renales. Aunque se han asociado al desarrollo de CCR determinados factores ambientales, fundamentalmente el tabaco, los mecanismos que subyacen a la carcinogénesis en estos tumores se relacionan con alteraciones a nivel genético, que según tengan lugar en la línea somática o germinal dan lugar las formas...

  9. Renal cell carcinoma with melanin pigment

    Science.gov (United States)

    Shetty, Jayaprakash; Chandrika; Laxman, Prabhu

    2010-01-01

    The incidence of renal cell carcinoma has been steadily increasing. There are several morphological types of renal cell carcinoma. Recognizing histologic patterns of renal cell carcinoma is important for correct diagnosis and subsequent medical care for the patient. Melanotic tumors in the kidney are very rare. Here, we present an unusual case of renal cell carcinoma with melanin pigment. PMID:20877613

  10. Pretreatment Serum Cystatin C Levels Predict Renal Function, but Not Tumor Characteristics, in Patients with Prostate Neoplasia

    Directory of Open Access Journals (Sweden)

    Feilong Yang

    2017-01-01

    Full Text Available To evaluate the role of Cystatin C (Cys-C in tumorigenesis and progression of prostate cancer (PCa, we retrospectively collected the clinical information from the records of 492 benign prostatic hyperplasia (BPH, 48 prostatic intraepithelial neoplasia (PIN, and 173 PCa patients, whose disease was newly diagnosed and histologically confirmed. Pretreatment serum Cys-C levels were compared across the various groups and then analyzed to identify relationships, if any, with clinical and pathological characteristics of the PCa patient group. There were no significant differences in serum Cys-C levels among the three groups (P > 0.05. In PCa patients with normal SCr levels, patient age was correlated with serum Cys-C level (P ≤ 0.001 but did not correlate with alkaline phosphatase (AKP, lactate dehydrogenase (LDH, prostate specific antigen (PSA, Gleason score, or bone metastasis status (P > 0.05. Age and SCr contributed in part to the variations in serum Cys-C levels of PCa patients (r = 0.356, P ≤ 0.001; r = 0.520, P ≤ 0.001. In conclusion, serum Cys-C levels predict renal function in patients with prostate neoplasia, but were not a biomarker for the development of prostate neoplasia, and were not correlated with the clinicopathological characteristics of PCa.

  11. Sporadic lymphoplasmacytic cholecystitis: a clinicopathologic entity.

    Science.gov (United States)

    Hammer, Suntrea T G; Appelman, Henry D

    2014-08-01

    To describe a sporadic form of lymphoplasmacytic cholecystitis (LPC), a condition known to occur in patients with chronic biliary tract disease. One year's worth of cholecystectomies was reviewed for sporadic cases of LPC. Histologic, radiologic, and clinical findings were reviewed and compared with noninflamed controls. Sporadic cases were also compared histologically with obstructive LPC cases. Sporadic LPC made up 7% of cholecystectomies, had a male predominance (54.2%), and more often presented with clinical signs of acute inflammation compared with controls. Radiologic findings identified gallstones in 71.4% of patients. The second most common finding was unexplained extrahepatic biliary dilation. There were no unique histologic findings to separate sporadic cases from those associated with pancreatobiliary disease. While obstructive LPC is traditionally described as acalculous, chronic cholecystitis, we show this inflammatory pattern occurs both in the presence of gallstones and outside of previously described disease categories. In addition, LPC occurs in a unique patient demographic (older men), often presenting similarly to acute cholecystitis. Copyright© by the American Society for Clinical Pathology.

  12. Phase I study of the mTOR inhibitor ridaforolimus and the HDAC inhibitor vorinostat in advanced renal cell carcinoma and other solid tumors.

    Science.gov (United States)

    Zibelman, Matthew; Wong, Yu-Ning; Devarajan, Karthik; Malizzia, Lois; Corrigan, Alycia; Olszanski, Anthony J; Denlinger, Crystal S; Roethke, Susan K; Tetzlaff, Colleen H; Plimack, Elizabeth R

    2015-10-01

    Drugs inhibiting the mammalian target of rapamycin (mTOR) are approved in the treatment of renal cell carcinoma (RCC), but resistance inevitably emerges. Proposed escape pathways include increased phosphorylation of Akt, which can be down regulated by histone deacetylase (HDAC) inhibitors. We hypothesized that co-treatment with the mTOR inhibitor ridaforolimus and the HDAC inhibitor vorinostat may abrogate resistance in RCC. This phase 1 study evaluated the co-administration of ridaforolimus and vorinostat in patients with advanced solid tumors. The primary objective was to determine the maximum tolerated dose (MTD) in RCC patients. Although all solid tumors were allowed, prior cytotoxic chemotherapy was limited to 1 regimen. Using a modified 3 + 3 dose escalation design, various dose combinations were tested concurrently in separate cohorts. Efficacy was a secondary endpoint. Fifteen patients were treated at one of three dose levels, thirteen with RCC (10 clear cell, 3 papillary). Dosing was limited by thrombocytopenia. The MTD was determined to be ridaforolimus 20 mg daily days 1-5 with vorinostat 100 mg BID days 1-3 weekly, however late onset thrombocytopenia led to a lower recommended phase II dose: ridaforolimus 20 mg daily days 1-5 with vorinostat 100 mg daily days 1-3 weekly. Two patients, both with papillary RCC, maintained disease control for 54 and 80 weeks, respectively. The combination of ridaforolimus and vorinostat was tolerable at the recommended phase II dose. Two patients with papillary RCC experienced prolonged disease stabilization, thus further study of combined HDAC and mTOR inhibition in this population is warranted.

  13. Dendritic cell based tumor vaccination in prostate and renal cell cancer: a systematic review and meta-analysis.

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    Andreas Draube

    Full Text Available BACKGROUND: More than 200 clinical trials have been performed using dendritic cells (DC as cellular adjuvants in cancer. Yet the key question whether there is a link between immune and clinical response remains unanswered. Prostate and renal cell cancer (RCC have been extensively studied for DC-based immunotherapeutic interventions and were therefore chosen to address the above question by means of a systematic review and meta-analysis. METHODOLOGY/PRINCIPAL FINDINGS: Data was obtained after a systematic literature search from clinical trials that enrolled at least 6 patients. Individual patient data meta-analysis was performed by means of conditional logistic regression grouped by study. Twenty nine trials involving a total of 906 patients were identified in prostate cancer (17 and RCC (12. Objective response rates were 7.7% in prostate cancer and 12.7% in RCC. The combined percentages of objective responses and stable diseases (SD amounted to a clinical benefit rate (CBR of 54% in prostate cancer and 48% in RCC. Meta-analysis of individual patient data (n = 403 revealed the cellular immune response to have a significant influence on CBR, both in prostate cancer (OR 10.6, 95% CI 2.5-44.1 and in RCC (OR 8.4, 95% CI 1.3-53.0. Furthermore, DC dose was found to have a significant influence on CBR in both entities. Finally, for the larger cohort of prostate cancer patients, an influence of DC maturity and DC subtype (density enriched versus monocyte derived DC as well as access to draining lymph nodes on clinical outcome could be demonstrated. CONCLUSIONS/SIGNIFICANCE: As a 'proof of principle' a statistically significant effect of DC-mediated cellular immune response and of DC dose on CBR could be demonstrated. Further findings concerning vaccine composition, quality control, and the effect of DC maturation status are relevant for the immunological development of DC-based vaccines.

  14. Thyroid Sporadic Goiter with Adult Heterotopic Bone Formation

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    Adriana Handra-Luca

    2015-01-01

    Full Text Available Thyroid heterotopic bone formation (HBF in goiter is a rare finding. Five thyroid resection specimens were analyzed for HBF. The results were correlated with clinicomorphological features. All patients were women (33–82 years. The preoperative diagnosis was thyroid goiter or nodule. Treatment consisted in thyroidectomy and lobectomy (3 and 2, resp.. Microscopy showed sporadic nodular goiter. Malformative blood vessels and vascular calcifications were seen in intra- and extrathyroid location (5 and 3, resp.. The number and size of HBFs (total: 28 ranged between 1 and 23/thyroid gland (one bilateral and 1 and 10 mm, respectively. Twelve HBFs were in contact with the thyroid capsule. Most were extranodular (21, versus 6 intranodular. The medical history was positive for dyslipidemia, hyperglycemia, renal dysfunction, and hyperuricemia (2, 3, and 3 cases and 1 case, resp. without any parathyroid abnormality. In conclusion, thyroid HBF may be characterized by subcapsular or extranodular location, various size (usually ≥2 mm, and vascular calcifications and malformations. Features of metabolic syndrome and renal dysfunction may be present, but their exact role in the pathogenesis of HBFs remains to be elucidated.

  15. BRCA 1/2-Mutation Related and Sporadic Breast and Ovarian Cancers: More Alike than Different

    Science.gov (United States)

    Burgess, Melissa; Puhalla, Shannon

    2014-01-01

    No longer is histology solely predictive of cancer treatment and outcome. There is an increasing influence of tumor genomic characteristics on therapeutic options. Both breast and ovarian cancers are at higher risk of development in patients with BRCA 1/2-germline mutations. Recent data from The Cancer Genome Atlas and others have shown a number of genomic similarities between triple negative breast cancers (TNBCs) and ovarian cancers. Recently, poly (ADP-ribose) polymerase (PARP) inhibitors have shown promising activity in hereditary BRCA 1/2-mutated and sporadic breast and ovarian cancers. In this review, we will summarize the current literature regarding the genomic and phenotypic similarities between BRCA 1/2-mutation related cancers, sporadic TNBCs, and sporadic ovarian cancers. We will also review Phase I, II, and III data using PARP inhibitors for these malignancies and compare and contrast the results with respect to histology. PMID:24579064

  16. Renal primitive neuroectodermal tumor as a second malignancy after chemotherapy and radiation for Non-Hodgkin's Lymphoma--treatment-related or just poor old bad luck?: A case report.

    Science.gov (United States)

    de Menezes, Jean-Louis; Patil, Hitendra M; Kannan, R; Pradhan, Sultan A

    2015-01-01

    Peripheral primitive neuroectodermal tumor (PNET) is a rare histology to be found in primary tumors of the kidney. There are less than a hundred cases reported in the English literature. Most of these have been diagnosed after surgery for a renal neoplasm diagnosed on imaging. PNET has rarely been reported as a second malignancy, and has never been reported as a second malignancy after non-Hodgkin's lymphoma (NHL). Herein, we present our case of a 38-year-old female who developed a second malignancy in the kidney after the treatment for NHL.

  17. Synergies of Targeting Tumor Angiogenesis and Immune Checkpoints in Non-Small Cell Lung Cancer and Renal Cell Cancer: From Basic Concepts to Clinical Reality.

    Science.gov (United States)

    Pircher, Andreas; Wolf, Dominik; Heidenreich, Axel; Hilbe, Wolfgang; Pichler, Renate; Heidegger, Isabel

    2017-10-31

    In recent years, considerable advances concerning therapeutic strategies in patients with metastatic cancer have been achieved. Particularly in renal cell cancer (RCC) and advanced stage non-small cell lung cancer (NSCLC), immune-activating and antiangiogenic (AA) drugs (i.e., checkpoint antibodies and vascular endothelial growth factor (VEGF)/VEGF receptors (VEGFR) targeting compounds, respectively) have been successfully developed. As immune-effector cells have to enter the tumor, it is tempting to speculate that the combination of immunotherapy with AA treatment may induce synergistic effects. In this short review, we explore the theoretical background and the therapeutic potential of this novel treatment option for patients with advanced RCC or NSCLC. We discuss the growing body of evidence that pro-angiogenic factors negatively modulate the T-cell-mediated immune response and examine the preclinical evidence for testing combined immune-activating and AA therapy concepts in clinical practice. Particular attention will also be paid to potential novel treatment-related adverse events induced by combination treatment.

  18. Breast cancer metastatic to the kidney with renal vein involvement.

    Science.gov (United States)

    Nasu, Hatsuko; Miura, Katsutoshi; Baba, Megumi; Nagata, Masao; Yoshida, Masayuki; Ogura, Hiroyuki; Takehara, Yasuo; Sakahara, Harumi

    2015-02-01

    The common sites of breast cancer metastases include bones, lung, brain, and liver. Renal metastasis from the breast is rare. We report a case of breast cancer metastatic to the kidney with extension into the renal vein. A 40-year-old woman had undergone left mastectomy for breast cancer at the age of 38. A gastric tumor, which was later proved to be metastasis from breast cancer, was detected by endoscopy. Computed tomography performed for further examination of the gastric tumor revealed a large left renal tumor with extension into the left renal vein. It mimicked a primary renal tumor. Percutaneous biopsy of the renal tumor confirmed metastasis from breast cancer. Surgical intervention of the stomach and the kidney was avoided, and she was treated with systemic chemotherapy. Breast cancer metastatic to the kidney may present a solitary renal mass with extension into the renal vein, which mimics a primary renal tumor.

  19. Renal hemangioma

    Directory of Open Access Journals (Sweden)

    Theodorico F. da Costa Neto

    2004-06-01

    Full Text Available INTRODUCTION: Renal hemangioma is a relatively rare benign tumor, seldom diagnosed as a cause of hematuria. CASE REPORT: A female 40-year old patient presented with continuous gross hematuria, anemia and episodic right lumbar pain, with onset about 3 months previously. The patient underwent multiple blood transfusions during her hospital stay and extensive imaging propedeutics was performed. Semi-rigid ureterorenoscopy evidenced a bleeding focus in the upper calix of the right kidney, with endoscopic treatment being unfeasible. The patient underwent right upper pole nephrectomy and presented a favorable outcome. Histopathological analysis of the surgical specimen showed that it was a renal hemangioma. COMMENTS: Imaging methods usually employed for diagnostic investigation of hematuria do not have good sensitivity for renal hemangioma. However, they are important to exclude the most frequent differential diagnoses. The ureterorenoscopy is the diagnostic method of choice and endoscopic treatment can be feasible when the lesion is accessible and electrocautery or laser are available. We emphasize the open surgical treatment as a therapeutic option upon failure of less invasive methods.

  20. Nephrogenic tumors

    International Nuclear Information System (INIS)

    Wiesbauer, P.

    2008-01-01

    Nephroblastomas are the most common malignant renal tumors in childhood. According to the guidelines of the SIOP (Societe Internationale d'Oncologie Pediatrique) and GPOH (Gesellschaft fuer Paediatrische Onkologie und Haematologie) pre-operative chemotherapy can be started without histological confirmation and thus initial imaging studies, in particular ultrasound, play an outstanding role for diagnostic purposes

  1. Primary renal synovial sarcoma

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    Girish D. Bakhshi

    2012-03-01

    Full Text Available Primary Renal Sarcoma is rare tumor comprising only 1% of all renal tumours. Synovial sarcomas are generally deep-seated tumors arising in the proximity of large joints of adolescents and young adults and account for 5-10% of all soft tissue tumours. Primary synovial sarcoma of kidney is rare and has poor prognosis. It can only be diagnosed by immunohistochemistry. It should be considered as a differential in sarcomatoid and spindle cell tumours. We present a case of 33-year-old female, who underwent left sided radical nephrectomy for renal tumour. Histopathology and genetic analysis diagnosed it to be primary renal synovial sarcoma. Patient underwent radiation therapy and 2 years follow up is uneventful. A brief case report with review of literature is presented.

  2. Genetic instability in inherited and sporadic leukemias.

    Science.gov (United States)

    Popp, Henning D; Bohlander, Stefan K

    2010-12-01

    Genetic instability due to increased DNA damage and altered DNA repair is of central significance in the initiation and progression of inherited and sporadic human leukemias. Although very rare, some inherited DNA repair insufficiency syndromes (e.g., Fanconi anemia, Bloom's syndrome) have added substantially to our understanding of crucial mechanisms of leukemogenesis in recent years. Conversely, sporadic leukemias account for the main proportion of leukemias and here DNA damaging reactive oxygen species (ROS) play a central role. Although the exact mechanisms of increased ROS production remain largely unknown and no single pathway has been detected thus far, some oncogenic proteins (e.g., the activated tyrosine kinases BCR-ABL1 and FLT3-ITD) seem to play a key role in driving genetic instability by increased ROS generation which influences the disease course (e.g., blast crisis in chronic myeloid leukemia or relapse in FLT3-ITD positive acute myeloid leukemia). Of course other mechanisms, which promote genetic instability in leukemia also exist. A newly emerging mechanism is the genome-wide alteration of epigenetic marks (e.g., hypomethylation of histone H3K79), which promotes chromosomal instability. Taken together genetic instability plays a critical role both in inherited and sporadic leukemias and emerges as a common theme in both inherited and sporadic leukemias. Beyond its theoretical impact, the analysis of genetic instability may lead the way to the development of innovative therapy strategies. © 2010 Wiley-Liss, Inc.

  3. Sporadic minute medullary thyroid carcinoma with a double RET mutation: A case report.

    Science.gov (United States)

    Yamamoto, Hiroyuki; Ishii, Jun; Chiba, Tomohiro; Nakazato, Yoko; Hirano, Kouichi; Kamma, Hiroshi

    2017-11-01

    We describe a 74-year-old man with a nodular goiter accompanied by an incidental sporadic minute medullary thyroid carcinoma (MTC). Histopathologically, the MTC was a well-defined 1.7 mm tumor in the upper one-third right lobe, with solid cell nests (SCNs) adjacent to the MTC. C-cells were scattered mainly around the SCNs, but C-cell hyperplasia was not evident in the background thyroid. The MTC cell phenotype was immunohistochemically identical to background C-cells, but was completely different from the SCN main cells. Direct DNA analyses of isolated MTC paraffin-embedded specimens revealed two RET proto-oncogene missense point mutations in exon 11 (i.e., C630R and C634W). The non-tumor thyroid tissue did not reveal any mutations. This study reports the smallest case of sporadic MTC with a double RET somatic mutation, substantiating that RET mutations can occur during a very early stage of carcinogenesis. The combined presence of C630R and C634W represent a novel somatic mutation in sporadic MTC. The present case indicates that the sporadic MTC originated from the surrounding C-cells of the SCNs without C-cell hyperplasia and that the SCN main cells may not be able to develop into an MTC. © 2017 Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.

  4. Down-regulation of BRCA1 in chronic pancreatitis and sporadic pancreatic adenocarcinoma.

    Science.gov (United States)

    Beger, Carmela; Ramadani, Marco; Meyer, Stephan; Leder, Gerd; Krüger, Martin; Welte, Karl; Gansauge, Frank; Beger, Hans G

    2004-06-01

    BRCA1 and BRCA2 are considered to be breast cancer susceptibility genes that may also contribute to pancreatic cancer development because family studies revealed mutation carriers to have an increased risk of developing pancreatic cancer. However, as demonstrated for breast and ovarian cancer, inactivation of BRCA in sporadic diseases is based on alteration in gene expression or functional alteration. To study a potential correlation of BRCA1 and BRCA2 to chronic pancreatitis and development of sporadic pancreatic adenocarcinoma, we have analyzed the expression of these genes by quantitative PCR and performed immunohistochemical analyses in normal pancreatic tissues, chronic pancreatitis, and pancreatic cancer specimens. BRCA1 expression was down-regulated in chronic alcoholic pancreatitis, in particular on the RNA level. Furthermore, our data indicate suppressed BRCA1 expression in pancreatic cancer on both the RNA and protein levels. Quantitative analysis of BRCA1 protein expression demonstrated regular staining in 50% of tumor specimens tested and reduced staining in 50% of tumor specimens tested. Correlation with the clinical outcome revealed a significantly better 1-year overall survival for patients with BRCA1-regular as compared with BRCA1-reduced or BRCA1-absent tumors. In contrast, no substantial differences in BRCA2 expression were found in chronic pancreatitis and pancreatic cancer samples. Our data demonstrate alteration of BRCA1 expression in chronic pancreatitis and sporadic pancreatic adenocarcinoma. We, for the first time, provide evidence for a role of BRCA1 in pancreatic carcinogenesis of noninherited tumors and for clinical outcome.

  5. Sporadic early-onset colorectal cancer is a specific sub-type of cancer: a morphological, molecular and genetics study.

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    Sylvain Kirzin

    Full Text Available Sporadic early onset colorectal carcinoma (EOCRC which has by definition no identified hereditary predisposition is a growing problem that remains poorly understood. Molecular analysis could improve identification of distinct sub-types of colorectal cancers (CRC with therapeutic implications and thus can help establish that sporadic EOCRC is a distinct entity. From 954 patients resected for CRC at our institution, 98 patients were selected. Patients aged 45-60 years were excluded to help define "young" and "old" groups. Thirty-nine cases of sporadic EOCRC (patients ≤ 45 years with microsatellite stable tumors were compared to both microsatellite stable tumors from older patients (36 cases, patients>60 years and to groups of patients with microsatellite instability. Each group was tested for TP53, KRAS, BRAF, PIK3CA mutations and the presence of a methylator phenotype. Gene expression profiles were also used for pathway analysis. Compared to microsatellite stable CRC from old patients, sporadic EOCRC were characterized by distal location, frequent synchronous metastases and infrequent synchronous adenomas but did not have specific morphological characteristics. A familial history of CRC was more common in sporadic EOCRC patients despite a lack of identified hereditary conditions (p = 0.013. Genetic studies also showed the absence of BRAF mutations (p = 0.022 and the methylator phenotype (p = 0.005 in sporadic EOCRC compared to older patients. Gene expression analysis implicated key pathways such as Wnt/beta catenin, MAP Kinase, growth factor signaling (EGFR, HGF, PDGF and the TNFR1 pathway in sporadic EOCRC. Wnt/beta catenin signaling activation was confirmed by aberrant nuclear beta catenin immunostaining (p = 0.01. This study strongly suggests that sporadic EOCRC is a distinct clinico-molecular entity presenting as a distal and aggressive disease associated with chromosome instability. Furthermore, several signaling pathways including the

  6. RENAL DAMAGE WITH MALIGNANT NEOPLASMS

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    I. B. Kolina

    2015-01-01

    Full Text Available The relationship between renal damage and malignant neoplasms is one of the most actual problems of the medicine of internal diseases. Very often, exactly availability of renal damage determines the forecast of cancer patients. The range of renal pathologies associated with tumors is unusually wide: from the mechanical effect of the tumor or metastases on the kidneys and/or the urinary tract and paraneoplastic manifestations in the form of nephritis or amyloidosis to nephropathies induced with drugs or tumor lysis, etc. Thrombotic complications that develop as a result of exposure to tumor effects, side effects of certain drugs or irradiation also play an important role in the development of the kidney damage. The most frequent variants of renal damage observed in the practice of medical internists (therapists, urologists, surgeons, etc., as well as methods of diagnosis and treatment approaches are described in the article. Timely and successful prevention and treatment of tumor-associated nephropathies give hope for retaining renal functions, therefore, a higher life standard after completion of anti-tumor therapy. Even a shortterm episode of acute renal damage suffered by a cancer patient must be accompanied with relevant examination and treatment. In the caseof transformation of acute renal damage into the chronic kidney disease, such patients need systematic and weighted renoprotective therapy and correct dosing of nephrotoxic drugs.

  7. Gene expression profiles of sporadic canine hemangiosarcoma are uniquely associated with breed.

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    Beth A Tamburini

    2009-05-01

    Full Text Available The role an individual's genetic background plays on phenotype and biological behavior of sporadic tumors remains incompletely understood. We showed previously that lymphomas from Golden Retrievers harbor defined, recurrent chromosomal aberrations that occur less frequently in lymphomas from other dog breeds, suggesting spontaneous canine tumors provide suitable models to define how heritable traits influence cancer genotypes. Here, we report a complementary approach using gene expression profiling in a naturally occurring endothelial sarcoma of dogs (hemangiosarcoma. Naturally occurring hemangiosarcomas of Golden Retrievers clustered separately from those of non-Golden Retrievers, with contributions from transcription factors, survival factors, and from pro-inflammatory and angiogenic genes, and which were exclusively present in hemangiosarcoma and not in other tumors or normal cells (i.e., they were not due simply to variation in these genes among breeds. Vascular Endothelial Growth Factor Receptor 1 (VEGFR1 was among genes preferentially enriched within known pathways derived from gene set enrichment analysis when characterizing tumors from Golden Retrievers versus other breeds. Heightened VEGFR1 expression in these tumors also was apparent at the protein level and targeted inhibition of VEGFR1 increased proliferation of hemangiosarcoma cells derived from tumors of Golden Retrievers, but not from other breeds. Our results suggest heritable factors mold gene expression phenotypes, and consequently biological behavior in sporadic, naturally occurring tumors.

  8. Gene Expression Profiles of Sporadic Canine Hemangiosarcoma Are Uniquely Associated with Breed

    Science.gov (United States)

    Tamburini, Beth A.; Trapp, Susan; Phang, Tzu Lip; Schappa, Jill T.; Hunter, Lawrence E.; Modiano, Jaime F.

    2009-01-01

    The role an individual's genetic background plays on phenotype and biological behavior of sporadic tumors remains incompletely understood. We showed previously that lymphomas from Golden Retrievers harbor defined, recurrent chromosomal aberrations that occur less frequently in lymphomas from other dog breeds, suggesting spontaneous canine tumors provide suitable models to define how heritable traits influence cancer genotypes. Here, we report a complementary approach using gene expression profiling in a naturally occurring endothelial sarcoma of dogs (hemangiosarcoma). Naturally occurring hemangiosarcomas of Golden Retrievers clustered separately from those of non-Golden Retrievers, with contributions from transcription factors, survival factors, and from pro-inflammatory and angiogenic genes, and which were exclusively present in hemangiosarcoma and not in other tumors or normal cells (i.e., they were not due simply to variation in these genes among breeds). Vascular Endothelial Growth Factor Receptor 1 (VEGFR1) was among genes preferentially enriched within known pathways derived from gene set enrichment analysis when characterizing tumors from Golden Retrievers versus other breeds. Heightened VEGFR1 expression in these tumors also was apparent at the protein level and targeted inhibition of VEGFR1 increased proliferation of hemangiosarcoma cells derived from tumors of Golden Retrievers, but not from other breeds. Our results suggest heritable factors mold gene expression phenotypes, and consequently biological behavior in sporadic, naturally occurring tumors. PMID:19461996

  9. Inactivation of the FLCN tumor suppressor gene induces TFE3 transcriptional activity by increasing its nuclear localization.

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    Seung-Beom Hong

    2010-12-01

    Full Text Available Germline mutations in a tumor suppressor gene FLCN lead to development of fibrofolliculomas, lung cysts and renal cell carcinoma (RCC in Birt-Hogg-Dubé syndrome. TFE3 is a member of the MiTF/TFE transcription factor family and Xp11.2 translocations found in sporadic RCC involving TFE3 result in gene fusions and overexpression of chimeric fusion proteins that retain the C-terminal DNA binding domain of TFE3. We found that GPNMB expression, which is regulated by MiTF, was greatly elevated in renal cancer cells harboring either TFE3 translocations or FLCN inactivation. Since TFE3 is implicated in RCC, we hypothesized that elevated GPNMB expression was due to increased TFE3 activity resulting from the inactivation of FLCN.TFE3 knockdown reduced GPNMB expression in renal cancer cells harboring either TFE3 translocations or FLCN inactivation. Moreover, FLCN knockdown induced GPNMB expression in FLCN-restored renal cancer cells. Conversely, wildtype FLCN suppressed GPNMB expression in FLCN-null cells. FLCN inactivation was correlated with increased TFE3 transcriptional activity accompanied by its nuclear localization as revealed by elevated GPNMB mRNA and protein expression, and predominantly nuclear immunostaining of TFE3 in renal cancer cells, mouse embryo fibroblast cells, mouse kidneys and mouse and human renal tumors. Nuclear localization of TFE3 was associated with TFE3 post-translational modifications including decreased phosphorylation.Increased TFE3 activity is a downstream event induced by FLCN inactivation and is likely to be important for renal tumor development. This study provides an important novel mechanism for induction of TFE3 activity in addition to TFE3 overexpression resulting from Xp11.2 translocations, suggesting that TFE3 may be more broadly involved in tumorigenesis.

  10. Predictors of Preoperative Tinnitus in Unilateral Sporadic Vestibular Schwannoma

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    Georgios Naros

    2017-08-01

    Full Text Available ObjectiveNearly two-thirds of patients with vestibular schwannoma (VS are reporting a significantly impaired quality of life due to tinnitus. VS-associated tinnitus is attributed to an anatomical and physiological damage of the hearing nerve by displacing growth of the tumor. In contrast, the current pathophysiological concept of non-VS tinnitus hypothesizes a maladaptive neuroplasticity of the central nervous system to a (hidden hearing impairment resulting in a subjective misperception. However, it is unclear whether this concept fits to VS-associated tinnitus. This study aims to determine the clinical predictors of VS-associated tinnitus to ascertain the compatibility of both pathophysiological concepts.MethodsThis retrospective study includes a group of 478 neurosurgical patients with unilateral sporadic VS evaluated preoperatively regarding the occurrence of ipsilateral tinnitus depending on different clinical factors, i.e., age, gender, tumor side, tumor size (T1–T4 according to the Hannover classification, and hearing impairment (Gardner–Robertson classification, GR1–5, using a binary logistic regression.Results61.8% of patients complain about a preoperative tinnitus. The binary logistic regression analysis identified male gender [OR 1.90 (1.25–2.75; p = 0.002] and hearing impairment GR3 [OR 1.90 (1.08–3.35; p = 0.026] and GR4 [OR 8.21 (2.29–29.50; p = 0.001] as positive predictors. In contrast, patients with large T4 tumors [OR 0.33 (0.13–0.86; p = 0.024] and complete hearing loss GR5 [OR 0.36 (0.15–0.84; p = 0.017] were less likely to develop a tinnitus. Yet, 60% of the patients with good clinical hearing (GR1 and 25% of patients with complete hearing loss (GR5 suffered from tinnitus.ConclusionThese data are good accordance with literature about non-VS tinnitus indicating hearing impairment as main risk factor. In contrast, complete hearing loss appears a negative predictor for tinnitus. For the first

  11. Tumor Necrosis Factor-Like Weak Inducer of Apoptosis Accelerates the Progression of Renal Fibrosis in Lupus Nephritis by Activating SMAD and p38 MAPK in TGF-β1 Signaling Pathway

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    Zhiqin Liu

    2016-01-01

    Full Text Available This study aim was to explore the effects of tumor necrosis factor-like weak inducer of apoptosis (TWEAK in lupus nephritis and its potential underlying mechanisms. MRL/lpr mice were used for in vivo experiments and human proximal tubular cells (HK2 cells were used for in vitro experiments. Results showed that MRL/lpr mice treated with vehicle solution or LV-Control shRNA displayed significant proteinuria and severe renal histopathological changes. LV-TWEAK-shRNA treatment reversed these changes and decreased renal expressions of TWEAK, TGF-β1, p-p38 MAPK, p-Smad2, COL-1, and α-SMA proteins. In vitro, hTWEAK treatment upregulated the expressions of TGF-β1, p-p38 MAPK, p-SMAD2, α-SMA, and COL-1 proteins in HK2 cells and downregulated the expressions of E-cadherin protein, which were reversed by cotreatment with anti-TWEAK mAb or SB431542 treatment. These findings suggest that TWEAK may contribute to chronic renal changes and renal fibrosis by activating TGF-β1 signaling pathway, and phosphorylation of Smad2 and p38 MAPK proteins was also involved in this signaling pathway.

  12. Multiple oncocytomas and renal carcinoma

    International Nuclear Information System (INIS)

    Velasquez, G.; Glass, T.A.; D'Souza, V.J.; Formanek, A.G.

    1984-01-01

    Renal oncocytoma, although rare, is being diagnosed more frequently, and criteria to differentiate it from other tumors have been described. Multiple oncocytomas have been reported, but an association between multiple oncocytomas and renal carcinoma in the same kidney has not been described. The authors report a case with two oncocytomas and a renal carcinoma in the right kidney as well as a right adrenal adenoma

  13. The driver landscape of sporadic chordoma.

    Science.gov (United States)

    Tarpey, Patrick S; Behjati, Sam; Young, Matthew D; Martincorena, Inigo; Alexandrov, Ludmil B; Farndon, Sarah J; Guzzo, Charlotte; Hardy, Claire; Latimer, Calli; Butler, Adam P; Teague, Jon W; Shlien, Adam; Futreal, P Andrew; Shah, Sohrab; Bashashati, Ali; Jamshidi, Farzad; Nielsen, Torsten O; Huntsman, David; Baumhoer, Daniel; Brandner, Sebastian; Wunder, Jay; Dickson, Brendan; Cogswell, Patricia; Sommer, Josh; Phillips, Joanna J; Amary, M Fernanda; Tirabosco, Roberto; Pillay, Nischalan; Yip, Stephen; Stratton, Michael R; Flanagan, Adrienne M; Campbell, Peter J

    2017-10-12

    Chordoma is a malignant, often incurable bone tumour showing notochordal differentiation. Here, we defined the somatic driver landscape of 104 cases of sporadic chordoma. We reveal somatic duplications of the notochordal transcription factor brachyury (T) in up to 27% of cases. These variants recapitulate the rearrangement architecture of the pathogenic germline duplications of T that underlie familial chordoma. In addition, we find potentially clinically actionable PI3K signalling mutations in 16% of cases. Intriguingly, one of the most frequently altered genes, mutated exclusively by inactivating mutation, was LYST (10%), which may represent a novel cancer gene in chordoma.Chordoma is a rare often incurable malignant bone tumour. Here, the authors investigate driver mutations of sporadic chordoma in 104 cases, revealing duplications in notochordal transcription factor brachyury (T), PI3K signalling mutations, and mutations in LYST, a potential novel cancer gene in chordoma.

  14. Leiomyosarcoma of the renal vein

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    Lemos Gustavo C.

    2003-01-01

    Full Text Available Leiomyosarcoma of the renal vein is a rare tumor of complex diagnosis. We presented a case of renal vein leiomyosarcoma detected in a routine study. The primary treatment was complete surgical removal of the mass. In cases where surgical removal is not possible the prognosis is poor, with high rates of local recurrence and distant spread.

  15. A divide-and-conquer strategy in tumor sampling enhances detection of intratumor heterogeneity in routine pathology: A modeling approach in clear cell renal cell carcinoma [version 2; referees: 4 approved

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    José I. Lopez

    2016-04-01

    Full Text Available Intratumor heterogeneity (ITH is an inherent process in cancer development which follows for most of the cases a branched pattern of evolution, with different cell clones evolving independently in space and time across different areas of the same tumor. The determination of ITH (in both spatial and temporal domains is nowadays critical to enhance patient treatment and prognosis. Clear cell renal cell carcinoma (CCRCC provides a good example of ITH. Sometimes the tumor is too big to be totally analyzed for ITH detection and pathologists decide which parts must be sampled for the analysis. For such a purpose, pathologists follow internationally accepted protocols. In light of the latest findings, however, current sampling protocols seem to be insufficient for detecting ITH with significant reliability. The arrival of new targeted therapies, some of them providing promising alternatives to improve patient survival, pushes the pathologist to obtain a truly representative sampling of tumor diversity in routine practice. How large this sampling must be and how this must be performed are unanswered questions so far.  Here we present a very simple method for tumor sampling that enhances ITH detection without increasing costs. This method follows a divide-and-conquer (DAC strategy, that is, rather than sampling a small number of large-size tumor-pieces as the routine protocol (RP advises, we suggest sampling many small-size pieces along the tumor. We performed a computational modeling approach to show that the usefulness of the DAC strategy is twofold: first, we show that DAC outperforms RP with similar laboratory costs, and second, DAC is capable of performing similar to total tumor sampling (TTS but, very remarkably, at a much lower cost. We thus provide new light to push forward a shift in the paradigm about how pathologists should sample tumors for achieving efficient ITH detection.

  16. Comparison Between Sporadic and Misdiagnosed Sporadic Creutzfeldt-Jakob Disease: A Report of Two Cases.

    Science.gov (United States)

    Zhao, Xiongfei; Yu, Yingxin; Zhao, Zhiru; Xu, Jiaping

    2015-06-01

    Definite accurate diagnosis for Creutzfeldt-Jakob disease (CJD) depends on neuropathologic examination of brain biopsy or autopsy. However, transmissible nature makes the invasive examination dangerous. This study was set to determine that the clinical features are for the diagnosis of CJD through a comparison study. We compared clinical features of two cases with initial diagnosis of sporadic CJD. One case was finally diagnosed as definite sporadic CJD. According to World Health Organization diagnosis criteria, the other one, which had been diagnosed as probable sporadic CJD, was confirmed as limbic encephalitis after long-term follow-up. Compared with the case of definite sporadic CJD, the misdiagnosed case did not present typical electroencephalogram (EEG) and diffusion-weighted in magnetic resonance images (DWI) of CJD. However, cerebrospinal fluid in the misdiagnosed patient showed 14-3-3 protein positivity. The patient conditions improved after treatment. Through this case comparison, we conclude that EEG and DWI are necessary for accurate diagnosis of sporadic CJD. Further, long-term follow-up is crucial to diagnosis and treatment of CJD.

  17. Treatment Options for Wilms Tumor

    Science.gov (United States)

    ... and interleukin-2 (IL-2) are types of biologic therapy used to treat childhood renal cell cancer . Interferon affects the division of cancer cells and ... Tumors Renal Cell Cancer (RCC) Treatment of renal cell cancer usually ... nodes. Biologic therapy ( interferon and interleukin-2 ) for cancer that ...

  18. BRCAness profile of sporadic ovarian cancer predicts disease recurrence.

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    Weiya Z Wysham

    Full Text Available The consequences of defective homologous recombination (HR are not understood in sporadic ovarian cancer, nor have the potential role of HR proteins other than BRCA1 and BRCA2 been clearly defined. However, it is clear that defects in HR and other DNA repair pathways are important to the effectiveness of current therapies. We hypothesize that a subset of sporadic ovarian carcinomas may harbor anomalies in HR pathways, and that a BRCAness profile (defects in HR or other DNA repair pathways could influence response rate and survival after treatment with platinum drugs. Clinical availability of a BRCAness profile in patients and/or tumors should improve treatment outcomes.To define the BRCAness profile of sporadic ovarian carcinoma and determine whether BRCA1, PARP, FANCD2, PTEN, H2AX, ATM, and P53 protein expression correlates with response to treatment, disease recurrence, and recurrence-free survival.Protein microarray analysis of ovarian cancer tissue was used to determine protein expression levels for defined DNA repair proteins. Correlation with clinical and pathologic parameters in 186 patients with advanced stage III-IV and grade 3 ovarian cancer was analyzed using Chi square, Kaplan-Meier method, Cox proportional hazard model, and cumulative incidence function.High PARP, FANCD2 and BRCA1 expressions were significantly correlated with each other; however, elevated p53 expression was associated only with high PARP and FANCD2. Of all patients, 9% recurred within the first year. Among early recurring patients, 41% had high levels of PARP, FANCD2 and P53, compared to 19.5% of patients without early recurrence (p = 0.04. Women with high levels of PARP, FANCD2 and/or P53 had first year cumulative cancer incidence of 17% compared with 7% for the other groups (P = 0.03.Patients with concomitantly high levels of PARP, FANCD2 and P53 protein expression are at increased risk of early ovarian cancer recurrence and platinum resistance.

  19. Radiosurgical treatment of sporadic vestibular schwannomas: A prospective cohort study

    International Nuclear Information System (INIS)

    Martel V, Freddy; Iniguez S, Rodrigo; Venencia M, Daniel; Tagle M, Patricio; Besa D, Pelayo; Lorenzoni S, Jose

    2008-01-01

    Objective: To analyze the preliminary experience of radiosurgery for Vestibular Schwannomas at the Pontificia Universidad Catolica de Chile. Material and methods: The first 17 patients with sporadic Vestibular Schwannomas treated by radiosurgery at our institution are reported. The marginal dose used was 12 to 12.5 Gy. prescribed at the 70 or 80 isodose fine. Patients were controlled at 6, 12 and 24 months with magnetic resonance, audiometric study and clinical examination. Results: In all of the 17 patients treated a decrease tumor enhancement on MR was demonstrated. In 16 patients (94%) a pattern of central tumor necrosis was observed during the firs year Actuarial useful hearing was maintained in 62.5% at 2 year after treatment. Facial nerve function was maintained in all of the 15 patients with normal function at treatment (100%). Trigeminal function was maintained in ah of the 14 patients (100%) with previous normal trigeminal function. The mean time to return to work or normal activities was 11.5 days after treatment. Conclusions: These preliminary results are comparable with results published in the literature and reinforce the demonstrate role of radiosurgery in the management of vestibular schwannomas

  20. [{sup 18}F]Fluciclatide in the in vivo evaluation of human melanoma and renal tumors expressing α{sub v}β{sub 3} and α{sub v}β{sub 5} integrins

    Energy Technology Data Exchange (ETDEWEB)

    Mena, Esther; Turkbey, Baris; Choyke, Peter; Kurdziel, Karen [NCI, NIH, Molecular Imaging Program, Bethesda, MD (United States); Owenius, Rikard [GE Healthcare, Life Sciences, Uppsala (Sweden); Sherry, Richard [NCI, NIH, Surgery Branch, Bethesda, MD (United States); Bratslavsky, Gennady [NCI, NIH, Urologic Oncology Branch, Bethesda, MD (United States); Macholl, Sven; Miller, Matthew P.; Somer, Ed J. [GE Healthcare, Life Sciences, Amersham (United Kingdom); Lindenberg, Liza [NCI, NIH, Molecular Imaging Program, Bethesda, MD (United States); National Cancer Institute, Molecular Imaging Program, Bethesda, MD (United States); Adler, Stephen [Clinical Research Directorate/CMRP SAIC-Frederick Inc., NCI-Frederick, Bethesda, MD (United States); Shih, Joanna [DCTD, NCI, NIH, Biometric Research Branch, Rockville, MD (United States)

    2014-10-15

    [{sup 18}F]Fluciclatide is an integrin-targeted PET radiopharmaceutical. α{sub v}β{sub 3} and α{sub v}β{sub 5} are upregulated in tumor angiogenesis as well as on some tumor cell surfaces. Our aim was to use [{sup 18}F]fluciclatide (formerly known as [{sup 18}F]AH111585) for PET imaging of angiogenesis in melanoma and renal tumors and compare with tumor integrin expression. Eighteen evaluable patients with solid tumors ≥2.0 cm underwent [{sup 18}F]fluciclatide PET/CT. All patients underwent surgery and tumor tissue samples were obtained. Immunohistochemical (IHC) staining with mouse monoclonal antibodies and diaminobenzidine (DAB) was applied to snap-frozen tumor specimens, and additional IHC was done on formalin-fixed paraffin-embedded samples. DAB optical density (OD) data from digitized whole-tissue sections were compared with PET SUV{sub 80%} {sub max}, and Patlak influx rate constant (K{sub i}) data, tumor by tumor. Tumors from all 18 patients demonstrated measurable [{sup 18}F ]fluciclatide uptake. At the final dynamic time-point (55 min after injection), renal malignancies (in 11 patients) demonstrated an average SUV{sub 80%} {sub max} of 6.4 ± 2.0 (range 3.8 - 10.0), while the average SUV{sub 80%} {sub max} for metastatic melanoma lesions (in 6 patients) was 3.0 ± 2.0 (range 0.7 - 6.5). There was a statistically significant difference in [{sup 18}F ]fluciclatide uptake between chromophobe and nonchromophobe renal cell carcinoma (RCCs), with SUV{sub 80%} {sub max} of 8.2 ± 1.8 and 5.4 ± 1.4 (P = 0.020) and tumor-to-normal kidney (T/N) ratios of 1.5 ± 0.4 and 0.9 ± 0.2, respectively (P = 0.029). The highest Pearson's correlation coefficients were obtained when comparing Patlak K{sub i} and α{sub v}β{sub 5} OD when segregating the patient population between melanoma and RCC (r = 0.83 for K{sub i} vs. melanoma and r = 0.91 for K{sub i} vs. RCC). SUV{sub 80%} {sub max} showed a moderate correlation with α{sub v}β{sub 5} and α{sub v}β{sub 3

  1. Arterial spin labelling in imaging of renal diseases and renal allograft pathology

    International Nuclear Information System (INIS)

    Hueper, Katja; Gutberlet, Marcel

    2016-01-01

    Arterial Spin Labelling (ASL) is a technique for non-invasive and contrast-free assessment of perfusion with MRI. Renal ASL allows examination of renal pathophysiology, evaluation of the course of renal disease and therapy effects by longitudinal measurements as well as characterization of renal tumors. In this article, techniques of ASL will be explained and challenges of renal ASL will be emphasized. In addition, examples for clinical application of ASL for diagnosis of renal disease and renal allograft pathology will be given.

  2. The Heterogeneity Between Lynch-Associated and Sporadic MMR Deficiency in Colorectal Cancers.

    Science.gov (United States)

    Liu, Guo-Chen; Liu, Ran-Yi; Yan, Jun-Ping; An, Xin; Jiang, Wu; Ling, Yi-Hong; Chen, Jie-Wei; Bei, Jin-Xin; Zuo, Xiao-Yu; Cai, Mu-Yan; Liu, Ze-Xian; Zuo, Zhi-Xiang; Liu, Ji-Hong; Pan, Zhi-Zhong; Ding, Pei-Rong

    2018-02-20

    Previous studies demonstrated that prognosis of germline deficiency in mismatch repair protein (dMMR) was different from that of sporadic dMMR. The underlying mechanism has not been studied. From a prospectively maintained database, we collected dMMR colorectal cancer (CRC) patients identified by postoperative immunohistochemistry screening. According to genetic test, patients were grouped as Lynch-associated or sporadic dMMR. We compared the clinical-pathological features, prognosis, and immunoreactive differences between the two groups. By whole-exome sequencing and neoantigen detection pipeline, mutational frequencies and neoantigen burdens were also compared. All statistical tests were two-sided. Sixty-seven sporadic dMMR and 85 Lynch-associated CRC patients were included in the study. Sporadic dMMR patients were older (P < .001) and their tumors were poorly differentiated (P = .03). The survival was better in the Lynch-associated group (P = .001). After adjustment, the difference still remained statistically significant (hazard ratio = 0.29, 95% confidence interval = 0.09 to 0.95, P = .04). The scores of Crohn's-like reaction (CRO; P < .001), immunoreactions in the invasive margin (IM; P = .01), tumor stroma (TS; P = .009), and cancer nest (CN; P = .02) of the Lynch-associated group were statistically significantly higher. The numbers of CD3+, CD8+, Foxp3+ tumor-infiltrating lymphocytes (TILs) in IM; CD3+, CD4+ TILs in TS; and CD3+, CD4+, CD8+ TILs in CN were statistically significantly higher in Lynch-associated dMMR patients. Based on the 16 patients who under went whole-exome sequencing, there were also more somatic mutations and neoantigen burdens in the Lynch-associated group compared with the sporadic dMMR group (439/pt vs 68/pt, P = .006; 628/pt vs 97/pt, P = .009). There are heterogeneities in dMMR CRCs. Lynch-associated dMMR patients present with more somatic mutations and neoantigens compared with sporadic dMMR, which probably results in stronger

  3. Cryoablation of Renal Angiomyolipoma

    DEFF Research Database (Denmark)

    Makki, Ahmad; Graumann, Ole; Høyer, Søren

    2017-01-01

    BACKGROUND: Small series have reported that cryoablation (CA) is a safe and feasible minimally invasive nephron-sparing alternative for the treatment of renal angiomyolipomas (renal AMLs). The aim of the present study was to investigate the safety and efficacy of CA in patients with renal AML....... MATERIALS AND METHODS: A retrospective review of 19 renal AML lesions treated with CA at Aarhus University Hospital, Denmark, over a 5-year period. RESULTS: The treatment was performed as laparoscopy-assisted CA on 7 lesions, and in the remaining 12 lesions CA was performed as a percutaneous ultrasound......-guided CA. The mean patient age was 46 years [interquartile range (IQR) 30] and the mean tumor volume was 50.1 cm(3) (IQR 53.3). In all cases, the procedure was effectively conducted with no conversion to open surgery, and no major complications were experienced. The mean follow-up time was 25 months (IQR...

  4. Risk factors for sporadic ovarian cancer

    Directory of Open Access Journals (Sweden)

    M. M. Vysotsky

    2010-01-01

    Full Text Available The review of the literature on the problems of sporadic ovarian cancer details the present views of its disputable risk factors, such as dietary habits, body weight, contraception, and labor, and age of commencing a sexual activity. It discusses the dietary and sexual behavior model that has changed since the Neolithic, as well as the number of menses and ovulations throughout the reproductive peri- od. The works by authors dealing with the impact of smoking and alcohol consumption on the risk of ovarian cancer are analyzed.

  5. A comparative study of ultrasonography, computed tomography and magnetic resonance imaging in the staging and invasion of adjacent structures by renal tumors; Estudo comparativo dos metodos de ultrassonografia, tomografia computadorizada e ressonancia magnetica no estadiamento e invasao das estruturas adjacentes por tumores renais

    Energy Technology Data Exchange (ETDEWEB)

    Ribeiro, S.M.; Ajzen, S.A.; Trindade, J.C.S. [Universidade Federal de Sao Paulo (UNIFESP), SP (Brazil). Faculdade de Medicina; UNESP, Botucatu, SP (Brazil). Faculdade de Medicina; E-mail: sribeiro@fmb.unesp.br

    2001-09-01

    Ultrasonography (US), Computed Tomography (CT), and Magnetic Resonance imaging (MR) were compared for the staging of renal tumors. The differences between these imaging techniques were also studied for their ability to detect adenopathies, vascular invasion, distant intra-abdominal metastases, and particularly adjacent organ invasion. Thirty-one patients with solid or complex renal masses were prospectively studied using US, CT, and MR. Differences between the results obtained were studied using the Cochran G test and the Mc NEMAR test. The sensitivity and specificity of each diagnostic technique were compared against a 'gold standard' of the surgical and histopathological findings. The following sensitivities were obtained: For the detection of adenopathy, US 63.6%, CT and MR 90.9%. For vascular invasion, US 42.8%, CT and MR 85.7%. For the adjacent organ invasion, US 28.5%, CT 85.7%, and MR 71.4%. Some of the criteria that suggest invasion of adjacent structures include: the envelopment of the adjacent structures by the tumor, tumor extension into the adjacent structures with an irregular appearance, and alterations in shape, size, and density of adjacent structures. Loss of fat planes between the tumor and adjacent structures is not a sign of tumor invasion. Significant differences were found in the detection capacity of US in relation to CT and MR, which were similar. All three techniques were highly sensitive and specific only in the detection of distant abdominal metastases. In addition to the accuracy of these diagnostic modalities for the detection and staging of tumors, invasiveness, risks and cost should be considered in relation to relative costs and benefits. (author)

  6. Gastric Medullary Carcinoma with Sporadic Mismatch Repair Deficiency and a TP53 R273C Mutation: An Unusual Case with Wild-Type BRAF

    Directory of Open Access Journals (Sweden)

    Brett M. Lowenthal

    2017-01-01

    Full Text Available Medullary carcinoma has long been recognized as a subtype of colorectal cancer associated with microsatellite instability and Lynch syndrome. Gastric medullary carcinoma is a very rare neoplasm. We report a 67-year-old male who presented with a solitary gastric mass. Total gastrectomy revealed a well-demarcated, poorly differentiated carcinoma with an organoid growth pattern, pushing borders, and abundant peritumoral lymphocytic response. The prior cytology was cellular with immunohistochemical panel consistent with upper gastrointestinal/pancreaticobiliary origin. Overall, the histopathologic findings were consistent with gastric medullary carcinoma. A mismatch repair panel revealed a mismatch repair protein deficient tumor with loss of MLH1 and PMS2 expression. BRAF V600E immunostain (VE1 and BRAF molecular testing were negative, indicating a wild-type gene. Tumor sequencing of MLH1 demonstrated a wild-type gene, while our molecular panel identified TP53 c.817C>T (p.R273C mutation. These findings were compatible with a sporadic tumor. Given that morphologically identical medullary tumors often occur in Lynch syndrome, it is possible that mismatch repair loss is an early event in sporadic tumors with p53 mutation being a late event. Despite having wild-type BRAF, this tumor is sporadic and unrelated to Lynch syndrome. This case report demonstrates that coordinate ancillary studies are needed to resolve sporadic versus hereditary rare tumors.

  7. Early Detection of Sporadic Pancreatic Cancer

    Science.gov (United States)

    Kenner, Barbara J.; Chari, Suresh T.; Cleeter, Deborah F.; Go, Vay Liang W.

    2015-01-01

    Abstract Innovation leading to significant advances in research and subsequent translation to clinical practice is urgently necessary in early detection of sporadic pancreatic cancer. Addressing this need, the Early Detection of Sporadic Pancreatic Cancer Summit Conference was conducted by Kenner Family Research Fund in conjunction with the 2014 American Pancreatic Association and Japan Pancreas Society Meeting. International interdisciplinary scientific representatives engaged in strategic facilitated conversations based on distinct areas of inquiry: Case for Early Detection: Definitions, Detection, Survival, and Challenges; Biomarkers for Early Detection; Imaging; and Collaborative Studies. Ideas generated from the summit have led to the development of a Strategic Map for Innovation built upon 3 components: formation of an international collaborative effort, design of an actionable strategic plan, and implementation of operational standards, research priorities, and first-phase initiatives. Through invested and committed efforts of leading researchers and institutions, philanthropic partners, government agencies, and supportive business entities, this endeavor will change the future of the field and consequently the survival rate of those diagnosed with pancreatic cancer. PMID:25938853

  8. Transarterial embolization (TAE) as add-on to percutaneous radiofrequency ablation (RFA) for the treatment of renal tumors: Review of the literature, overview of state-of-the-art embolization materials and further perspective of advanced image-guided tumor ablation

    Energy Technology Data Exchange (ETDEWEB)

    Sommer, C.M., E-mail: christof.sommer@med.uni-heidelberg.de [Clinic for Diagnostic and Interventional Radiology, University Hospital Heidelberg, Heidelberg (Germany); Clinic for Diagnostic and Interventional Radiology, Klinikum Stuttgart, Katharinenhospital, Stuttgart (Germany); Pallwein-Prettner, L., E-mail: leo.pallwein-prettner@bhs.at [Department of Diagnostic and Interventional Radiology, Krankenhaus der Barmherzigen Schwestern Linz, Linz (Austria); Vollherbst, D.F., E-mail: dominik@vollherbst.de [Clinic for Radiology, Minimally-Invasive Therapies and Nuclear Medicine, SLK Kliniken Heilbronn GmbH, Heilbronn (Germany); Seidel, R., E-mail: roland.seidel@uks.eu [Clinic for Diagnostic and Interventional Radiology, Saarland University Medical Center, Homburg/Saar (Germany); Rieder, C., E-mail: christian.rieder@mevis.fraunhofer.de [Fraunhofer MEVIS, Institute for Medical Image Computing, Bremen (Germany); Radeleff, B.A., E-mail: boris.radeleff@med.uni-heidelberg.de [Clinic for Diagnostic and Interventional Radiology, University Hospital Heidelberg, Heidelberg (Germany); Kauczor, H.U., E-mail: hu.kauczor@med.uni-heidelberg.de [Clinic for Diagnostic and Interventional Radiology, University Hospital Heidelberg, Heidelberg (Germany); Wacker, F., E-mail: wacker.frank@mh-hannover.de [Department of Diagnostic and Interventional Radiology, Hannover Medical School, Hannover (Germany); Richter, G.M., E-mail: g.richter@klinikum-stuttgart.de [Clinic for Diagnostic and Interventional Radiology, Klinikum Stuttgart, Katharinenhospital, Stuttgart (Germany); Bücker, A., E-mail: arno.buecker@uks.eu [Clinic for Diagnostic and Interventional Radiology, Saarland University Medical Center, Homburg/Saar (Germany); Rodt, T., E-mail: rodt.thomas@mh-hannover.de [Department of Diagnostic and Interventional Radiology, Hannover Medical School, Hannover (Germany); and others

    2017-01-15

    Highlights: • TAE as add-on to percutaneous RFA is feasible, safe, and very effective. • State-of-the-art embolization materials include tightly-size-calibrated microspheres. • MWA, cryoablation and IRE are TA systems beyond RFA. • Visible beads rank among the most promising innovative embolization materials. • Software-based solutions will be increasingly important for treatment guidance. - Abstract: Percutaneous radiofrequency ablation (RFA) for the treatment of stage I renal cell carcinoma has recently gained significant attention as the now available long-term and controlled data demonstrate that RFA can result in disease-free and cancer-specific survival comparable with partial and/or radical nephrectomy. In the non-controlled single center trials, however, the rates of treatment failure vary. Operator experience and ablation technique may explain some of the different outcomes. In the controlled trials, a major limitation is the lack of adequate randomization. In case reports, original series and overview articles, transarterial embolization (TAE) before percutaneous RFA was promising to increase tumor control and to reduce complications. The purpose of this study was to systematically review the literature on TAE as add-on to percutaneous RFA for renal tumors. Specific data regarding technique, tumor and patient characteristics as well as technical, clinical and oncologic outcomes have been analyzed. Additionally, an overview of state-of-the-art embolization materials and the radiological perspective of advanced image-guided tumor ablation (TA) will be discussed. In conclusion, TAE as add-on to percutaneous RFA is feasible and very effective and safe for the treatment of T1a tumors in difficult locations and T1b tumors. Advanced radiological techniques and technologies such as microwave ablation, innovative embolization materials and software-based solutions are now available, or will be available in the near future, to reduce the limitations of

  9. Sporadic colorectal cancer: Studying ways to an end

    Science.gov (United States)

    Fidalgo, Paulo; Filipe, Bruno; Albuquerque, Cristina; Fonseca, Ricardo; Chaves, Paula; Pereira, António D

    2015-01-01

    Introduction Although colorectal cancer (CRC) has often been regarded as a single entity, different pathways may lead to macroscopically similar cancers. These pathways may evolve into a patchy colonic field defect that we aimed to study in consecutive CRC patients. Methods In a single-center, observational, prospective study, consecutive CRC patients were included if surgery and a perioperative colonoscopy were planned. Personal and familial history data were collected. Tumors were studied for microsatellite instability (MSI) status, DNA repair protein expression (DRPE) and presence of BRAF and/or APC mutations. Macroscopically normal mucosa samples were tested for APC mutations. Presence and location of synchronous and metachronous adenomas and patient follow-up were analyzed. The association of two categorical variables was tested through the Fisher’s exact test (SPSS 19). Results Twenty-four patients (12 male, mean age 69 years) were studied. High-grade MSI (MSI-H) was found in eight tumors—these were significantly more common in the right colon (p = 0.047) and more likely to have an altered DRPE (p = 0.007). BRAF mutation was found in two of six tested MSI-H tumors. APC gene mutations were found in nine of 16 non-MSI-H tumors and absent in normal mucosa samples. There was a nonsignificant co-localization of CRC and synchronous adenomas and a significant co-localization (p = 0.05) of synchronous and metachronous adenomas. Discussion Sporadic CRCs evolve through distinct pathways, evidenced only by pathological and molecular analysis, but clinically relevant both for patients and their families. In non-MSI-H tumors, the expected APC gene mutations were not detected by the most commonly used techniques in a high number of cases. More studies are needed to fully characterize these tumors and to search for common early events in normal mucosa patches, which might explain the indirect evidence found here for a field defect in the colon. PMID:27087959

  10. Tumour suppressor genes in sporadic epithelial ovarian cancer

    DEFF Research Database (Denmark)

    Liu, Ying; Ganesan, Trivadi S

    2002-01-01

    Ovarian cancer is the most frequent cause of death from gynaecological malignancies in the western world, and sporadic epithelial ovarian cancer is its most predominant form. The aetiology of sporadic ovarian cancer remains unknown. Genetic studies have enabled a better understanding...... of the evolution of tumour progression. A major focus of research has been to identify tumour suppressor genes implicated in sporadic ovarian cancer over the past decade. Several tumour suppressor genes have been identified by strategies such as positional cloning and differential expression display. Further...... research is warranted to understand fully their contribution to the pathogenesis of sporadic ovarian cancer....

  11. Renal cell carcinoma in a horseshoe kidney and preoperative superselective renal artery embolization: a case report

    International Nuclear Information System (INIS)

    Kim, Tae Hoon

    2005-01-01

    Only rarely is renal cell carcinoma encountered in a horseshoe kidney. This is a case report on renal cell carcinoma in a horseshoe kidney, in which superselective renal artery embolization was performed preoperatively. CT and digital subtraction angiography revealed a horseshoe kidney with a 3-cm tumor in the left side. Superselective renal artery embolization of the tumor was performed as a prerequisite procedure for the organ-preserving surgery of simple enucleation. Preoperative superselective renal artery embolization can be an effective tool to facilitate organ-preserving surgery in a horseshoe kidney

  12. Contrast-enhanced three-dimensional MR imaging using a volumetric interpolated breath-hold examination (VIBE): clinical utility in the evaluation of renal tumors

    International Nuclear Information System (INIS)

    Lee, Young Hwan; Kim, Chong Soo; Lee, Jeong Min

    2002-01-01

    To compare, in terms of technical feasibility, image quality and clinical efficacy, contrast-enhanced three-dimensional (3D) MR imaging using volumetric interpolated breath-hold examination (VIBE) with two-dimensional gradient-echo MR imaging for the evaluation of renal messes. Twenty-three patients with 25 renal masses underwent dynamic MR imaging using a 1.5-T MR system and the 3D VIBE, 2D fast low angle shot (FLASH), and combined fat saturation techniques after the injection of 20 ml of Gd-DTPA. We compared postcontrast 2D FLASH and 3D VIBE images with precontrast 2D FLASH images. For quantitative analysis, the signal-to-noise and lesion to kidney contrast-to-noise ratio of the images were calculated using the three different techniques. For qualitative analysis, two experienced radiologists analyzed the images in terms of artifacts, lesion conspicuity and delineation, and general image quality. Delineation of the anatomy of renal vasculature and pelvocalyceal systems on reconstructed 3D VIBE MIP images was also assessed. Quantitative analysis showed that the SNR of a renal mass was slightly higher at postcontrast 2D FLASG than at 3D VIBE imaging, and the SNR of renal cortex was higher at 3D VIBE than at postcontrast 2D FLASF imaging. The differences were, though, statistically insignificant (p>0.05). The CNR of al renal mass was, however, significantly higher at 3D VIBE than at 2D FLASH imaging (p<0.05). Qualitative analysis showed that general image quality was best at postcontrast 3D VIBE, followed by 2D FLASH and precontrast 2D FLASH imaging, and image artifacts were worst at post-contrast 2D FLASH image (p<0.05). In terms of lesion conspicuity and delineation, 3D VIBE gave the best results and postcontrast images were better than precontrast (p<0.05). Reconstructed angiographic and urographic images using the VIBE technique provided information about the anatomy of the renal vasculature and pelvocalyceal system. 3D VIBE MR imaging offers comparable or

  13. BRCA1 and BRCA2 Gene Mutations Screening In Sporadic Breast Cancer Patients In Kazakhstan.

    Directory of Open Access Journals (Sweden)

    Ainur R. Akilzhanova

    2013-05-01

    Full Text Available Background: A large number of distinct mutations in the BRCA1 and BRCA2 genes have been reported worldwide, but little is known regarding the role of these inherited susceptibility genes in breast cancer risk among Kazakhstan women. Aim: To evaluate the role of BRCA1/2 mutations in Kazakhstan women presenting with sporadic breast cancer. Methods: We investigated the distribution and nature of polymorphisms in BRCA1 and BRCA2 entire coding regions in 156 Kazakhstan sporadic breast cancer cases and 112 age-matched controls using automatic direct sequencing. Results: We identified 22 distinct variants, including 16 missense mutations and 6 polymorphisms in BRCA1/2 genes. In BRCA1, 9 missense mutations and 3 synonymous polymorphisms were observed. In BRCA2, 7 missense mutations and 3 polymorphisms were detected. There was a higher prevalence of observed mutations in Caucasian breast cancer cases compared to Asian cases (p<0.05; higher frequencies of sequence variants were observed in Asian controls. No recurrent or founder mutations were observed in BRCA1/2 genes. There were no statistically significant differences in age at diagnosis, tumor histology, size of tumor, and lymph node involvement between women with breast cancer with or without the BRCA sequence alterations. Conclusions: Considering the majority of breast cancer cases are sporadic, the present study will be helpful in the evaluation of the need for the genetic screening of BRCA1/2 mutations and reliable genetic counseling for Kazakhstan sporadic breast cancer patients. Evaluation of common polymorphisms and mutations and breast cancer risk in families with genetic predisposition to breast cancer is ongoing in another current investigation. 

  14. Fluorescent and chromogenic in situ hybridization of CEN17q as a potent useful diagnostic marker for Birt-Hogg-Dubé syndrome-associated chromophobe renal cell carcinomas.

    Science.gov (United States)

    Kato, Ikuma; Iribe, Yasuhiro; Nagashima, Yoji; Kuroda, Naoto; Tanaka, Reiko; Nakatani, Yukio; Hasumi, Hisashi; Yao, Masahiro; Furuya, Mitsuko

    2016-06-01

    Birt-Hogg-Dubé syndrome (BHD) is a familial disorder associated with a germline mutation of FLCN that is a tumor suppressor gene. Patients with BHD have high risks for developing multiple renal cell carcinomas (RCCs). The frequent histological types are hybrid oncocytic/chromophobe tumors (HOCTs) and chromophobe RCCs. The morphology of HOCTs could alert pathologists to the possibility of BHD. On the other hand, chromophobe RCCs occurring in BHD patients demonstrate positive immunostaining for cytokeratin-7, CD82, and Ksp-cadherin similar to their sporadic counterparts. Highly reliable markers for BHD-associated chromophobe RCCs have not been identified. In the present study, we analyzed the state of chromosome 17 in 18 renal tumors composed of 8 chromophobe RCCs, 7 HOCTs, and 3 papillary RCCs obtained from BHD patients using fluorescent and chromogenic in situ hybridization probes for the centromeric region of chromosome 17 long arm. All chromophobe RCCs and HOCTs were disomic except for 1 chromophobe RCC that showed monosomy. On the other hand, 12 of 14 sporadic chromophobe RCCs were monosomic (P = .0008). The state of chromosomes 2 and 6 were also statistically different (P = .0074 and P = .0007, respectively). Three BHD-associated papillary RCCs demonstrated either trisomy (n = 2) or disomy (n = 1). Three of 5 sporadic papillary RCCs showed trisomy. The results indicate that fluorescent and chromogenic in situ hybridization of the centromeric region of chromosome 17 long arm should be a potent useful marker for chromophobe RCCs in patients who have not been diagnosed with BHD and thereby help to determine whether the cases should be considered for genetic testing. Copyright © 2016 Elsevier Inc. All rights reserved.

  15. Renal Ultrasound in the Diagnosis of the Non-functioning Kidney

    International Nuclear Information System (INIS)

    Kang, Ik Won; Suh, Jeong Soo

    1982-01-01

    Renal ultrasound is independent of renal function and capable of renal imaging in impaired or dysplastic renal diseases. Authors reviewed renal ultrasonographic findings of 36 cases which showed non-visualization in intravenous pyelography from Feb. 1979 to Sep. 1982 at Seoul National university Hospital. The results are as follows: 1. Causes of non-visualization of the kidney in IVP were unilateral hydronephrosis(18 cases), renal tuberculosis(7), renal failure(6), renal agenesis(3), tumor(1),and pyonephrosis(1) 2. The sonographic findings were diagnostic in all the cases of unilateral hydronephrosis, renal agenesis and renal tumor. 3. The sonographic findings were not diagnostic but suggestive in more than half cases of renal tuberculosis. 4. Renal ultrasound was not helpful in the diagnosis of renal failure, but useful in delineation of renal size and shape

  16. MRI of sporadic Creutzfeldt-Jakob disease

    International Nuclear Information System (INIS)

    Kong, A.; Vliet, A. Van der.

    2008-01-01

    Full text: The key MRI findings in five cases of sporadic Creutzfeldt-Jakob disease (CJD) are illustrated with four 'definite' and one 'probable' according to World Health Organization criteria. Close attention to fluid-attenuation inversion recovery and diffusion-weighted imaging sequences are important for diagnosis, noting especially restricted diffusion in cortical and deep grey matter. Our study and those of others show predominant cortical, caudate and thalamic involvement. This pattern is highly sensitive and specific for the diagnosis. Fluid-attenuation inversion recovery and diffusion-weighted imaging signal abnormality becomes progressively more extensive and bilateral as disease progresses, but may become less pronounced in end-stage disease because of atrophy.

  17. Tumor necrosis adds prognostically significant information to grade in clear cell renal cell carcinoma: A study of 842 consecutive cases from a single institution. Khor LY, Dhakal HP, Jia X, Reynolds JP, McKenney JK, Rini BI, Magi-Galluzzi C, Przybycin CG.Am J Surg Pathol. September 2016;40(9):1224-1231.

    Science.gov (United States)

    Kryvenko, Oleksandr N

    2017-06-01

    Tumor necrosis has been shown to be an independent predictor of adverse outcome in renal cell carcinoma. A modification of the International Society of Urological Pathology (ISUP) grading system for renal cell carcinomas has recently been proposed, which incorporates the presence of tumor necrosis into grade. The investigators proposing this system found that necrosis added significant prognostic information to ISUP grade. We attempted to describe our experience with the effect of tumor necrosis in relationship to nuclear grade by reviewing the slides from a large consecutive series of localized clear cell renal cell carcinomas from our institution and obtaining long-term clinical follow-up information (overall survival). Of the 842 clear cell renal cell carcinomas reviewed, 265 (31.5%) were ISUP grade 1 or 2, 437 (51.9%) were ISUP grade 3, and 140 (16.6%) were ISUP grade 4. Tumor necrosis was present in 177 (21%) cases. A total of 547 (64.9%) cases were stage pT1, 83 (9.9%) were stage pT2, 193 (22.9%) were stage pT3a, and 19 (2.3%) were pT3b or higher. Median follow-up was 73.2 months (range: 0.12-273.6), and 310 (36.8%) patients died. On univariable analysis, there was no significant difference in outcome for tumors of ISUP grades 1 to 3. After adjustment for age, tumor stage, and tumor size, ISUP grade 4 and necrosis were significant predictors of overall survival on multivariable analysis. When the recently proposed modified grading system incorporating tumor necrosis was applied to our data, there was no significant difference in overall survival between patients with modified grade 1 tumors and those with modified grade 2 tumors (P = 0.31); however, there was a statistically significant difference between patients with modified grade 1 or 2 tumors and those with modified grade 3 tumors (P = 0.04), and a substantial difference in outcome between those with modified grade 3 and modified grade 4 tumors (P10% necrosis. In conclusion, our study shows that tumor

  18. Dietary factors and microsatellite instability in sporadic colon carcinomas

    NARCIS (Netherlands)

    Diergaarde, B.; Braam, H.; Muijen, van G.N.P.; Ligtenberg, M.J.L.; Kok, F.J.; Kampman, E.

    2003-01-01

    Microsatellite instability (MSI) occurs in 10-20% of the sporadic colon carcinomas and appears to be primarily due to alterations in hMLH1 and hMSH2. Little is known about the role of diet in MSI-related colon carcinogenesis. We used data from a Dutch population-based case-control study on sporadic

  19. No evidence of somatic aryl hydrocarbon receptor interacting protein mutations in sporadic endocrine neoplasia

    DEFF Research Database (Denmark)

    Raitila, A; Georgitsi, M; Karhu, A

    2007-01-01

    . Here, we have analyzed 32 pituitary adenomas and 79 other tumors of the endocrine system for somatic AIP mutations by direct sequencing. No somatic mutations were identified. However, two out of nine patients with prolactin-producing adenoma were shown to harbor a Finnish founder mutation (Q14X...... as non-secreting pituitary adenomas have been reported, most mutation-positive patients have had growth hormone-producing adenomas diagnosed at relatively young age. Pituitary adenomas are also component tumors of some familial endocrine neoplasia syndromes such as multiple endocrine neoplasia type 1...... (MEN1) and Carney complex (CNC). Genes underlying MEN1 and CNC are rarely mutated in sporadic pituitary adenomas, but more often in other lesions contributing to these two syndromes. Thus far, the occurrence of somatic AIP mutations has not been studied in endocrine tumors other than pituitary adenomas...

  20. The RET protooncogene in sporadic pheochromocytomas: Frequent multiple endocrine neoplasias type 2 - like mutations and new molecular defects

    Energy Technology Data Exchange (ETDEWEB)

    Beldjord, C.; Desclaux-Arramond, F.; Raffin-Sanson, M. [Universitat Rene Descartes, Paris (France)] [and others

    1994-09-01

    To assess the pathophysiological role of the RET protooncogene in sporadic pheochromocytomas, we examined the two regions of the gene in which molecular defects are specifically associated with the Multiple Endocrine Neoplasias (MEN) type 2A (exons 8-11) and type 2B (exon 16). The sequences of both regions were amplified by RT-PCR or PCR from tumor RNA and/or constitutive DNA. The amplified products were analyzed by denaturing gradient gel electrophoresis using chemical clamps. In 28 patients with unilateral sporadic tumors six RET mutations were found: three in the MEN2A region, and three in the MEN2B region. Five patients had missense mutations: two in the MEN2A region (C634W and D631Y), and three in the MEN2B region (M918T). Analysis of leukocyte DNA in three of these patients confirmed that RET mutations were only present in tumor DNA. The sixth patient had lost exon 10 in the tumor cDNA due to the deletion of the dinucleotide AG at the 3{prime} splice acceptor site of intron 9; this molecular defect was only found in the tumor DNA. Thus RET mutations of the MEN 2A and MEN 2B regions are also found in ca. 20% of sporadic pheochromocytomas. We describe new types of molecular defects of the RET protooncogene in the MEN2A region which involve non-cysteine residues and the loss of exon 10. Further studies should be extended to analyze the entire RET gene. These findings have a profound clinical impact for the management of patients with supposedly sporadic pheochromocytomas.

  1. Renal failure in patients with multiple myeloma.

    Science.gov (United States)

    Almueilo, Samir H

    2015-01-01

    Renal dysfunction is encountered in 20-25% of patients with multiple myeloma (MM) at the time of diagnosis. There is often a precipitating event. Several biochemical and clinical correlations with renal failure in MM have been reported. Renal failure in MM is associated with worse outcome of the disease. We retrospectively analyzed the medical records of 64 patients with MM admitted to our institution during the period January 1992 to December 2012. Abnormal renal function was observed in 24 (37.5%) patients and 17 (26.6%) of them had renal failure; 14 of the 17 (82.4%) of patients with renal failure had Stage III MM. Urine Bence- Jones protein was positive in ten (58.8%) patients with renal failure versus ten (21.3%) patients without renal failure (P = 0.004). Potential precipitating factors of renal failure were determined in nine patients. Renal function normalized in 11 patients with simple measures, while six patients required hemodialysis; one remained dialysis dependent till time of death. Early mortality occurred in five (29.4%) patients with renal failure as compared with two (4.3%) patients in the group without renal failure (P = 0.005). In conclusion, renal failure is associated with a higher tumor burden and Bence-Jones proteinuria in patients with MM. It is reversible in the majority of patients; however, early mortality tends to be higher in patients with persistent renal failure.

  2. Impact of the Tumor Necrosis Factor Receptor-Associated Protein 1 (Trap1) on Renal DNaseI Shutdown and on Progression of Murine and Human Lupus Nephritis

    DEFF Research Database (Denmark)

    Fismen, Silje; Thiyagarajan, Dhivya; Seredkina, Natalya

    2013-01-01

    from the DNaseI gene. Furthermore, we translate these observations to human lupus nephritis. In this study, mouse and human DNaseI and Trap1 mRNA levels were determined by quantitative PCR and compared with protein expression levels and clinical data. Cellular localization was analyzed by immune...... electron microscopy, IHC, and in situ hybridization. Data indicate that silencing of DNaseI gene expression correlates inversely with expression of the Trap1 gene. Our observations suggest that the mouse model is relevant for the aspects of disease progression in human lupus nephritis. Acquired silencing...... of the renal DNaseI gene has been shown to be important for progression of disease in both the murine and human forms of lupus nephritis. Early mesangial nephritis initiates a cascade of inflammatory signals that lead to up-regulation of Trap1 and a consequent down-regulation of renal DNaseI by transcriptional...

  3. Peroxiredoxins, thioredoxin, and Y-box-binding protein-1 are involved in the pathogenesis and progression of dialysis-associated renal cell carcinoma.

    Science.gov (United States)

    Fushimi, Fumiyoshi; Taguchi, Kenichi; Izumi, Hiroto; Kohno, Kimitoshi; Kuwano, Michihiko; Ono, Mayumi; Nakashima, Yutaka; Takesue, Tetsuro; Naito, Seiji; Oda, Yoshinao

    2013-10-01

    Patients with end-stage renal disease are exposed to increased oxidative stress and impairment of antioxidant mechanisms. We focused on dialysis renal cell carcinoma (RCC), including epithelial hyperplasia in acquired cystic disease of the kidney (ACDK). We attempted to obtain insight into the carcinogenesis and tumor progression in terms of cellular defense mechanisms associated with oxidative stress by investigating the expression of antioxidant proteins by immunohistochemistry. We evaluated retrospectively 43 cases of dialysis RCC and, as a control group, 49 cases of sporadic RCC. Peroxiredoxin (Prx) 1, 3, 4, 5, and 6 expression in dialysis RCC was positively correlated with the duration of dialysis. In epithelial hyperplasia, in 17 cases of acquired cystic disease of the kidney, Prxs and thioredoxin were highly expressed. Moreover, in dialysis RCC, Prx 3, 4, and 5 immunoreactivity and nuclear expression of Y-box-binding protein-1 were higher than in sporadic RCC. In dialysis RCC, Prx 3, 4, and 5 immunoreactivity positively correlated with the Fuhrman nuclear grade. These data suggest that oxidative stress during dialysis enhances antioxidant activity, with an inhibiting effect on carcinogenesis. Once cancer has developed, antioxidant activity might have a stimulating effect on the progression of dialysis RCC.

  4. Sporadic aurorae observed in East Asia

    Directory of Open Access Journals (Sweden)

    D. M. Willis

    2007-03-01

    Full Text Available All the accessible auroral observations recorded in Chinese and Japanese histories during the interval AD 1840–1911 are investigated in detail. Most of these auroral records have never been translated into a Western language before. The East Asian auroral reports provide information on the date and approximate location of each auroral observation, together with limited scientific information on the characteristics of the auroral luminosity such as colour, duration, extent, position in the sky and approximate time of occurrence. The full translations of the original Chinese and Japanese auroral records are presented in an appendix, which contains bibliographic details of the various historical sources. (There are no known reliable Korean observations during this interval. A second appendix discusses a few implausible "auroral" records, which have been rejected. The salient scientific properties of all exactly dated and reliable East Asian auroral observations in the interval AD 1840–1911 are summarised succinctly. By comparing the relevant scientific information on exactly dated auroral observations with the lists of great geomagnetic storms compiled by the Royal Greenwich Observatory, and also the tabulated values of the Ak (Helsinki and aa (Greenwich and Melbourne magnetic indices, it is found that 5 of the great geomagnetic storms (aa>150 or Ak>50 during either the second half of the nineteenth century or the first decade of the twentieth century are clearly identified by extensive auroral displays observed in China or Japan. Indeed, two of these great storms produced auroral displays observed in both countries on the same night. Conversely, at least 29 (69% of the 42 Chinese and Japanese auroral observations occurred at times of weak-to-moderate geomagnetic activity (aa or Ak≤50. It is shown that these latter auroral displays are very similar to the more numerous (about 50 examples of sporadic aurorae observed in the United States

  5. Sporadic aurorae observed in East Asia

    Directory of Open Access Journals (Sweden)

    D. M. Willis

    2007-03-01

    Full Text Available All the accessible auroral observations recorded in Chinese and Japanese histories during the interval AD 1840–1911 are investigated in detail. Most of these auroral records have never been translated into a Western language before. The East Asian auroral reports provide information on the date and approximate location of each auroral observation, together with limited scientific information on the characteristics of the auroral luminosity such as colour, duration, extent, position in the sky and approximate time of occurrence. The full translations of the original Chinese and Japanese auroral records are presented in an appendix, which contains bibliographic details of the various historical sources. (There are no known reliable Korean observations during this interval. A second appendix discusses a few implausible "auroral" records, which have been rejected. The salient scientific properties of all exactly dated and reliable East Asian auroral observations in the interval AD 1840–1911 are summarised succinctly. By comparing the relevant scientific information on exactly dated auroral observations with the lists of great geomagnetic storms compiled by the Royal Greenwich Observatory, and also the tabulated values of the Ak (Helsinki and aa (Greenwich and Melbourne magnetic indices, it is found that 5 of the great geomagnetic storms (aa>150 or Ak>50 during either the second half of the nineteenth century or the first decade of the twentieth century are clearly identified by extensive auroral displays observed in China or Japan. Indeed, two of these great storms produced auroral displays observed in both countries on the same night. Conversely, at least 29 (69% of the 42 Chinese and Japanese auroral observations occurred at times of weak-to-moderate geomagnetic activity (aa or Ak≤50. It is shown that these latter auroral displays are very similar to the more numerous (about 50 examples of sporadic

  6. Imaging of renal metastases

    International Nuclear Information System (INIS)

    Bruneton, J.N.; Normand, F.; Balu-Maestro, C.; Rogopoulos, A.; Drouillard, J.; Laurent, F.

    1988-01-01

    Metastases are the most frequent malignant tumors of the kidney, but these lesions are of late onset in neoplastic disease. The 19 cases reported here were all investigated with various imaging techniques (CT 12 cases, ultrasonography 12 cases, urography 8 cases, angiography 2 cases, MRI 1 case). The most common primary malignancies were lung cancer, melanoma and cancer of the controlateral kidney. In this series, 8 of the lesions were solitary, and 9 were unilateral. Tumor vascularity was evaluated in 15 cases: 14 of these lesions were hypovascular. The differential diagnosis includes small cysts, lymphoma, bilateral renal cancer, multiple small abscesses and multiple small infarcts [fr

  7. Retroperitoneal neoplasms within the perirenal space in infants and children: Differentiation of renal and non-renal origin in enhanced CT images

    Energy Technology Data Exchange (ETDEWEB)

    Wu Yinghua [Department of Radiology, West China Hospital, Sichuan University, Chengdu 610041 (China); The Secondly Clinical Medicine College, Chengdu University of Traditional Chinese Medicine, Chengdu 610041 (China); Song Bin, E-mail: cjr.songbin@vip.163.co [Department of Radiology, West China Hospital, Sichuan University, Chengdu 610041 (China); Xu Juan [Department of Radiology, The Fourth People Hospital, Sichuan Province, Chengdu 610015 (China); Chen Weixia [Department of Radiology, West China Hospital, Sichuan University, Chengdu 610041 (China); Zhao Xiaofei; Jia Rui [The Secondly Clinical Medicine College, Chengdu University of Traditional Chinese Medicine, Chengdu 610041 (China); Wu Bi; Li Zhenlin [Department of Radiology, West China Hospital, Sichuan University, Chengdu 610041 (China)

    2010-09-15

    Purpose: To retrospectively demonstrate the specific CT findings of retroperitoneal neoplasms to diagnosis and differential diagnosis renal and non-renal tumors within the perirenal space in infants and children. Materials and methods: We retrospectively reviewed the clinical data and CT images of 42 consecutive patients with surgically and pathologically proven retroperitoneal neoplasms within the perirenal space. The patients were divided into renal tumors group (n = 16) and non-renal tumors group (n = 26). The former included nephroblastoma (n = 15) and renal lymphoma (n = 1), while the latter included neuroblastoma (n = 12), retroperitoneal teratoma (n = 6), adrenal ganglioneuroma (n = 4), retroperitoneal lymphoma (n = 2), ectopic pheochromocytoma (n = 1) and adrenal cortical carcinoma (n = 1). The clinical information of these patients and the major CT imaging findings which were related to lesion localization in the two groups were compared and statistically analyzed using Pearson Chi-Square Test and Risk Estimate. Results: The mean diameter of tumors was 9.82 {+-} 6.13 cm (n = 42 range: 2.3-32 cm). The demographic data and chief clinical symptoms between the renal tumor group and the non-renal tumor group showed no statistically significant differences (P > 0.05). 30.8% (8/26) of non-renal tumor patients presented elevated urinary vanillylmandelic acid (VMA) level, while no patient showed elevated VMA in renal tumor group (P < 0.05). Some CT imaging signs of the renal tumors including 'crescent sign' (odds ratio, OR = 52), 'beak sign' (OR = 84), 'embedded organ sign' (OR = 84), and 'prominent feeding artery sign' (OR = 36) showed significantly higher incidence when compared to the non-renal tumors (P < 0.001). The sign of 'renal displacement and renal axis rotation' (OR = 0.059) was seen in 23 of 26 (88.5%) non-renal tumors, but in only 5 of 16 (31.3%) renal tumors (P < 0.001). The sign of 'extra-renal

  8. Retroperitoneal neoplasms within the perirenal space in infants and children: Differentiation of renal and non-renal origin in enhanced CT images

    International Nuclear Information System (INIS)

    Wu Yinghua; Song Bin; Xu Juan; Chen Weixia; Zhao Xiaofei; Jia Rui; Wu Bi; Li Zhenlin

    2010-01-01

    Purpose: To retrospectively demonstrate the specific CT findings of retroperitoneal neoplasms to diagnosis and differential diagnosis renal and non-renal tumors within the perirenal space in infants and children. Materials and methods: We retrospectively reviewed the clinical data and CT images of 42 consecutive patients with surgically and pathologically proven retroperitoneal neoplasms within the perirenal space. The patients were divided into renal tumors group (n = 16) and non-renal tumors group (n = 26). The former included nephroblastoma (n = 15) and renal lymphoma (n = 1), while the latter included neuroblastoma (n = 12), retroperitoneal teratoma (n = 6), adrenal ganglioneuroma (n = 4), retroperitoneal lymphoma (n = 2), ectopic pheochromocytoma (n = 1) and adrenal cortical carcinoma (n = 1). The clinical information of these patients and the major CT imaging findings which were related to lesion localization in the two groups were compared and statistically analyzed using Pearson Chi-Square Test and Risk Estimate. Results: The mean diameter of tumors was 9.82 ± 6.13 cm (n = 42 range: 2.3-32 cm). The demographic data and chief clinical symptoms between the renal tumor group and the non-renal tumor group showed no statistically significant differences (P > 0.05). 30.8% (8/26) of non-renal tumor patients presented elevated urinary vanillylmandelic acid (VMA) level, while no patient showed elevated VMA in renal tumor group (P < 0.05). Some CT imaging signs of the renal tumors including 'crescent sign' (odds ratio, OR = 52), 'beak sign' (OR = 84), 'embedded organ sign' (OR = 84), and 'prominent feeding artery sign' (OR = 36) showed significantly higher incidence when compared to the non-renal tumors (P < 0.001). The sign of 'renal displacement and renal axis rotation' (OR = 0.059) was seen in 23 of 26 (88.5%) non-renal tumors, but in only 5 of 16 (31.3%) renal tumors (P < 0.001). The sign of 'extra-renal central plane of tumor' (OR = 0.038) was displayed in 24

  9. RENAL MALIGNANT NEOPLASMS: RENAL CELL CARCINOMA

    Directory of Open Access Journals (Sweden)

    Elisangela Giachini

    2017-06-01

    Full Text Available The aim of this study is to evaluate the incidence and prevalence of malignant kidney tumors, to contribute to identifying factors which the diagnosis of renal cell carcinomas. Through this study, we understand that kidney disease over the years had higher incidence rates, especially in adults in the sixth decade of life. The renal cell carcinoma (RCC is the third most common malignancy of the genitourinary tract, affecting 2% to 3% of the population. There are numerous ways of diagnosis; however, the most important are ultrasonography, magnetic resonance imaging and computed tomography. In general most of the patients affected by the CCR, have a good prognosis when diagnosed early and subjected to an effective treatment. This study conducted a literature review about the CCR, through this it was possible to understand the development needs of the imaging methods used for precise diagnosis and classification of RCC through the TNM system.

  10. Renal sonography in 1982

    International Nuclear Information System (INIS)

    Weill, F.; Rohmer, P.; Bihr, E.; Zeltner, F.

    1982-01-01

    In this general review, examination techniques and developments in the field of echo-anatomy and echo-pathology will be described. We shall also underscore present diagnostic limitations, which require complementary diagnostic procedures, whose relationships with sonography will be outlined. Since it is impossible, in such a general review, to deal with the entire renal pathology in sufficient detail, our main topic will be tumoral processes. (orig.)

  11. Primary renal leiomyosarcoma: A diagnostic challenge

    Directory of Open Access Journals (Sweden)

    Shashi Dhawan

    2012-01-01

    Full Text Available Primary leiomyosarcoma is an extremely rare entity constituting only 0.5-1% of all invasive renal tumors. It is frequently diagnosed on histological examination because it does not have any specific diagnostic features clinically and radiologically. At times, it is difficult to differentiate leiomyosarcoma from the sarcomatoid renal cell carcinoma even in histopathology as both the tumors have spindle-shaped atypical cells. Moreover, some epithelial markers can be present in pure smooth muscle sarcomas, while some smooth muscle markers are positive in carcinomas. Hence, a diagnosis of primary renal leiomyosarcoma should be made with caution. Since the prognosis for a renal sarcoma is particularly poor, differentiation from sarcomatoid renal cell carcinoma is necessary. The diagnostic challenge of one such tumor is discussed.

  12. Expression of Opa interacting protein 5 (OIP5) is associated with tumor stage and prognosis of clear cell renal cell carcinoma.

    Science.gov (United States)

    Gong, Mancheng; Xu, Yangyang; Dong, Wenjing; Guo, Guiying; Ni, Wenjun; Wang, Yan; Wang, Yongquan; An, Ruihua

    2013-10-01

    Opa interacting protein 5 (OIP5), overexpressed in some types of human cancers, has been reported to be associated with the carcinogenesis of human cancer. However, the biological function and clinical significance of OIP5 in human Clear Cell Renal Cell Carcinoma (CCRCC) remains unknown. In the present study, we found the expression of OIP5 was markedly upregulated in surgical CCRCC specimens and CCRCC cell lines. Immunohistochemical analysis revealed that paraffin-embedded archival CCRCC specimens exhibited higher levels of OIP5 expression than normal renal tissues. Further statistical analysis suggested the upregulation of OIP5 was positively correlated with the Fuhrman grade (P = 0.02), T classification (P = 0.015), N classification (P = 0.018) and clinical stage (P = 0.035). Also, patients with high OIP5 expression dramatically exhibited shorter survival time (P = 0.001). In addition, the OIP5 expression was an independent prognostic marker of overall survival of CCRCC patients in a multivariate analysis (P = 0.008). Experimentally, we demonstrated that silencing OIP5 in CCRCC cell lines by specific siRNA clearly inhibited cell growth. In conclusion, our findings suggested that OIP5 could be a valuable marker of CCRCC progression and prognosis, and a promising therapeutic target for CCRCC. Copyright © 2013 Elsevier GmbH. All rights reserved.

  13. The evolving genetic risk for sporadic ALS.

    Science.gov (United States)

    Gibson, Summer B; Downie, Jonathan M; Tsetsou, Spyridoula; Feusier, Julie E; Figueroa, Karla P; Bromberg, Mark B; Jorde, Lynn B; Pulst, Stefan M

    2017-07-18

    To estimate the genetic risk conferred by known amyotrophic lateral sclerosis (ALS)-associated genes to the pathogenesis of sporadic ALS (SALS) using variant allele frequencies combined with predicted variant pathogenicity. Whole exome sequencing and repeat expansion PCR of C9orf72 and ATXN2 were performed on 87 patients of European ancestry with SALS seen at the University of Utah. DNA variants that change the protein coding sequence of 31 ALS-associated genes were annotated to determine which were rare and deleterious as predicted by MetaSVM. The percentage of patients with SALS with a rare and deleterious variant or repeat expansion in an ALS-associated gene was calculated. An odds ratio analysis was performed comparing the burden of ALS-associated genes in patients with SALS vs 324 normal controls. Nineteen rare nonsynonymous variants in an ALS-associated gene, 2 of which were found in 2 different individuals, were identified in 21 patients with SALS. Further, 5 deleterious C9orf72 and 2 ATXN2 repeat expansions were identified. A total of 17.2% of patients with SALS had a rare and deleterious variant or repeat expansion in an ALS-associated gene. The genetic burden of ALS-associated genes in patients with SALS as predicted by MetaSVM was significantly higher than in normal controls. Previous analyses have identified SALS-predisposing variants only in terms of their rarity in normal control populations. By incorporating variant pathogenicity as well as variant frequency, we demonstrated that the genetic risk contributed by these genes for SALS is substantially lower than previous estimates. © 2017 American Academy of Neurology.

  14. Choroidal metastasis from renal cell carcinoma presenting with cataract

    International Nuclear Information System (INIS)

    Shoaib, K.K.; Haq, I.; Zafar, N.

    2008-01-01

    We report a case of rare involvement of the eye with choroidal metastasis from renal cell carcinoma presenting 08 years after the primary tumor was removed. The patient initially presented with cataract most probably induced by the tumor. After cataract extraction, tumor was detected when it induced vitreous involvement and retinal detachment. Enucleation was performed and an extraocular mass was also excised. Histopathology confirmed the diagnosis of metastasis from renal cell carcinoma in the ocular tumor, extraocular mass and the vitreous. (author)

  15. Reducing Aircraft Down for Lack of Parts with Sporadic Demand

    National Research Council Canada - National Science Library

    Bachman, Tovey

    2004-01-01

    .... Because of their sporadic demand, it is difficult to decide when to buy these items and in what quantities. As systems become more reliable and failure rates decrease, the number of these infrequently demanded parts is likely to grow...

  16. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease

    Science.gov (United States)

    Kallenberg, K.; Summers, D. M.; Romero, C.; Taratuto, A.; Heinemann, U.; Breithaupt, M.; Varges, D.; Meissner, B.; Ladogana, A.; Schuur, M.; Haik, S.; Collins, S. J.; Jansen, Gerard H.; Stokin, G. B.; Pimentel, J.; Hewer, E.; Collie, D.; Smith, P.; Roberts, H.; Brandel, J. P.; van Duijn, C.; Pocchiari, M.; Begue, C.; Cras, P.; Will, R. G.; Sanchez-Juan, P.

    2009-01-01

    Several molecular subtypes of sporadic Creutzfeldt–Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt–Jakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic Creutzfeldt–Jakob disease molecular subtypes and thus might contribute to the earlier identification of the whole spectrum of sporadic Creutzfeldt–Jakob disease cases. This multi-centre international study aimed to provide a rationale for the amendment of the clinical diagnostic criteria for sporadic Creutzfeldt–Jakob disease. Patients with sporadic Creutzfeldt–Jakob disease and fluid attenuated inversion recovery or diffusion-weight imaging were recruited from 12 countries. Patients referred as ‘suspected sporadic Creutzfeldt–Jakob disease’ but with an alternative diagnosis after thorough follow up, were analysed as controls. All magnetic resonance imaging scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus and cerebellum. Magnetic resonance imaging scans were evaluated in 436 sporadic Creutzfeldt–Jakob disease patients and 141 controls. The pattern of high signal intensity with the best sensitivity and specificity in the differential diagnosis of sporadic Creutzfeldt–Jakob disease was identified. The optimum diagnostic accuracy in the differential diagnosis of rapid progressive dementia was obtained when either at least two cortical regions (temporal, parietal or occipital) or both caudate nucleus and putamen displayed a high signal in fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging. Based on our analyses, magnetic

  17. [Renal angioscopy].

    Science.gov (United States)

    Franco Miranda, E; Rodríguez Tolra, J; Díaz Rodrigues, J; Serrallach Mila, N

    1994-01-01

    Presentation as a novelty of the application of endoscopic methods in the display of the renal artery (angioscopy). Review of findings seen in the renal artery of a donor corpse with polytraumatism using direct view with a MiniScope-type rigid urethroscopy and the possible future application of this technique.

  18. Chromosomal abnormalities in non-neoplastic renal tissue

    NARCIS (Netherlands)

    vandenBerg, E; Dijkhuizen, T; Storkel, S; Molenaar, WM; deJong, B

    1995-01-01

    Chromosome aberrations were studied in short-term cultures of non-neoplastic renal tissue and tumor tissue in 60 patients, 41 male and 19 female, with renal cell cancer (RCC), and in normal renal parenchyma from two cases, one male and one female, at autopsy with non-kidney related disease.

  19. Multiple Renal Artery Pseudoaneurysms in Patients Undergoing Renal Artery Embolization Following Partial Nephrectomy: Correlation with RENAL Nephrometry Scores

    Energy Technology Data Exchange (ETDEWEB)

    Gupta, Nakul [Houston Methodist Hospital (United States); Patel, Anish [The University of Texas Southwestern Medical Center (United States); Ensor, Joe [Houston Methodist Research Institute, The Houston Methodist Cancer Center (United States); Ahrar, Kamran; Ahrar, Judy; Tam, Alda; Odisio, Bruno; Huang, Stephen; Murthy, Ravi; Mahvash, Armeen; Avritscher, Rony; McRae, Stephen; Sabir, Sharjeel; Wallace, Michael [The University of Texas MD Anderson Cancer Center, Department of Interventional Radiology (United States); Matin, Surena [The University of Texas MD Anderson Cancer Center, Department of Urology (United States); Gupta, Sanjay, E-mail: sgupta@mdanderson.org [The University of Texas MD Anderson Cancer Center, Department of Interventional Radiology (United States)

    2017-02-15

    PurposeTo describe the incidence of multiple renal artery pseudoaneurysms (PSA) in patients referred for renal artery embolization following partial nephrectomy and to study its relationship to RENAL nephrometry scores.Materials and MethodsThe medical records of 25 patients referred for renal artery embolization after partial nephrectomy were retrospectively reviewed for the following parameters: size and number of tumors, RENAL nephrometry scores, angiographic abnormalities, technical and clinical outcomes, and estimated glomerular filtration rates (eGFRs) after embolization.ResultsTwenty-four patients had primary renal tumors, while 1 patient had a pancreatic tumor invading the kidney. Multiple tumors were resected in 4 patients. Most patients (92 %) were symptomatic, presenting with gross hematuria, flank pain, or both. Angiography revealed PSA with (n = 5) or without (n = 20) AV fistulae. Sixteen patients (64 %) had multiple PSA involving multiple renal vessels. Higher RENAL nephrometry scores were associated with an increasing likelihood of multiple PSA. Multiple vessels were embolized in 14 patients (56 %). Clinical success was achieved after one (n = 22) or two (n = 3) embolization sessions in all patients. Post-embolization eGFR values at different time points after embolization were not significantly different from the post-operative eGFR.ConclusionA majority of patients requiring renal artery embolization following partial nephrectomy have multiple pseudoaneurysms, often requiring selective embolization of multiple vessels. Higher RENAL nephrometry score is associated with an increasing likelihood of multiple pseudoaneurysms. We found transarterial embolization to be a safe and effective treatment option with no long-term adverse effect on renal function in all but one patient with a solitary kidney.

  20. Impact of the off-clamp endoscopic robot-assisted simple enucleation (ERASE) of clinical T1 renal tumors on the postoperative renal function: Results from a matched-pair comparison.

    Science.gov (United States)

    Mari, Andrea; Morselli, Simone; Sessa, Francesco; Campi, Riccardo; Di Maida, Fabrizio; Greco, Isabella; Siena, Giampaolo; Tuccio, Agostino; Vittori, Gianni; Serni, Sergio; Carini, Marco; Minervini, Andrea

    2018-02-13

    To evaluate the surgical and functional outcomes of a matched-paired series of on-clamp vs off-clamp endoscopic robot-assisted simple enucleation (ERASE) and standardized renorraphy in a tertiary referral institution, to search for predictors of functional drop after surgery and to investigate the influence of off-clamp technique in patients presenting these characteristics. A matched-pair comparison of 120 on-clamp vs 120 off-clamp over 491 patients treated with ERASE was performed. Perioperative and functional outcomes were compared between groups. Patients treated with on-clamp and off-clamp technique had comparable complication and positive surgical margin rate. The off-clamp group had a significantly lower eGFR drop compared to the on-clamp group at 3rd postoperative day (POD) (1% vs 7%, p = 0.0001) and at 30th POD (2.5% vs 9%, p = 0.01) from baseline. This difference lost its statistical significance at 6th month and at last follow-up (median 40 months). At multivariable analysis the Charlson comorbidity index (OR 2.06, p 15% eGFR drop from baseline to last follow-up. In a subanalysis over 64 comorbid patients, those patients who underwent off-clamp ERASE had a significantly lower eGFR drop compared to the comorbid counterpart during the whole follow-up. The off-clamp ERASE is a safe surgical technique with a significantly lower renal function drop compared to on-clamp ERASE in the early perioperative time. Patients with comorbidity might represent a subgroup of patients having a functional benefit after off-clamp RAPN even in the long-term period. Copyright © 2018 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.

  1. Clinical and pathological features of papillary renal cell carcinoma ...

    African Journals Online (AJOL)

    M.M. Gargouri

    2016-08-21

    Aug 21, 2016 ... Abstract. Introduction and objectives: Papillary renal cell carcinoma (PRCC) accounts for 10–15% of renal tumors in adults. This type of tumor contains more than 75% of tubulo-papillary structures and is divided histologically into two subtypes. The distinction between these two subtypes is essential ...

  2. Clinical and pathological features of papillary renal cell carcinoma ...

    African Journals Online (AJOL)

    Introduction and objectives: Papillary renal cell carcinoma (PRCC) accounts for 10–15% of renal tumors in adults. This type of tumor contains more than 75% of tubulo-papillary structures and is divided histologically into two subtypes. The distinction between these two subtypes is essential because of their prognostic value.

  3. Tratamento endovascular de angiomiolipoma renal por embolização arterial seletiva Endovascular treatment of renal angiomiolipoma by selective arterial embolization

    Directory of Open Access Journals (Sweden)

    Renato Menezes Palácios

    2012-12-01

    Full Text Available Relata-se um caso de um paciente com queixa principal de dor lombar à esquerda, portador de angiomiolipomas renais (AMLRs bilaterais, com a lesão mais volumosa de 6,2 cm em seu maior diâmetro, submetido a tratamento endovascular por embolização arterial superseletiva com microesferas. Os AMLRs são tumores benignos raros. A maioria é esporádica, enquanto uma minoria está associada à Esclerose Tuberosa Complexa (ETC. Os AMLRs maiores do que 4 cm devem ser tratados devido ao maior risco de complicações, principalmente hemorrágicas. A embolização arterial seletiva (EAS é um tratamento efetivo e seguro para os AMLRs.We report a case of a patient with a major complaint of left lumbar pain, diagnosed with bilateral renal angiomyolipomas (AMLRs, with the most voluminous lesion of 6.2 cm in its largest diameter, underwent endovascular superselective arterial embolization with microspheres. The AMLRs are rare benign tumors. Most are sporadic, while a minority is associated with Tuberous Sclerosis Complex (ETC. The AMLRs larger than 4 cm must be treated due to higher risk of complications, especially hemorrhagic. A selective arterial embolization (EAS is an effective and safe treatment for AMLRs.

  4. Evaluation of the prognostic significance of perirenal fat invasion and tumor size in patients with pT1-pT3a localized renal cell carcinoma in a comprehensive multicenter study of the CORONA project. Can we improve prognostic discrimination for patients with stage pT3a tumors?

    Science.gov (United States)

    Brookman-May, Sabine D; May, Matthias; Wolff, Ingmar; Zigeuner, Richard; Hutterer, Georg C; Cindolo, Luca; Schips, Luigi; De Cobelli, Ottavio; Rocco, Bernardo; De Nunzio, Cosimo; Tubaro, Andrea; Coman, Ioman; Truss, Michael; Dalpiaz, Orietta; Feciche, Bogdan; Figenshau, Robert S; Madison, Kerry; Sánchez-Chapado, Manuel; Santiago Martin, Maria del Carmen; Salzano, Luigi; Lotrecchiano, Giuseppe; Zastrow, Stefan; Wirth, Manfred; Sountoulides, Petros; Shariat, Shahrokh; Waidelich, Raphaela; Stief, Christian; Gunia, Sven

    2015-05-01

    The current TNM system for renal cell carcinoma (RCC) merges perirenal fat invasion (PFI) and renal vein invasion (RVI) as stage pT3a despite limited evidence concerning their prognostic equivalence. In addition, the prognostic value of PFI compared to pT1-pT2 tumors remains controversial. To analyze the prognostic significance of PFI, RVI, and tumor size in pT1-pT3a RCC. Data for 7384 pT1a-pT3a RCC patients were pooled from 12 centers. Patients were grouped according to stages and PFI/RVI presence as follows: pT1-2N0M0 (n=6137; 83.1%), pT3aN0M0 + PFI (n=1036; 14%), and pT3aN0M0 (RVI ± PFI; n=211; 2.9%). Radical nephrectomy or nephron-sparing surgery (NSS) (1992-2010). Cancer-specific survival was estimated using the Kaplan-Meier method. Univariate and multivariate Cox proportional-hazards regression models, as well as sensitivity and discrimination analyses, were used to evaluate the impact of clinicopathologic parameters on cancer-specific mortality (CSM). Compared to stage pT1-2, patients with stage pT3a RCC were significantly more often male (59.4% vs 53.1%) and older (64.9 vs 62.1 yr), more often had clear cell RCC (85.2% vs 77.7%), Fuhrman grade 3-4 (29.4% vs 13.4%), and tumor size >7 cm (39.1% vs 13%), and underwent NSS less often (7.5% vs 36.6%; all p<0.001). According to multivariate analysis, CSM was significantly higher for the PFI and RVI ± PFI groups compared to pT1-2 patients (hazard ratio [HR] 1.94 and 2.12, respectively; p<0.001), whereas patients with PFI only and RVI ± PFI did not differ (HR 1.17; p=0.316). Tumor size instead enhanced CSM by 7% per cm in stage pT3a (HR 1.07; p<0.001) with a 7 cm cutoff yielding the highest prediction accuracy. Since the prognostic impact of PFI and RVI on CSM seems to be comparable, merging both as stage pT3a RCC might be justified. Enhanced prognostic discrimination of stage pT3a RCC appears to be possible by applying a tumor size cutoff of 7 cm within an alternative staging system. Prognosis prediction for

  5. [Heredity in renal and prostatic neoplasia].

    Science.gov (United States)

    Prayer Galetti, T; D'Arrigo, L; De Zorzi, L; Patarnello, T

    1997-09-01

    There is an ever growing report of data supporting the evidence that accumulated genetic changes underlie the development of neoplasia. The paradigma of this multistep process is colon cancer were cancer onset is associated, over decades, with at least seven genetic events. The number of genetic alterations increases moving from adenomatous lesions to colon cancer and, although the genetic alterations occur according to a preferred sequence, the total accumulation of changes rather than their sequential order is responsible of tumor biological behavior. It is noteworthy that, at least for this neoplasia, carcinogenesis appears to arise as a result of the mutational activation of oncogenes coupled with the mutational inactivation of tumor suppressor genes. In some cases mutant suppressor genes appear to exert a phenotypic effect even when present in the heterozygous state thus been non "recessive" at the cellular level. The general features of this model may apply also to renal cell cancer (RCC) and prostate cancer (CaP). Extensive literature exists on the cytogenetic and molecular findings in RCC. Only 2% of RCC are familiar, but molecular genetic studies of these cancers have provided important informations on RCC pathogenesis. As with other cancers, familiar RCC is characterized by an early age of onset and frequent multicentricity. A pathological classification useful in studying these patients subdivide renal cancers in papillary (pRCC) and non papillary (RCC) neoplasms. The most common cause of inherited RCC is the Von Hippel Lindau disease (VHL) a dominantly inherited multisystem disorder characterized by retinal and cerebellar hemangioblastomas, pheochromocytomas, pancreatic cysts and RCC. Over 70% of these patients will develop an RCC by their sixth decade. In 1993 the isolation of the tumor suppressor gene in VHL disease at the level of chromosome 3p25-p26 have lead to a better understanding of RCC. Most missense mutations are associated with high risk of

  6. Evaluation of renal function after laparoscopic partial nephrectomy with renal scintigraphy using 99mtechnetium-mercaptoacetyltriglycine

    International Nuclear Information System (INIS)

    Kobayashi, Yasuyuki; Usui, Yukio; Shima, Masanori; Hoshi, Akio; Terachi, Toshiro; Miyakita, Hideshi; Inatsuchi, Hiroyoshi

    2006-01-01

    We evaluated the functions of an affected kidney after laparoscopic partial nephrectomy (LPN) using renal scintigraphy with 99m technetium-mercaptoacetyltriglycine ( 99m Tc-MAG3). Split renal function of 10 patients who underwent LPN for renal tumors was assessed using renal scintigraphy with 99m TcMAG3 before surgery, and 1 week and 3 months post-surgery. Median operating time was 196.5 mm, median tumor diameter was 2.3 cm, mean blood loss was 64 mL and mean ischemic time was 38.5 mm. Median change in serum creatinine level pre- to post-surgery was 0.15 mg/dL. Median contribution of the affected kidney to total renal function (calculated using 99m Tc-MAG3) was 50.0%, 41.7% and 36.1% before surgery, 1 week and 3 months after LPN, respectively. In one patient, the tumor was resected after cooling of the affected kidney with ice slush for 15 min, and the split renal function ratio remained as high as 50% at 3 months post-operatively despite a total ischemic time of 61 min. This paper evaluated renal function on the affected side before and after surgery by measuring split renal function with renal scintigraphy using 99m Tc-MAG3. Risk factors for renal dysfunction in the affected kidney after LPN include age over 70 years with more than 30 min warm ischemic time, re-clamping of the renal artery procedure, and a warm ischemic time greater than 60 min. We believe that renal cooling with slush ice prevents renal dysfunction of the affected kidney after LPN with longer warm ischemic times. However, an easier renal cooling technique should be sought for regular use of cooling procedures in LPN. (author)

  7. Renal paraneoplastic vasculitis complicating lung adenocarcinoma

    Directory of Open Access Journals (Sweden)

    Besma Ben Dhaou

    2014-01-01

    Full Text Available Renal paraneoplastic vasculitis (RNPV is rare. It can be revealed by glomerulonephritis, microaneurysms or renal failure. RPNV may precede the onset of the primary tumor, and treatment and prognosis depend on the etiology (primary tumor. A 54-year-old man who had a primary lung adenocarcinoma was admitted for nephrotic syndrome. The investigations revealed RNPV. The patient was treated with corticosteroids at high dose and cyclophosphamide with improvement of the renal condition; however, the patient died from worsening of his pulmonary neoplasia.

  8. Whole Exome Sequencing Identifies TSC1/TSC2 Biallelic Loss as the Primary and Sufficient Driver Event for Renal Angiomyolipoma Development.

    Science.gov (United States)

    Giannikou, Krinio; Malinowska, Izabela A; Pugh, Trevor J; Yan, Rachel; Tseng, Yuen-Yi; Oh, Coyin; Kim, Jaegil; Tyburczy, Magdalena E; Chekaluk, Yvonne; Liu, Yang; Alesi, Nicola; Finlay, Geraldine A; Wu, Chin-Lee; Signoretti, Sabina; Meyerson, Matthew; Getz, Gad; Boehm, Jesse S; Henske, Elizabeth P; Kwiatkowski, David J

    2016-08-01

    Renal angiomyolipoma is a kidney tumor in the perivascular epithelioid (PEComa) family that is common in patients with Tuberous Sclerosis Complex (TSC) and Lymphangioleiomyomatosis (LAM) but occurs rarely sporadically. Though histologically benign, renal angiomyolipoma can cause life-threatening hemorrhage and kidney failure. Both angiomyolipoma and LAM have mutations in TSC2 or TSC1. However, the frequency and contribution of other somatic events in tumor development is unknown. We performed whole exome sequencing in 32 resected tumor samples (n = 30 angiomyolipoma, n = 2 LAM) from 15 subjects, including three with TSC. Two germline and 22 somatic inactivating mutations in TSC2 were identified, and one germline TSC1 mutation. Twenty of 32 (62%) samples showed copy neutral LOH (CN-LOH) in TSC2 or TSC1 with at least 8 different LOH regions, and 30 of 32 (94%) had biallelic loss of either TSC2 or TSC1. Whole exome sequencing identified a median of 4 somatic non-synonymous coding region mutations (other than in TSC2/TSC1), a mutation rate lower than nearly all other cancer types. Three genes with mutations were known cancer associated genes (BAP1, ARHGAP35 and SPEN), but they were mutated in a single sample each, and were missense variants with uncertain functional effects. Analysis of sixteen angiomyolipomas from a TSC subject showed both second hit point mutations and CN-LOH in TSC2, many of which were distinct, indicating that they were of independent clonal origin. However, three tumors had two shared mutations in addition to private somatic mutations, suggesting a branching evolutionary pattern of tumor development following initiating loss of TSC2. Our results indicate that TSC2 and less commonly TSC1 alterations are the primary essential driver event in angiomyolipoma/LAM, whereas other somatic mutations are rare and likely do not contribute to tumor development.

  9. Renal angiomyolipoma with inferior vena caval thrombus in a 32-year-old male

    Directory of Open Access Journals (Sweden)

    Matthew W Christian

    2009-01-01

    Full Text Available Renal angiomyolipoma (AML rarely presents with evidence of extension into the renal vein, inferior vena cava (IVC or atrium. We report a case of a renal AML with a tumor thrombus to the IVC in a 32-year-old male. The patient subsequently underwent a right radical nephrectomy with IVC tumor thrombectomy. To our knowledge, there are four published cases of renal AML presenting with tumor thrombus in males. This case report describes the management of the youngest male ever to develop a renal AML with IVC tumor thrombus.

  10. Effect of Bidens pilosa extract on renal functions and some tumor markers of Ehrlich Ascites Carcinoma bearing mice exposed to γ-radiation

    International Nuclear Information System (INIS)

    El-Kabany, H.; Ibrahim, S.I.

    2013-01-01

    The Ethanolic extract of Bidens pilosa (EtBP) was tested in Swiss albino mice transplanted with Ehrlich ascites carcinoma (EAC) and exposed to γ-radiation. EAC mice received intraperitoneal (i.p) 250 mg/kg body weight EtBP for nine days , 24hr after tumor inoculation. Mice exposed to 4 Gy γ-radiation 30 min after the first dose of EtBP. Seventy female mice were classified into 6 groups (15 mice in each group) as follows, control, mice treated with EtBP for 9 consecutive days, mice bearing EAC cells, EAC bearing mice treated with EtBP, 24 hour after tumor inoculation, EAC bearing mice and irradiated, and EAC bearing mice treated with EtBP and exposed to γ-radiation. Five animals from each group were sacrificed 18 hr after administration of the last EtBP dose. Blood and ascetic fluid were collected and kidneys were removed for biochemical and histopathological studies. The remaining animals were observed daily for recording survival percentage and body weight. Results showed that treatment of EAC bearing mice with EtBP and/or exposure to γ- radiation increased the survival percentage of the animals and decreased their body weight compared to EAC group. Inoculation of mice with EAC cells resulted in biochemical and histopathological changes leading to kidney damage. Animals of EAC bearing mice with EtBP and /or exposure to γ- radiation significantly restored the elevated levels of serum urea and creatinine, tumor necrosis factor-alpha (TNF-α), metalo matrix protein (mmp-2 and mmp9), also the elevated level of lipid peroxidation (MDA) in kidneys tissue, compared to EAC group. On the other hand, a significantly decline was observed in glutathione (GSH) and super oxide dismutase (SOD) contents in kidney tissue of EAC group. Treatment of EAC bearing mice with EtBP and/or exposure to γ-radiation resulted in increase GSH and SOD in kidney tissue and increased caspase-3 in ascetic fluid, comparing to EAC group. It could be concluded that EtBP through its antioxidant

  11. Loss of heterozygosity and microsatellite instability are rare in sporadic dedifferentiated liposarcoma: a study of 43 well-characterized cases.

    Science.gov (United States)

    Davis, Jessica L; Grenert, James P; Horvai, Andrew E

    2014-06-01

    Defects in mismatch repair proteins have been identified in Lynch syndrome-associated liposarcomas, as well as in rare sporadic sarcomas. However, it is unclear if mismatch repair defects have a role in sarcoma tumorigenesis. Microsatellite instability is a surrogate marker of mismatch repair defects. To determine whether sporadic dedifferentiated liposarcomas display microsatellite instability and, if so, to evaluate whether such instability differs between the lipogenic and nonlipogenic components of these tumors. The diagnoses of conventional dedifferentiated liposarcoma were confirmed by a combination of morphologic, immunophenotypic, and molecular studies. Standard fluorescence-based polymerase chain reaction, including 5 mononucleotide microsatellite markers (BAT25, BAT26, NR21, NR24, and MONO27), as well as 2 pentanucleotide repeat markers (Penta C and Penta D), was used to test for instability and loss of heterozygosity. We demonstrated only a single case (1 of 43) with microsatellite instability at one mononucleotide marker. No sarcomas showed high-level microsatellite instability. However, loss of heterozygosity at the pentanucleotide markers was observed in 8 of 43 cases. The presence of loss of heterozygosity was overrepresented in the nonlipogenic (dedifferentiated) components compared with the paired lipogenic (well differentiated) components. Mismatch repair defects do not contribute to sporadic dedifferentiated liposarcoma tumorigenesis. Whether the observed loss of heterozygosity drives tumorigenesis in liposarcoma, for example by affecting tumor suppressor or cell cycle regulator genes, remains to be determined.

  12. Malignancy and chronic renal failure.

    Science.gov (United States)

    Peces, Ramon

    2003-01-01

    Increased incidence of cancer at various sites is observed in patients with end-stage renal disease (ESRD). Certain malignant diseases, such as lymphomas and carcinomas of the kidney, prostate, liver and uterus, show an enhanced prevalence compared with the general population. In particular, renal cell carcinoma (RCC) shows an excess incidence in ESRD patients. A multitude of factors, directly or indirectly associated with the renal disease and the treatment regimens, may contribute to the increased tumor formation in these patients. Patients undergoing renal replacement therapy (RRT) are prone to develop acquired cystic kidney disease (ACKD), which may subsequently lead to the development of RCC. In pre-dialysis patients with coexistent renal disease, as in dialysis and transplant patients, the presence of ACKD may predispose to RCC. Previous use of cytotoxic drugs (eg, cyclophosphamide) or a history of analgesic abuse, are additional risk factors for malignancy. Malignancy following renal transplantation is an important medical problem during the follow-up. The most common malignancies are lymphoproliferative disorders (early after transplantation) and skin carcinomas (late after transplantation). Another important confounder for risk of malignancy after renal transplantation is the type of immunosuppression. The type of malignancy is different in various countries and dependent on genetic and environmental factors. Finally, previous cancer treatment in a uremic patient on the transplant waiting list is of great importance in relation to waiting time and post-malignancy screening.

  13. mTOR-inhibitor treatment of metastatic renal cell carcinoma: contribution of Choi and modified Choi criteria assessed in 2D or 3D to evaluate tumor response

    Energy Technology Data Exchange (ETDEWEB)

    Lamuraglia, M. [Laboratoire d' Imagerie Biomedicale, Sorbonne Universites, UPMC Univ Paris 06, INSERM, CNRS, Paris (France); Raslan, S.; Penna, R.R.; Wagner, M. [Groupe Hospitalier Pitie-Salpetriere, APHP UPMC, Service de Radiologie Polyvalente et Oncologique, Paris Cedex 13 (France); Elaidi, R.; Oudard, S. [APHP, Oncology Unit, Georges-Pompidou Hospital, Paris (France); Escudier, B. [Gustave-Roussy Institute, Medical Oncology Department, Villejuif (France); Slimane, K. [Novartis Pharma, Rueil-Malmaison (France); Lucidarme, O. [Groupe Hospitalier Pitie-Salpetriere, APHP UPMC, Service de Radiologie Polyvalente et Oncologique, Paris Cedex 13 (France); Laboratoire d' Imagerie Biomedicale, Sorbonne Universites, UPMC Univ Paris 06, INSERM, CNRS, Paris (France)

    2016-01-15

    To determine whether 2D or 3D Choi and modified Choi (mChoi) criteria could assess the efficacy of everolimus against metastatic renal cell carcinoma (mRCC). RECIST-1.1, Choi, and mChoi criteria were applied retrospectively to analyse baseline and 2-month contrast-enhanced computed tomography (CECT) images in 48 patients with mRCC enrolled in the everolimus arm of the French randomized double-blind multicentre phase III trial comparing everolimus versus placebo (RECORD-1). The primary endpoint was centrally reviewed progression-free survival (PFS) calculated from the initial RECORD-1 analysis. Mean attenuation was determined for 2D target lesion regions of interest drawn on CECT sections whose largest diameters had been measured, and for the 3D whole target lesion. The median PFS was 5.5 months. The median PFS for everolimus responders defined using 3D mChoi criteria was significantly longer than for non-responders (7.6 versus 5.4 months, respectively), corresponding to a hazard ratio for progression of 0.45 (95 % CI: 0.22-0.92), with respective 1-year survival rates of 31 % and 9 %. No other 2D or 3D imaging criteria at 2 months identified patients who would benefit from everolimus. At 2 months, only 3D mChoi criteria were able to identify mRCC patients with a PFS benefit from everolimus. (orig.)

  14. Tumores da córtex da supra-renal: o uso do p53 na diferenciação entre carcinomas e adenomas

    Directory of Open Access Journals (Sweden)

    Falconi Rodrigo A. R.

    2000-01-01

    Full Text Available A marcação imunohistoquímica da proteina p53 foi estudada em tumores da adrenal conservados em formol ou em blocos de parafina, pelo método da avidina-biotina-peroxidase com recuperação antigênica. Foram estudados 24 carcinomas e 26 adenomas com o objetivo de verificar se o marcador mostrava capacidade de distinção entre eles. Em 62,5% dos carcinomas a marcação foi positiva enquanto que nos adenomas foi de 15,4%, diferença essa estatisticamente significante (p=0,0003. A sensibilidade, especificidade e valor preditivo positivo desse marcador para o diagnóstico do câncer foram, respectivamente: 83,3%, 71,8% e 62,5%. Não houve relação entre o índice de marcação e outros parâmetros clínicos, como peso do tumor, estádio local, recidiva e metástases. Os autores concluem que o marcador é útil no diagnóstico diferencial de massas da adrenal, mas não tem relação com a agressividade biológica da neoplasia maligna.

  15. Computed tomographic evaluation of the renal vein and inferior vena cava in renal cell carcinoma

    International Nuclear Information System (INIS)

    Masuda, Fujio; Nakada, Gyojiro; Onishi, Tetsuo; Higashi, Yoichiro; Machida, Toyohei

    1980-01-01

    Renal cell carcinoma not rarely invades the renal vein and inferior vena cava with formation of tumor thrombus. On the other hand, congenital anomalies of these venous channels are occasionally encountered. At the time of surgery of renal cell carcinoma, therefore, preoperative evaluation of the renal vein and inferior vena cava is desirable. In 22 cases of renal cell carcinoma treated at the Jikei University Hospital during 16 months period from Jan. 1979 to apr. 1980, the ranal vein and inferior vena cava were examined by computed tomography (CT). Intravenous tumor thrombus was diagnosed in 4, double inferior vena cava in one and retroaortic left renal vein in one. All these CT diagnosis were confirmed to be correct by surgery. Three of tumor thrombus cases showed involvement of inferior vena cava. CT findings included dilated vein in 4 and filling defect after contrast enhancement in 2. In double inferior vena cava, CT at the level of the upper pole of kidney showed the left inferior vena cava traversing in front of the aorta and conjoining the left inferior vena cava, CT at the level of renal vein the left renal vein draining into the left inferior vena cava and CT at the lower level two round inferior vena cava with the aorta between them. It was a diagnostically useful finding that the left inferior vena cava was markedly stained in CT after contrast enhancement jestas the aorta and the right inferior vena cava. In retroaortic left renal vein, CT showed the left renal vein running behind the aorta and draining into the inferior vena cava. Preoperative information on congenital anomalies of the renal vein and inferior vena cava or tumor thrombus and its extension are very important particularly for radical surgery to be conducted safely without postoperative complications. Computed tomography is quite useful in evaluation of morbid state of the renal vein and inferior vena cava. (author)

  16. Timing the Landmark Events in the Evolution of Clear Cell Renal Cell Cancer: TRACERx Renal

    DEFF Research Database (Denmark)

    Mitchell, Thomas J.; Turajlic, Samra; Rowan, Andrew

    2018-01-01

    Clear cell renal cell carcinoma (ccRCC) is characterized by near-universal loss of the short arm of chromosome 3, deleting several tumor suppressor genes. We analyzed whole genomes from 95 biopsies across 33 patients with clear cell renal cell carcinoma. We find hotspots of point mutations in the...

  17. Renal disease and mitochondrial genetics.

    Science.gov (United States)

    Rötig, Agnès

    2003-01-01

    Respiratory chain (RC) deficiencies have long been regarded as neuromuscular diseases mainly originating from mutations in the mitochondrial DNA. Oxidative phosphorylation, i.e. adenosine triphosphate (ATP) synthesis-coupled electron transfer from substrate to oxygen through the RC, does not occur only in the neuromuscular system. Therefore, a RC deficiency can theoretically give rise to any symptom, in any organ or tissue, at any age and with any mode of inheritance, owing to the dual genetic origin of RC enzymes (nuclear DNA and mitochondrial DNA). Mitochondrial diseases can give rise to various syndromes or association, namely, neurologic and neuromuscular diseases, cardiac, renal, hepatic, hematological and endocrin or dermatological presentations. The most frequent renal symptom is proximal tubular dysfunction with a more or less complete de Toni-Debre-Fanconi Syndrome. A few patients have been reported with tubular acidosis, Bartter Syndrome, chronic tubulointerstitial nephritis or nephrotic syndrome. The diagnosis of a RC deficiency is difficult when only renal symptoms are present, but should be easier when another, seemingly unrelated symptom is observed. Metabolic screening for abnormal oxidoreduction status in plasma, including lactate/pyruvate and ketone body molar ratios, can help to identify patients for further investigations. These include the measurement of oxygen consumption by mitochondria and the assessment of mitochondrial respiratory enzyme activities by spectrophotometric studies. Any mode of inheritance can be observed: sporadic, autosomal dominant or recessive, or maternal inheritance.

  18. GATA3: a multispecific but potentially useful marker in surgical pathology: a systematic analysis of 2500 epithelial and nonepithelial tumors.

    Science.gov (United States)

    Miettinen, Markku; McCue, Peter A; Sarlomo-Rikala, Maarit; Rys, Janusz; Czapiewski, Piotr; Wazny, Krzysztof; Langfort, Renata; Waloszczyk, Piotr; Biernat, Wojciech; Lasota, Jerzy; Wang, Zengfeng

    2014-01-01

    neuroendocrine tumors. Mesenchymal tumors were only sporadically positive, except epithelia of biphasic synovial sarcomas. GATA3 is a useful marker in the characterization of not only mammary and urothelial but also renal and germ cell tumors, mesotheliomas, and paragangliomas. The multiple specificities of GATA3 should be taken into account when using this marker to detect metastatic mammary or urothelial carcinomas.

  19. Methylation of the estrogen receptor CpG island distinguishes spontaneous and plutonium-induced tumors from nitrosamine-induced lung tumors

    Energy Technology Data Exchange (ETDEWEB)

    Belinsky, S.A.; Baylin, S.B.; Issa, J.J. [Johns Hopkins Univ., Baltimore, MD (United States)

    1995-12-01

    CpG islands located in the promoter region of genes constitute one mechanism for regulating transcription. These islands are normally free of methylation, regardless of the expression state of the gene. Hypermethylation of CpG islands, the addition of a methyl group to the internal cytosine within CpG dinucleotides, can cause silencing of a gene. Hypermethylation has been detected as an early event at specific chromosome loci during the development of colon cancer and represents one mechanism used by neoplatic cells to inactivate tumor suppressor genes. Recent studies have demonstrated this mechanism in inactivation of the VHL tumor suppressor gene in 19% of sporadic renal tumors and the p16 {sup INK4a} tumor suppressor gene in 30% of non-small cell lung cancers. A recent report indicates that the estrogen receptor gene could also be inactivated through methylation. In addition, estrogen receptor CpG island methylation arises as a direct function of age in normal colonic mucosa and is present in virtually all colonic tumors. In cultured colon cancer cells, methylation-associated loss of expression of the estrogen receptor gene results in deregulated growth, suggesting a role for the estrogen receptor in colon cancer development. These results provide further evidence that gene silencing through methylation could be a predominant epigenetic mechanism underlying the development of many different types of cancer. The purpose of the current investigation was to determine whether estrogen receptor CpG island methylation is involved in the development of lung cancer. The frequency for methylation of the estrogen receptor CpG island in rodent lung tumors is summarized.

  20. Methylation of the estrogen receptor CpG island distinguishes spontaneous and plutonium-induced tumors from nitrosamine-induced lung tumors

    International Nuclear Information System (INIS)

    Belinsky, S.A.; Baylin, S.B.; Issa, J.J.

    1995-01-01

    CpG islands located in the promoter region of genes constitute one mechanism for regulating transcription. These islands are normally free of methylation, regardless of the expression state of the gene. Hypermethylation of CpG islands, the addition of a methyl group to the internal cytosine within CpG dinucleotides, can cause silencing of a gene. Hypermethylation has been detected as an early event at specific chromosome loci during the development of colon cancer and represents one mechanism used by neoplatic cells to inactivate tumor suppressor genes. Recent studies have demonstrated this mechanism in inactivation of the VHL tumor suppressor gene in 19% of sporadic renal tumors and the p16 INK4a tumor suppressor gene in 30% of non-small cell lung cancers. A recent report indicates that the estrogen receptor gene could also be inactivated through methylation. In addition, estrogen receptor CpG island methylation arises as a direct function of age in normal colonic mucosa and is present in virtually all colonic tumors. In cultured colon cancer cells, methylation-associated loss of expression of the estrogen receptor gene results in deregulated growth, suggesting a role for the estrogen receptor in colon cancer development. These results provide further evidence that gene silencing through methylation could be a predominant epigenetic mechanism underlying the development of many different types of cancer. The purpose of the current investigation was to determine whether estrogen receptor CpG island methylation is involved in the development of lung cancer. The frequency for methylation of the estrogen receptor CpG island in rodent lung tumors is summarized

  1. Sporadic Hirschsprung`s disease due to a novel nonsense mutation in the RET protooncogene

    Energy Technology Data Exchange (ETDEWEB)

    Carlson, K.M.; Donis-Keller, H.; Langer, J.C. [and others

    1994-09-01

    Hirschsprung`s disease (HSCR, aganglionic megacolon) is characterized by a lack of ganglion cells along variable lengths of the hindgut. This is most likely due to a failure of the progenitor cells (that are destined to become the ganglion cells of the submucosal and myenteric plexuses) to complete their distal migration in the colon. Recently, mutations in the RET protoocogene have been reported in association with HSCR. We report a novel nonsense mutation resulting in a severely truncated protein. Germline DNA from a panel of 6 HSCR patients was analyzed by SSCP for 20 exons of RET. Eight exons were also directly sequenced. We identified a novel mutation within RET exon 2. The mutation (TAC{sub 36}{yields}TAG{sub 36}), which occurs at nucleotide position 108, involves the replacement of tyrosine with a stop codon and results in a truncated 35 amino acid protein. This mutation is the most 5{prime} nonsense mutation reported thus far. Interestingly, the patient has no prior family history of HSCR and was also diagnosed with multiple developmental anomalies including dysplastic kidney. Recent gene targeting studies with mouse models have shown that RET is essential for normal renal development. However, a parallel phenotype has not been seen in other reported HSCR patients with RET mutations. The observations reported here provide evidence that RET plays a role in human renal development. Ongoing studies will determine the extent of RET involvement in sporadic cases of HSCR.

  2. Similar phenotype characteristics comparing familial and sporadic premature ovarian failure.

    Science.gov (United States)

    Janse, Femi; Knauff, Erik A H; Niermeijer, Martinus F; Eijkemans, Marinus J; Laven, Joop S E; Lambalk, Cornelius B; Fauser, Bart C J M; Goverde, Angelique J

    2010-07-01

    Premature ovarian failure (POF) is characterized by secondary amenorrhea before the age of 40 years, along with repeated increased follicle-stimulating hormone and low estrogen concentrations. POF is considered a complex genetic disease with a familial presentation in 12% to 50% of cases. POF may originate from different genes and various gene-environment interactions. The aim of this study was to identify possible differences in phenotype comparing women with familial and women with sporadic POF. A multicenter study was initiated in the Netherlands using standardized phenotyping. For each woman, medical history, menstrual cycle, and fertility and smoking status were assessed and a standardized examination was performed. Based on a detailed three-generation family history, women were identified as having either familial (defined as having at least one relative with POF) or sporadic POF. A total of 58 familial cases and 142 sporadic cases of POF were identified. Maternal age at menopause was significantly lower in the women with familial compared with the women with sporadic POF (41.0 +/- 7.5 and 49.7 +/- 2.6 y, respectively; P hormone-binding globulin concentration was significantly higher in the women with familial than in the women with sporadic POF (73.6 +/- 37.1 and 55.2 +/- 26.9 nmol/L, respectively; P = 0.002). All other characteristics, such as parity, bone mineral density, and serum follicle-stimulating hormone and lipid levels were similar, as was the incidence of autoimmunity and cytogenetic abnormalities. Familial and sporadic POF do not differ in phenotype except for maternal menopause age and sex hormone-binding globulin concentration. Future studies are needed to unravel the genotype-phenotype interactions in POF.

  3. Tumor lysis syndrome in children

    International Nuclear Information System (INIS)

    Suarez, Amaranto

    2004-01-01

    Tumor lysis syndrome is a metabolic emergency characterized by electrolyte alteration with or without acute renal failure. It occurs mainly in patients with malignant tumors that have a high growth fraction, or after cytotoxic therapy, as a result of the massive degradation of malignant cells and the release of high amounts of intracellular elements that exceed the capacity of renal excretion. The objective of the treatment is the prevention of nephropathy due to uric acid deposits, and the correction of metabolic acidosis and electrolyte alterations. This paper reviews the incidence, the physiopathology, and the treatment of tumor lysis syndrome in children

  4. Prognostic impact of changes in base excision repair machinery in sporadic colorectal cancer.

    Science.gov (United States)

    Azambuja, Daniel B; Leguisamo, Natalia M; Gloria, Helena C; Kalil, Antonio Nocchi; Rhoden, Ernani; Saffi, Jenifer

    2018-01-01

    to evaluate the prognostic value of base excision repair proteins in sporadic colorectal cancer. Pre-treatment tumor samples from 72 patients with sporadic colorectal adenocarcinoma were assessed for APC, MPG, Polβ, XRCC1 and Fen1 expression by immunohistochemistry. The associations of molecular data were analyzed in relation to clinical features and TNM staging as a prognosis predictor and disease-free survival. Higher levels of MPG, Polβ and XRCC1, but not Fen1, were associated with unfavorable pathological outcomes, such as poor cellular differentiation, advanced TNM stages, presence of lymphatic and perineural invasions and metastatic lymph nodes. MPG and Polβ overexpression were associated with right-sided CRC. However, only MPG high expression is associated with shorter disease-free survival in CRC patients. Our results suggest that increased expression of MPG, Polβ and XRCC1 are more likely to evolve to poor pathological outcomes, but only the elevated expression of MPG protein predicts recurrence. The BER proteins appear to be suitable candidates to refine the TNM current staging of colorectal cancer. Copyright © 2017 Elsevier GmbH. All rights reserved.

  5. Sporadic potassium layers and their connection to sporadic E layers in the mesopause region at Beijing, China

    Directory of Open Access Journals (Sweden)

    Jing Jiao

    2017-06-01

    Full Text Available A double-laser beam lidar to measure potassium (K layer at Beijing (40.5° N, 116.2° E was successfully developed in 2010. The parameters of sporadic Ks layers and their distributions were given. The seasonal distribution of Ks occurrence frequency was obtained, with two maxima in July and January. The seasonal distributions of sporadic Es layer occurrence frequency over Beijing differ from those of Ks. However, the good correlation between Es and Ks in the case-by-case studies supports the mechanism of neutralization of metal ions in a descending Es layer.

  6. A recessive syndrome of intellectual disability, moderate overgrowth, and renal dysplasia predisposing to Wilms tumor is caused by a mutation in FIBP gene.

    Science.gov (United States)

    Akawi, Nadia; Ben-Salem, Salma; Lahti, Laura; Partanen, Juha; Ali, Bassam R; Al-Gazali, Lihadh

    2016-08-01

    Clinical classification of overgrowth syndromes represents a challenge since a wide spectrum of disorders result in marked overgrowth. Therefore, there is a continuous effort to identify the genetic basis of these disorders that will eventually facilitate their molecular classification. Here, we have identified the genetic etiology and the pathogenetic mechanism underlying a rare autosomal recessive overgrowth syndrome in three affected siblings. The overgrowth phenotype in the patients was accompanied by developmental delay, learning disabilities, and variable congenital abnormalities. To elucidate the genetic etiology of the disorder, whole-genome genotyping and whole-exome sequencing were used. The disease was mapped to 3p21.1-p14.2 and 11q13.1-q13.4, where an in-frame insertion (c.175_176insTAA) in FIBP gene was revealed. The resulting indel (p.H59LN) was predicted to change the protein conformation with likely deleterious effect on its function as one of the fibroblast growth factor signaling mediators. In vitro cellular proliferation assay and in situ hypridization in vivo were then performed to understand the pathophysiology of the disease. The patients' skin fibroblasts showed an increased proliferation capacity compared to the controls' explaining the observed overgrowth phenotype. In addition, we detected Fibp expression most notably in the brains of mice embryos suggesting a possible effect on cognitive functions early in development. To date, only one patient has been reported with a homozygous nonsense mutation in FIBP exhibiting an overgrowth syndrome with multiple congenital abnormalities. Taken all together, these findings provide convincing evidence implicating FIBP aberrations in the newly recognized overgrowth syndrome and expand the associated phenotypes to include possible Wilms tumor predisposition. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  7. Brain sonography in African infants with complicated sporadic ...

    African Journals Online (AJOL)

    Background: To determine the structural findings in brain sonography of African infants with complicated sporadic bacterial meningitis. Materials and Methods: Retrospective assessment of medical records of patients who underwent brain sonography on account of complicated bacterial meningitis. The brain sonography ...

  8. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease

    NARCIS (Netherlands)

    I. Zerr; K. Kallenberg; D.M. Summers; C. Romero; A. Taratuto; U. Heinemann; M. Breithaupt; D. Varges; B. Meissner; A. Ladogana (Anna); M. Schuur (Maaike); S. Haik; S.J. Collins (Steven); G.H. Jansen (Gerard); G.B. Stokin; J. Pimentel; E. Hewer; D. Collie; P. Smith; H. Roberts; J.P. Brandel; P. Tikka-Kleemola (Päivi); M. Pocchiari (Maurizio); C. Begue; P. Cras (Patrick); R.G. Will; P. Sanchez-Juan (Pascual)

    2009-01-01

    textabstractSeveral molecular subtypes of sporadic Creutzfeldt-Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications

  9. Comparing Sporadic and Outbreak-associated Foodborne Illness

    Centers for Disease Control (CDC) Podcasts

    2016-11-04

    Dr. Eric Ebel, a veterinarian and risk analyst with USDA’s Food Safety and Inspection Service, discusses his article on sporadic and outbreak-associated cases of foodborne illness.  Created: 11/4/2016 by National Center for Emerging and Zoonotic Infectious Diseases (NCEZID).   Date Released: 11/4/2016.

  10. Prevalence of Abnormal Cervical Smears from Sporadic Screening ...

    African Journals Online (AJOL)

    The aim of the study was to find the prevalence of abnormal smears in an unscreened population of sexually active women attending a gynaecological clinic. “Pap” smears were taken sporadically for cytological examination from sexually active women attending gynaecological clinics at the Federal Medical Centre Gombe.

  11. Legionnaires’ Disease: Clinicoradiological Comparison of Sporadic Versus Outbreak Cases

    Directory of Open Access Journals (Sweden)

    Hafiz Rizwan Talib Hashmi

    2017-06-01

    Full Text Available Background: In 2015, New York City experienced the worst outbreak of Legionnaires’ disease in the history of the city. We compare patients seen during the 2015 outbreak with sporadic cases of Legionella during the past 5 years. Methods: We conducted a retrospective chart review of 90 patients with Legionnaires’ disease, including sporadic cases of Legionella infection admitted from 2010 to 2015 (n = 55 and cases admitted during the 2015 outbreak (n = 35. Results: We saw no significant differences between the 2 groups regarding demographics, smoking habits, alcohol intake, underlying medical disease, or residence type. Univariate and multivariate analyses showed that patients with sporadic case of Legionella had a longer stay in the hospital and intensive care unit as well as an increased stay in mechanical ventilation. Short-term mortality, discharge disposition, and most clinical parameters did not differ significantly between the 2 groups. Conclusions: We found no specific clinicoradiological characteristics that could differentiate sporadic from epidemic cases of Legionella . Early recognition and high suspicion for Legionnaires’ disease are critical to provide appropriate treatment. Cluster of cases should increase suspicion for an outbreak.

  12. Dyschromatosis symmetrica hereditaria: Report of a sporadic case ...

    African Journals Online (AJOL)

    ... hands and feet, with no family history of similar lesions. The diagnosis was confirmed by the typical histologic finding of basal hyperpigmentation with normal number of melanocytes and absence of melanin incontinence from a hyperpigmented lesion. Keywords: DyschromatosisSymmetricaHereditaria, Nigerian, Sporadic ...

  13. Interpretation of electrodiagnostic findings in sporadic progressive muscular atrophy

    NARCIS (Netherlands)

    Visser, J.; de Visser, M.; van den Berg-Vos, R. M.; van den Berg, L. H.; Wokke, J. H. J.; de Jong, J. M. B. V.; Franssen, H.

    2008-01-01

    Objective We present the electrophysiologic data at baseline of 37 patients who were included in our prospective study on sporadic adult-onset progressive muscular atrophy (PMA). The aim was to correlate electrophysiological. signs of lower motor neuron (LMN) loss with clinical signs of LMN loss,

  14. [The practice guideline 'Dermatomyositis, polymyositis and sporadic inclusion body myositis'

    NARCIS (Netherlands)

    Hoogendijk, J.E.; Bijlsma, J.W.J.; Engelen, B.G.M. van; Lindeman, E.J.M.; Royen-Kerkhof, A. van; Rie, M.A. de; Visser, M. de; Jennekens, F.G.I.

    2005-01-01

    This guideline presents recommendations for the diagnosis and treatment of dermatomyositis, polymyositis and sporadic inclusion body myositis (sIBM) according to the best available evidence. Characteristic skin abnormalities can be sufficient for the diagnosis of dermatomyositis. In case of doubt, a

  15. Renal radiopharmaceuticals

    International Nuclear Information System (INIS)

    Moretti, J.L.; Prevot, M.; Beco, V. de

    1995-01-01

    Renal tracers are classified according to their routes of excretion. Glomerular tracers most in use are 51 Cr EDTA and 99m Tc DTPA, the latter giving glomerular filtration values for each kidney with the help of scintigraphic imaging. Tubular tracers are a changing matter, 99m Tc MAG3 and 99m Tc EC would take the place of 123 I hippuran. Since 99m Tc glucoheptonate is not specific of the glomerular or tubular function and is a poor static imaging tracer, 99m Tc DMSA is the agent of choice for measuring the split functional renal mass. (authors). 16 refs., 5 figs

  16. [Kidney function and renal cancer surgery].

    Science.gov (United States)

    Izzedine, Hassan; Méjean, Arnaud; Escudier, Bernard

    2014-02-01

    Although radical nephrectomy is still practiced in many patients with large renal tumors, oncology and nephrology arguments for kidney-sparing approach for small renal masses has taken over this first. Indeed, partial nephrectomy provides equivalent oncologic results while preserving renal function and thereby limit morbidity and cardiovascular mortality related to chronic kidney disease. In addition, patients who develop kidney cancer often have medical comorbidities that may affect renal function, such as diabetes and hypertension. Histological examination of renal tissue adjacent to the tumor showed significant pathological changes in the majority of patients. For elderly patients or patients with comorbidities, active surveillance allows kidney-sparing approach with extremely low rates of progression and metastasis of cancer disease. Despite these significant advances in understanding for the treatment of small renal masses, partial nephrectomy remains underused. Better management must take into account the preservation of renal function in order to increase overall survival. A strategy for the systematic evaluation of renal function in patients with CR, with multidisciplinary staff (nephrologist urologist and oncologist), is therefore highly desirable.

  17. Long-term preclinical magnetic resonance imaging alterations in sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Zanusso, Gianluigi; Camporese, Giulia; Ferrari, Sergio; Santelli, Luca; Bongianni, Matilde; Fiorini, Michele; Monaco, Salvatore; Manara, Renzo; Cagnin, Annachiara

    2016-10-01

    An asymptomatic 74-year-old woman, on follow-up for a carotid body tumor, showed magnetic resonance imaging (MRI) focal restricted diffusion confined to the left temporal and occipital cortices. Thirteen months later, diffusion-weighted images revealed a bilateral cortical ribbon sign involving all lobes. After 1 month, the patient developed gait instability and cognitive decline rapidly evolving to severe dementia and death within 3 months. Prion protein gene sequence, molecular, and neuropathological studies confirmed the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 subtype. Here we show the kinetics of MRI changes and prion spreading in preclinical sCJD MM1. Ann Neurol 2016;80:629-632. © 2016 American Neurological Association.

  18. Primary sporadic Burkitt lymphoma of the orbit, clinical characteristics, management, and outcomes: a case study.

    Science.gov (United States)

    Bouali, Sofiene; Said, Imed Ben; Yedeas, Mohamed Dahmani; Abderrahmen, Khansa; Maatar, Nidhal; Boubaker, Adnen; Kallel, Jalel; Jemel, Hafedh

    2016-03-01

    Involvement of the orbit with Burkitt's lymphoma is a very rare presentation of extra-nodal lymphoma. We report a case of a 2-year-old female presented an unusual location of sporadic Burkitt's lymphoma arising in the orbital region. Diagnostic magnetic resonance imagining identified an oval-shaped mass on the lateral rectus of the right orbit that caused dislocation of eyeball, for which she underwent a biopsy from the periorbital swellings. The mass was histologically confirmed as Burkitt's lymphoma, and postoperative aggressive chemotherapy was initiated. We describe clinical diagnosis, histological aspects, radiological features, and current management of this rapidly growing malignant tumor. Because of the rapid progression of Burkitt lymphoma, and considering that it responds well to treatment, early recognition and appropriate management are important factors for survival and to preserve visual function.

  19. Primary renal neoplasia of dogs.

    Science.gov (United States)

    Bryan, Jeffrey N; Henry, Carolyn J; Turnquist, Susan E; Tyler, Jeff W; Liptak, Julius M; Rizzo, Scott A; Sfiligoi, Gabriella; Steinberg, Steven J; Smith, Annette N; Jackson, Tarraca

    2006-01-01

    Primary renal tumors are diagnosed uncommonly in dogs. Signs and survival will differ among different categories of primary renal tumors. Data were collected from the medical records of 82 dogs with primary renal tumors diagnosed by examination of tissue obtained by ultrasound-guided biopsy, needle aspiration, surgery, or at postmortem examination. This was a multi-institutional, retrospective study. Forty-nine dogs had carcinomas, 28 had sarcomas, and 5 had nephroblastomas. The dogs were geriatric (mean 8.1 years; range: 1-17) with a weight of 24.9 kg (range: 4.5-80). Tumors occurred with equal frequency in each kidney with 4% occurring bilaterally. Initial signs included one or more of hematuria, inappetance, lethargy. weight loss, or a palpable abdominal mass. Pain was reported more frequently in dogs with sarcomas (5/28). The most common hematologic abnormalities were neutrophilia (22/63), anemia (21/64), and thrombocytopenia (6/68). Polycythemia was present in 3 dogs and resolved with treatment. Hematuria (28/49), pyuria (26/49), proteinuria (24/50), and isosthenuria (20/56) were the most frequently observed abnormalities on urinalysis. Pulmonary metastases were noted on thoracic radiographs in 16% of dogs at diagnosis. Seventy-seven percent of dogs had metastatic disease at the time of death. Median survival for dogs with carcinomas was 16 months (range 0-59 months), for dogs with sarcomas 9 months (range 0-70 months), and for dogs with nephroblastomas 6 months (range 0-6 months). Primary renal tumors in dogs are generally highly malignant with surgery being the only treatment that improves survival.

  20. Arterial spin labelling in imaging of renal diseases and renal allograft pathology; MRT-Perfusionsmessung mit Arterial Spin Labelling. Anwendung fuer die Niere und Transplantatniere

    Energy Technology Data Exchange (ETDEWEB)

    Hueper, Katja; Gutberlet, Marcel [Medizinische Hochschule Hannover (Germany). Inst. fuer Diagnostische und Interventionelle Radiologie; Kuehn, Bernd [Siemens AG/Siemens Healthcare GmbH, Erlangen (Germany)

    2016-06-15

    Arterial Spin Labelling (ASL) is a technique for non-invasive and contrast-free assessment of perfusion with MRI. Renal ASL allows examination of renal pathophysiology, evaluation of the course of renal disease and therapy effects by longitudinal measurements as well as characterization of renal tumors. In this article, techniques of ASL will be explained and challenges of renal ASL will be emphasized. In addition, examples for clinical application of ASL for diagnosis of renal disease and renal allograft pathology will be given.

  1. [Chromophobe renal cell carcinoma, an exceptional entity in childhood: a case report].

    Science.gov (United States)

    Fernández Pineda, I; Cabello Laureano, R; Maraví Petri, A; Carranza Carranza, A; Congregado Córdoba, J; De Agustín Asensio, J C

    2008-06-01

    Chromophobe renal cell carcinoma (CRCC) is a rare variant of renal carcinomas arising from the intercalated cells of the distal renal tubule and representing 5% among all renal tumors. Its biological behaviour is variable, less aggresive than clear cell renal carcinoma. Histochemical, ultrastructural and molecular genetic characteristics are different from other renal carcinomas. Age at presentation is about the 6th decade of life. We report an exceptional 10 year-old boy case with a CRCC. Diagnostic and therapeutic aspects for the management of this tumor are reviewed.

  2. Wilm's tumor in adulthood

    International Nuclear Information System (INIS)

    Matveev, B.P.; Bukharkin, B.V.; Gotsadze, D.T.

    1984-01-01

    Wilms' tumor occurs extremely rarely in adults. There is no consensus in the literature on the problems of clinical manifestations, diagnosis and treatment of the diseasa. Ten adult patients (aged 16-29) with Wilms' tumor formed the study group. They made up 0.9 per cent of the total number of kidney tumor patients. The peculiarities of the clinical course that distinguish adult nephroblastoma from renal cancer and Wilms' tumor of the infancy were analysed. The latent period appeared to be long. Problems of diagnosis are discussed. Angiography proved to be of the highest diagnostic value. Complex treatment including transperitoneal nephrectory, radiation and chemotherapy was carried out in 7 cases, palliative radiation treatmenchemotherapy andn 3. Unlike pediatric nephroblastomt - i Wilms' tumor in adults was resistant to radiation. Treatment results still remained unsatisfactory: 6 patients died 7-19 months after the beginning of treatment

  3. Summary and consensus in 7th International Conference on acoustic neuroma: An update for the management of sporadic acoustic neuromas

    Directory of Open Access Journals (Sweden)

    Hao Wu

    2016-12-01

    Full Text Available Sporadic vestibular schwannoma (acoustic neuroma is a benign tumor arising from cochleovestibular nerve. Nowadays, various specialties and medical centers are treating this disease, and the multidisciplinary collaboration is the trend. In an effort to promote a uniform standard for reporting clinical results, even for treatment indications, the mainly controversies were posed and discussed during the 7th International Conference on acoustic neuroma, and the agreement was summarized by the Committee of this conference. The main symptoms grading and tumor stage should note its name of classification for making them comparable. The goal of the modern managements for vestibular schwannoma is to improve the quality of life with lower mortality, lower morbidity and better neurological function preservation. The experience of surgical team and their preference might be a major factor for the outcome. Because of lacking of long-term follow-up large data after radiotherapy, and with the development of microsurgery, radiotherapy is now less recommended except for recurrent cases or elderly patients. Keywords: Sporadic acoustic neuroma, Vestibular schwannoma, Management, Symptoms grading, Tumor stage, Microsurgery, Radiotherapy

  4. Renal denervation

    DEFF Research Database (Denmark)

    Olsen, Lene Kjær; Kamper, Anne-Lise; Svendsen, Jesper Hastrup

    2015-01-01

    PURPOSE OF REVIEW: Renal denervation (RDN) has, within recent years, been suggested as a novel treatment option for patients with resistant hypertension. This review summarizes the current knowledge on this procedure as well as limitations and questions that remain to be answered. RECENT FINDINGS...

  5. Estudo comparativo dos métodos de ultra-sonografia, tomografia computadorizada e ressonância magnética no estadiamento e invasão das estruturas adjacentes por tumores renais A comparative study of ultrasonography, computed tomography and magnetic resonance imaging in the staging and invasion of adjacent structures by renal tumors

    Directory of Open Access Journals (Sweden)

    S.M. RIBEIRO

    2001-09-01

    para o diagnóstico como no estadiamento, devem ser considerados aspectos relativos à invasibilidade, riscos e preço na ponderação dos custos e benefícios dos diversos exames de diagnóstico por imagem.BACKGROUND: Ultrasonography (US, Computed Tomography (CT, and Magnetic Resonance imaging (MR were compared for the staging of renal tumors. The differences between these imaging techniques were also studied for their ability to detect adenopathies, vascular invasion, distant intra-abdominal metastases, and particularly adjacent organ invasion. METHODS: Thirty-one patients with solid or complex renal masses were prospectively studied using US, CT, and MR. Differences between the results obtained were studied using the COCHRAN G test and the Mc NEMAR test. The sensitivity and specificity of each diagnostic technique were compared against a "gold standard" of the surgical and histopathological findings. RESULTS: The following sensitivities were obtained: For the detection of adenopathy, US 63.6%, CT and MR 90.9%. For vascular invasion, US 42.8%, CT and MR 85.7%. For the adjacent organ invasion, US 28.5%, CT 85.7%, and MR 71.4%. Some of the criteria that suggest invasion of adjacent structures include: the envelopment of the adjacent structures by the tumor, tumor extension into the adjacent structures with an irregular appearance, and alterations in shape, size, and density of adjacent structures. Loss of fat planes between the tumor and adjacent structures is not a sign of tumor invasion. CONCLUSIONS: Significant differences were found in the detection capacity of US in relation to CT and MR, which were similar. All three techniques were highly sensitive and specific only in the detection of distant abdominal metastases. In addition to the accuracy of these diagnostic modalities for the detection and staging of tumors, invasiveness, risks and cost should be considered in relation to relative costs and benefits.

  6. Lympho-vascular invasion in BRCA related breast cancer compared to sporadic controls

    Directory of Open Access Journals (Sweden)

    van der Wall Elsken

    2010-04-01

    Full Text Available Abstract Background Germline mutations in the BRCA1 gene predispose to the development of breast cancer, exhibiting a specific histological phenotype. Identification of possible hallmarks of these tumors is important for selecting patients for genetic screening and provides inside in carcinogenetic pathways. Since BRCA1-associated breast cancers have pushing borders that prevent them from easily reaching vessels and are often of the medullary (like type that is known to have a low rate of lympho-vascular invasion (LVI, we hypothesized that absence of LVI could characterize BRCA1 related breast cancer. Methods A population of 68 BRCA1 related invasive breast cancers was evaluated for LVI by an experienced breast pathologist blinded to mutation status, and compared to a control group matched for age, grade and tumor type. Results LVI was present in 25.0% of BRCA1 related cases, compared to 20.6% of controls (P = 0.54, OR = 1.29, CI 0.58-2.78. Conclusion LVI is frequent in BRCA1 germline mutation related breast cancers, but seems to occur as often in sporadic controls matched for age, grade and tumor type. Apparently, these hereditary cancers find their way to the blood and lymph vessels despite their well demarcation and often medullary differentiation.

  7. Clinical and Genetic Aspects of Sporadic Non-Medullar Thyroid Cancer

    Directory of Open Access Journals (Sweden)

    U Rumjanzeva

    2006-03-01

    Full Text Available The role of somatic mutations in sporadic thyroid cancer is unclear today. Probably they coming out as aetiological factors in carcinogenesis as well as, respectfully to many authors, can to participate in TC pathogenesis and to determine the clinical course and prognosis of the disease. For today as main oncogenes taking part in initiation of thyroid malignant tumors are considered: RET/PTC, TRK, PTEN, P53, RAS, MET, PPARγ. By means of genetic investigations scientists are trying to solve problems with thyroid cancer differentiated diagnostics (cytokeratin-19, cytokeratin-20, mesothelial cells antigen (Hector Battifora MEsotelial (cell or HBME-1, loss of heterozigitoty (LOH in short arm of 3 chromosome (gene VHL -von Hippel Lindau, 3р26. Recently in foreign literature appeared reports of activated mutations in gene BRAF which most frequently are occurred in melanoma and papillary TC. Prognosis of thyroid cancer may reflected by the LOH as a biological breakage as well as changes of tumor suppressive gene P53 which fraught with decrease of disease prognosis. Thus, both researchers and clinicians have many questions concerning the role of genome, particularly in order to precise of genetic abnormality influence on tumor growth and therefore for assessment of clinical prognosis and with aim to chose adequate treatment tactic in each case.

  8. The storage period of the formalin-fixed paraffin-embedded tumor blocks does not influence the concentration and purity of the isolated DNA in a series of 83 renal and thyroid carcinomas.

    Science.gov (United States)

    Nechifor-Boilă, Adela Corina; Loghin, Andrada; Vacariu, Victor; Halaţiu, Vasile Bogdan; Borda, Angela

    2015-01-01

    Optimal recovery of nucleic acids from formalin-fixed paraffin-embedded (FFPE) tissues is highly dependent on a series of pre-extraction steps, mainly related (but not limited) to fixation. The aim of our study was to investigate if the storage period of the FFPE blocks had a significant effect on the isolated DNA. We examined the quantity and purity of the isolated DNA from 83 FFPE blocks, corresponding to malignant thyroid (n=28) and renal (n=55) carcinomas that had been stored in our department for up to eight years. The DNA extraction protocol was based on a precipitation method (MasterPure™ DNA Purification Kit, Epicentre), in accordance to the manufacturer instructions, optimized in our laboratory. A spectrophotometer was used to determine the yield (A260) and purity (A260/A280 ratio) of the isolated DNA. We successfully isolated good DNA quantity and purity from all our study cases (mean concentration: 223.4 ± 104.16 ng/μL; mean A260/A280 ratio: 1.68 ± 0.09). Moreover, no statistically significant differences were observed between tumor blocks stored for 2-3 years and 7-8 years, respectively, both in terms of DNA quantity (p=0.196) and purity (p=0.663). In conclusion, we successfully validated an efficient, reproducible DNA extraction technique that provided a good range of DNA concentrations and purity, regardless the type of tissue (thyroid or kidney). Moreover, we demonstrated that the storage period of the FFPE blocks does not have a significant influence on the DNA quantity and purity.

  9. Renal Myxoma, an Incidental Finding

    Directory of Open Access Journals (Sweden)

    Parth Thakker

    2017-07-01

    Full Text Available Myxomas are mesenchymal tumors commonly found in the heart and skin. Renal myxomas are rare, having only been documented 14 times. Our case is a 55-year-old woman who presented to our clinic after a right renal mass was incidentally found on CT. Evaluation with MRI showed a mass that appeared to arise from the supero-medial cortex of the right kidney. As the imaging was concerning for renal cell carcinoma, the patient underwent a partial nephrectomy. Microscopic examination showed a well-circumscribed mass with polygonal to spindle-shaped cells in a granular eosinophilic cytoplasm. Immunohistochemical staining for CD-10, Desmin, HMB-45, and Pankeratin were negative.

  10. AIP mutations in Brazilian patients with sporadic pituitary adenomas: a single-center evaluation

    Directory of Open Access Journals (Sweden)

    Paula Bruna Araujo

    2017-11-01

    Full Text Available Aryl hydrocarbon receptor-interacting protein (AIP gene mutations (AIPmut are the most frequent germline mutations found in apparently sporadic pituitary adenomas (SPA. Our aim was to evaluate the frequency of AIPmut among young Brazilian patients with SPA. We performed an observational cohort study between 2013 and 2016 in a single referral center. AIPmut screening was carried out in 132 SPA patients with macroadenomas diagnosed up to 40 years or in adenomas of any size diagnosed until 18 years of age. Twelve tumor samples were also analyzed. Leukocyte DNA and tumor tissue DNA were sequenced for the entire AIP-coding region for evaluation of mutations. Eleven (8.3% of the 132 patients had AIPmut, comprising 9/74 (12% somatotropinomas, 1/38 (2.6% prolactinoma, 1/10 (10% corticotropinoma and no non-functioning adenomas. In pediatric patients (≤18 years, AIPmut frequency was 13.3% (2/15. Out of the 5 patients with gigantism, two had AIPmut, both truncating mutations. The Y268* mutation was described in Brazilian patients and the K273Rfs*30 mutation is a novel mutation in our patient. No somatic AIP mutations were found in the 12 tumor samples. A tumor sample from an acromegaly patient harboring the A299V AIPmut showed loss of heterozygosity. In conclusion, AIPmut frequency in SPA Brazilian patients is similar to other populations. Our study identified two mutations exclusively found in Brazilians and also shows, for the first time, loss of heterozygosity in tumor DNA from an acromegaly patient harboring the A299V AIPmut. Our findings corroborate previous observations that AIPmut screening should be performed in young patients with SPA.

  11. Everolimus and Vatalanib in Treating Patients With Advanced Solid Tumors

    Science.gov (United States)

    2018-01-12

    Gastrinoma; Glucagonoma; Insulinoma; Metastatic Gastrointestinal Carcinoid Tumor; Metastatic Pheochromocytoma; Pancreatic Polypeptide Tumor; Recurrent Gastrointestinal Carcinoid Tumor; Recurrent Islet Cell Carcinoma; Recurrent Melanoma; Recurrent Neuroendocrine Carcinoma of the Skin; Recurrent Non-small Cell Lung Cancer; Recurrent Pheochromocytoma; Recurrent Renal Cell Cancer; Somatostatinoma; Stage III Neuroendocrine Carcinoma of the Skin; Stage IV Melanoma; Stage IV Non-small Cell Lung Cancer; Stage IV Renal Cell Cancer; Thyroid Gland Medullary Carcinoma; Unspecified Adult Solid Tumor, Protocol Specific

  12. Carcinoma of the renal pelvis and ureter

    Directory of Open Access Journals (Sweden)

    Fernando Korkes

    2006-12-01

    Full Text Available OBJECTIVE: To assess the occurrence of upper urinary tract urothelial tumors (UUTT in Brazil. MATERIALS AND METHODS: We performed a clinical and histopathologic study of 33 patients who were diagnosed with a malignant neoplasm in the renal pelvis or ureter in the period of 1994 to 2004, in a single institution. RESULTS: Among the patients with upper urinary tract carcinoma, 70% were males and 30% females, with mean age of 65 ± 16 years (ranging from 31 to 91 years. Nineteen patients presented renal pelvis tumor (58%, 9 ureteral tumor (27% and 5 synchronic pelvic and ureteral tumors (15%. Renal pelvis tumors represented 2.8% of all the urothelial neoplasms, and 11.4% of all renal neoplasms treated in the same period. Ureteral tumors represented 1.6% of all the urothelial malignancies surgically managed in these 11 years. Tobacco smoking was the most common risk factor, and analgesic abuse was not reported by those patients. Most carcinomas were high-grade and muscle-invasive. Mean time to diagnosis was 7 months, being hematuria the most common symptom. CONCLUSIONS: A high association was also found between UUTT and bladder urothelial carcinoma. UUTT were mostly seen in men in their seventies and related to a high overall and cancer-related mortality rate. The overall disease-specific survival was 40%, much lower than found in most of the reported series.

  13. Transcriptional targeting of primary and metastatic tumor neovasculature by an adenoviral type 5 roundabout4 vector in mice.

    Directory of Open Access Journals (Sweden)

    Zhi Hong Lu

    Full Text Available New approaches targeting metastatic neovasculature are needed. Payload capacity, cellular transduction efficiency, and first-pass cellular uptake following systemic vector administration, motivates persistent interest in tumor vascular endothelial cell (EC adenoviral (Ad vector targeting. While EC transductional and transcriptional targeting has been accomplished, vector administration approaches of limited clinical utility, lack of tumor-wide EC expression quantification, and failure to address avid liver sequestration, challenged prior work. Here, we intravenously injected an Ad vector containing 3 kb of the human roundabout4 (ROBO4 enhancer/promoter transcriptionally regulating an enhanced green fluorescent protein (EGFP reporter into immunodeficient mice bearing 786-O renal cell carcinoma subcutaneous (SC xenografts and kidney orthotopic (KO tumors. Initial experiments performed in human coxsackie virus and adenovirus receptor (hCAR transgenic:Rag2 knockout mice revealed multiple ECs with high-level Ad5ROBO4-EGFP expression throughout KO and SC tumors. In contrast, Ad5CMV-EGFP was sporadically expressed in a few tumor vascular ECs and stromal cells. As the hCAR transgene also facilitated Ad5ROBO4 and control Ad5CMV vector EC expression in multiple host organs, follow-on experiments engaged warfarin-mediated liver vector detargeting in hCAR non-transgenic mice. Ad5ROBO4-mediated EC expression was undetectable in most host organs, while the frequencies of vector expressing intratumoral vessels and whole tumor EGFP protein levels remained elevated. In contrast, AdCMV vector expression was only detectable in one or two stromal cells throughout the whole tumor. The Ad5ROBO4 vector, in conjunction with liver detargeting, provides tractable genetic access for in-vivo EC genetic engineering in malignancies.

  14. White matter involvement in sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Caverzasi, Eduardo; Mandelli, Maria Luisa; DeArmond, Stephen J; Hess, Christopher P; Vitali, Paolo; Papinutto, Nico; Oehler, Abby; Miller, Bruce L; Lobach, Irina V; Bastianello, Stefano; Geschwind, Michael D; Henry, Roland G

    2014-12-01

    Sporadic Creutzfeldt-Jakob disease is considered primarily a disease of grey matter, although the extent of white matter involvement has not been well described. We used diffusion tensor imaging to study the white matter in sporadic Creutzfeldt-Jakob disease compared to healthy control subjects and to correlated magnetic resonance imaging findings with histopathology. Twenty-six patients with sporadic Creutzfeldt-Jakob disease and nine age- and gender-matched healthy control subjects underwent volumetric T1-weighted and diffusion tensor imaging. Six patients had post-mortem brain analysis available for assessment of neuropathological findings associated with prion disease. Parcellation of the subcortical white matter was performed on 3D T1-weighted volumes using Freesurfer. Diffusion tensor imaging maps were calculated and transformed to the 3D-T1 space; the average value for each diffusion metric was calculated in the total white matter and in regional volumes of interest. Tract-based spatial statistics analysis was also performed to investigate the deeper white matter tracts. There was a significant reduction of mean (P=0.002), axial (P=0.0003) and radial (P=0.0134) diffusivities in the total white matter in sporadic Creutzfeldt-Jakob disease. Mean diffusivity was significantly lower in most white matter volumes of interest (PCreutzfeldt-Jakob disease. Mean diffusivity reduction reflected concomitant decrease of both axial and radial diffusivity, without appreciable changes in white matter anisotropy. Tract-based spatial statistics analysis showed significant reductions of mean diffusivity within the white matter of patients with sporadic Creutzfeldt-Jakob disease, mainly in the left hemisphere, with a strong trend (P=0.06) towards reduced mean diffusivity in most of the white matter bilaterally. In contrast, by visual assessment there was no white matter abnormality either on T2-weighted or diffusion-weighted images. Widespread reduction in white matter mean

  15. Bone tumor

    Science.gov (United States)

    Tumor - bone; Bone cancer; Primary bone tumor; Secondary bone tumor; Bone tumor - benign ... The cause of bone tumors is unknown. They often occur in areas of the bone that grow rapidly. Possible causes include: Genetic defects ...

  16. Morphologic and molecular characteristics of uterine leiomyomas in hereditary leiomyomatosis and renal cancer (HLRCC) syndrome.

    Science.gov (United States)

    Sanz-Ortega, Julian; Vocke, Cathy; Stratton, Pamela; Linehan, William Marston; Merino, Maria J

    2013-01-01

    Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a hereditary cancer syndrome in which affected individuals are predisposed to the development of multiple leiomyomas of the skin and uterus and aggressive forms of kidney cancer. Affected individuals harbor a germline heterozygous loss-of-function mutation of the fumarate hydratase (FH) gene. Uterine leiomyomas are present in up to 77% of women with this syndrome. Previous studies have shown that inactivation of the FH gene is unusual for nonsyndromic leiomyomas. Therefore, it might be possible to distinguish 2 genetic groups of smooth muscle tumors: the most common group of sporadic uterine leiomyomas without FH gene inactivation and the more unusual group of HLRCC leiomyomas in patients who harbor a germline mutation of FH, although the exact prevalence of hereditary HLRCC is unknown. We reviewed the clinical, morphologic, and genotypic features of uterine leiomyomas in 19 HLRCC patients with FH germline mutations. Patients with HLRCC syndrome were younger in age compared with those with regular leiomyomata. DNA was extracted by microdissection, and analysis of loss of heterozygosity (LOH) at 1q43 was performed. Uterine leiomyomas in HLRCC have young age of onset and are multiple, with size ranging from 1 to 8 cm. Histopathologically, HLRCC leiomyomas frequently had increased cellularity, multinucleated cells, and atypia. All cases showed tumor nuclei with large orangeophilic nucleoli surrounded by a perinucleolar halo similar to the changes found in HLRCC. Occasional mitoses were found in 3 cases; however, the tumors did not fulfill the criteria for malignancy. Our study also showed that LOH at 1q43 was frequent in HLRCC leiomyomas (8/10 cases), similarly to what has been previously found in renal cell carcinomas from HLRCC patients. LOH is considered to be the second hit that inactivates the FH gene. We conclude that uterine leiomyomas associated with HLRCC syndrome have characteristic morphologic

  17. Comparison of transcriptomic signature of post-Chernobyl and postradiotherapy thyroid tumors.

    Science.gov (United States)

    Ory, Catherine; Ugolin, Nicolas; Hofman, Paul; Schlumberger, Martin; Likhtarev, Illya A; Chevillard, Sylvie

    2013-11-01

    We previously identified two highly discriminating and predictive radiation-induced transcriptomic signatures by comparing series of sporadic and postradiotherapy thyroid tumors (322-gene signature), and by reanalyzing a previously published data set of sporadic and post-Chernobyl thyroid tumors (106-gene signature). The aim of the present work was (i) to compare the two signatures in terms of gene expression deregulations and molecular features/pathways, and (ii) to test the capacity of the postradiotherapy signature in classifying the post-Chernobyl series of tumors and reciprocally of the post-Chernobyl signature in classifying the postradiotherapy-induced tumors. We now explored if postradiotherapy and post-Chernobyl papillary thyroid carcinomas (PTC) display common molecular features by comparing molecular pathways deregulated in the two tumor series, and tested the potential of gene subsets of the postradiotherapy signature to classify the post-Chernobyl series (14 sporadic and 12 post-Chernobyl PTC), and reciprocally of gene subsets of the post-Chernobyl signature to classify the postradiotherapy series (15 sporadic and 12 postradiotherapy PTC), by using conventional principal component analysis. We found that the five genes common to the two signatures classified the learning/training tumors (used to search these signatures) of both the postradiotherapy (seven PTC) and the post-Chernobyl (six PTC) thyroid tumor series as compared with the sporadic tumors (seven sporadic PTC in each series). Importantly, these five genes were also effective for classifying independent series of postradiotherapy (five PTC) and post-Chernobyl (six PTC) tumors compared to independent series of sporadic tumors (eight PTC and six PTC respectively; testing tumors). Moreover, part of each postradiotherapy (32 genes) and post-Chernobyl signature (16 genes) cross-classified the respective series of thyroid tumors. Finally, several molecular pathways deregulated in post

  18. A Rare Cause of Prepubertal Gynecomastia: Sertoli Cell Tumor

    Directory of Open Access Journals (Sweden)

    Fatma Dursun

    2015-01-01

    Full Text Available Prepubertal gynecomastia due to testis tumors is a very rare condition. Nearly 5% of the patients with testicular mass present with gynecomastia. Sertoli cell tumors are sporadic in 60% of the reported cases, while the remaining is a component of multiple neoplasia syndromes such as Peutz-Jeghers syndrome and Carney complex. We present a 4-year-old boy with gynecomastia due to Sertoli cell tumor with no evidence of Peutz-Jeghers syndrome or Carney complex.

  19. Severe acute tumor lysis syndrome in patients with germ-cell tumors

    Directory of Open Access Journals (Sweden)

    Guilherme Alvarenga Feres

    2008-01-01

    Full Text Available Germ-cell tumors are a high-proliferative type of cancer that may evolve to significant bulky disease. Tumor lysis syndrome is rarely reported in this setting. The reports of three patients with germ-cell tumors who developed severe acute tumor lysis syndrome following the start of their anticancer therapy are presented. All patients developed renal dysfunction and multiorgan failure. Patients with extensive germ-cell tumors should be kept on close clinical and laboratory monitoring. Physicians should be aware of this uncommon but severe complication and consider early admission to the intensive care unit for the institution of measures to prevent acute renal failure.

  20. Renal mucus gland cystadenomas in a horse.

    Science.gov (United States)

    Loynachan, Alan T; Bryant, Uneeda K; Williams, Neil M

    2008-07-01

    A 35-year-old horse was submitted to the necropsy service at the University of Kentucky Livestock Disease Diagnostic Center. At necropsy, multiple 1-4-cm-diameter cystic structures were incidentally identified unilaterally in the right renal medulla and the cortex. On histologic examination, the cystic structures compressed the normal renal architecture, were lined by tall columnar epithelium that formed occasional papillary projections, and contained large amounts of mucicarmine and periodic acid-Schiff-positive mucinous material. The masses were diagnosed as renal mucus-gland cystadenomas. This tumor should be considered as a differential diagnosis when cystic structures are identified in the equine kidney.

  1. Renal candidiasis

    International Nuclear Information System (INIS)

    Khanna, S.; Malik, N.; Khandelwal, N.

    1990-01-01

    Most fungal infections of the urinary tract are caused by Candida albicans, a yeast-like saprophytic fungus which may become apathogen under various conditions which lower the host resistance. The use of computed tomography in the diagnosis of renal fungus balls is the subject of this communication with emphasis on the radiologists role in the recognition of this entity. (H.W.). 6 refs.; 2 figs

  2. Hiperparatiroidismos renales

    Directory of Open Access Journals (Sweden)

    Valentín Malagón Castro

    1957-04-01

    Full Text Available En la presente monografía presentamos una síntesis, lo más completa posible, del gran problema de los Hiperparatirodismos secundarios a lesiones renales, enfocando su estudio con un criterio unicista, con el objeto de hacer más didáctico este amplio capítulo de la patología.

  3. The Role of Iron In Sporadic E Layers

    Science.gov (United States)

    Vondrak, T.; Woodcock, K. R. I.; Plane, J. M. C.

    Sporadic E layers in the lower thermosphere are mostly composed of metallic ions, of which Fe+ is the most abundant. Because dielectric recombination (Fe+ + elec- tron) is very slow, the lifetime of Fe+ above about 100 km is at least several days. However, below this height molecular ions such as FeO+, FeO2+ and FeN2+ form in- creasingly rapidly through reactions with O3, O2 and N2, respectively. These undergo rapid dissociative recombination with electrons, causing Fe+ to be neutralised increas- ingly rapidly as a sporadic E layer descends. Indeed, this is the most likely mechanism for the formation of the sporadic neutral Fe layers that are observed by lidar. However, atomic O plays a very important role in reducing these molecular ions back to Fe+, competing with dissociative recombination and thus slowing the rate at which Fe+ is neutralised and a sporadic E layer dissipates. This paper will discuss a laboratory and modelling study of the reactions of FeO+, FeO2+ and FeN2+ with atomic O. These reactions were studied (for the first time) in a fast flow tube, using the pulsed laser ablation of a rotating iron rod as the source of Fe+ ions in the upstream section of the tube. Reactants were then added to produce molecular ions, and atomic O further downstream through a movable injector. Fe+ and the molecular ions were detected at the downstream end of the tube using a two-stage quadrupole mass spectrometer. The spectroscopy of the FeO+ ion, observed by laser induced fluorescence, will also be discussed as a candidate for future ground-based lidar studies of the ion chemistry of the lower thermosphere.

  4. Measurements of the ionization heights of sporadic radio-meteors

    International Nuclear Information System (INIS)

    Baggaley, W.J.; Webb, T.H.

    1980-01-01

    The echo heights and echo point ionization densities of 4587 sporadic radio-meteors have been determined using a calibrated interferometric height-finding system. Over the height interval 92 to 96 km no association was found between height and ionization but, for radio-meteors ablating above and below this region, significant and opposite trends exist in the data. It is suggested that this could be evidence for the influx of two distinct meteoroid populations. (author)

  5. Ultrasonography of hydronephrosis and renal masses

    International Nuclear Information System (INIS)

    Lee, Kyung Weon; Kim, Chong Gun; Kim, Yeon Jin; Rhee, Byung Chull

    1984-01-01

    We have analyzed ultrasonographic findings of 55 cases of hydronephrosis and 34 cases of renal masses. The results are as follows: 1. 55 cases of hydronephrosis revealed renal enlargement in 55 cases, separation of central echo complex in 27 cases, multiple anechoic areas radiating from the center in 25 cases and dilated renal pelvis in 24 cases. 2. Among the masses in 34 cases, simple renal cyst were 15 cases, polycystic kidney in 8 cases, hypernephroma in 8 cases, Wilm's tumor in 2 cases and agiomyolipoma in 1 case. 3. Simple renal cyst revealed single in 14 cases (93%) and well defined anechoic mass with posterior enhancement in all cases. 4. Polycystic kidney revealed bilateral irregular shaped renal enlargement and multiple anechoic cysts throughout the kidney. 2 cases (25%) involved liver. 5. 6 cases (75%) of hypernephroma revealed ill defined moderately echogenic mass without posterior enhancement. 6. All cases of Wilm's tumor revealed well defined large mixed echogenic mass in right kidney. 7. Angiomyolipoma revealed bilateral dense echogenic mass with large hemorrhage cyst in right kidney. 8. The ultrasonography is useful noninvasive diagnostic modality of evaluation of renal masses and hydronephrosis.

  6. Screening of hypoxia-inducible genes in sporadic ALS.

    LENUS (Irish Health Repository)

    Cronin, Simon

    2008-10-01

    Genetic variations in two hypoxia-inducible angiogenic genes, VEGF and ANG, have been linked with sporadic amyotrophic lateral sclerosis (SALS). Common variations in these genes may reduce the levels or functioning of their products. VEGF and ANG belong to a larger group of angiogenic genes that are up-regulated under hypoxic conditions. We hypothesized that common genetic variation across other members of this group may also predispose to sporadic ALS. To screen other hypoxia-inducible angiogenic genes for association with SALS, we selected 112 tagging single nucleotide polymorphisms (tgSNPs) that captured the common genetic variation across 16 VEGF-like and eight ANG-like hypoxia-inducible genes. Screening for association was performed in 270 Irish individuals with typical SALS and 272 ethnically matched unrelated controls. SNPs showing association in the Irish phase were genotyped in a replication sample of 281 Swedish sporadic ALS patients and 286 Swedish controls. Seven markers showed association in the Irish. The one modest replication signal observed in the Swedish replication sample, at rs3801158 in the gene inhibin beta A, was for the opposite allele vs. the Irish cohort. We failed to detect association of common variation across 24 candidate hypoxia-inducible angiogenic genes with SALS.

  7. Next generation sequencing in sporadic retinoblastoma patients reveals somatic mosaicism.

    Science.gov (United States)

    Amitrano, Sara; Marozza, Annabella; Somma, Serena; Imperatore, Valentina; Hadjistilianou, Theodora; De Francesco, Sonia; Toti, Paolo; Galimberti, Daniela; Meloni, Ilaria; Cetta, Francesco; Piu, Pietro; Di Marco, Chiara; Dosa, Laura; Lo Rizzo, Caterina; Carignani, Giulia; Mencarelli, Maria Antonietta; Mari, Francesca; Renieri, Alessandra; Ariani, Francesca

    2015-11-01

    In about 50% of sporadic cases of retinoblastoma, no constitutive RB1 mutations are detected by conventional methods. However, recent research suggests that, at least in some of these cases, there is somatic mosaicism with respect to RB1 normal and mutant alleles. The increased availability of next generation sequencing improves our ability to detect the exact percentage of patients with mosaicism. Using this technology, we re-tested a series of 40 patients with sporadic retinoblastoma: 10 of them had been previously classified as constitutional heterozygotes, whereas in 30 no RB1 mutations had been found in lymphocytes. In 3 of these 30 patients, we have now identified low-level mosaic variants, varying in frequency between 8 and 24%. In 7 out of the 10 cases previously classified as heterozygous from testing blood cells, we were able to test additional tissues (ocular tissues, urine and/or oral mucosa): in three of them, next generation sequencing has revealed mosaicism. Present results thus confirm that a significant fraction (6/40; 15%) of sporadic retinoblastoma cases are due to postzygotic events and that deep sequencing is an efficient method to unambiguously distinguish mosaics. Re-testing of retinoblastoma patients through next generation sequencing can thus provide new information that may have important implications with respect to genetic counseling and family care.

  8. Stable and sporadic symbiotic communities of coral and algal holobionts

    Science.gov (United States)

    Hester, Eric R; Barott, Katie L; Nulton, Jim; Vermeij, Mark JA; Rohwer, Forest L

    2016-01-01

    Coral and algal holobionts are assemblages of macroorganisms and microorganisms, including viruses, Bacteria, Archaea, protists and fungi. Despite a decade of research, it remains unclear whether these associations are spatial–temporally stable or species-specific. We hypothesized that conflicting interpretations of the data arise from high noise associated with sporadic microbial symbionts overwhelming signatures of stable holobiont members. To test this hypothesis, the bacterial communities associated with three coral species (Acropora rosaria, Acropora hyacinthus and Porites lutea) and two algal guilds (crustose coralline algae and turf algae) from 131 samples were analyzed using a novel statistical approach termed the Abundance-Ubiquity (AU) test. The AU test determines whether a given bacterial species would be present given additional sampling effort (that is, stable) versus those species that are sporadically associated with a sample. Using the AU test, we show that coral and algal holobionts have a high-diversity group of stable symbionts. Stable symbionts are not exclusive to one species of coral or algae. No single bacterial species was ubiquitously associated with one host, showing that there is not strict heredity of the microbiome. In addition to the stable symbionts, there was a low-diversity community of sporadic symbionts whose abundance varied widely across individual holobionts of the same species. Identification of these two symbiont communities supports the holobiont model and calls into question the hologenome theory of evolution. PMID:26555246

  9. Blockade of Death Ligand TRAIL Inhibits Renal Ischemia Reperfusion Injury

    International Nuclear Information System (INIS)

    Adachi, Takaomi; Sugiyama, Noriyuki; Gondai, Tatsuro; Yagita, Hideo; Yokoyama, Takahiko

    2013-01-01

    Renal ischemia-reperfusion injury (IRI) is a leading cause of acute kidney injury (AKI). Many investigators have reported that cell death via apoptosis significantly contributed to the pathophysiology of renal IRI. Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) is a member of the tumor necrosis factor superfamily, and induces apoptosis and inflammation. However, the role of TRAIL in renal IRI is unclear. Here, we investigated whether TRAIL contributes to renal IRI and whether TRAIL blockade could attenuate renal IRI. AKI was induced by unilateral clamping of the renal pedicle for 60 min in male FVB/N mice. We found that the expression of TRAIL and its receptors were highly upregulated in renal tubular cells in renal IRI. Neutralizing anti-TRAIL antibody or its control IgG was given 24 hr before ischemia and a half-dose booster injection was administered into the peritoneal cavity immediately after reperfusion. We found that TRAIL blockade inhibited tubular apoptosis and reduced the accumulation of neutrophils and macrophages. Furthermore, TRAIL blockade attenuated renal fibrosis and atrophy after IRI. In conclusion, our study suggests that TRAIL is a critical pathogenic factor in renal IRI, and that TRAIL could be a new therapeutic target for the prevention of renal IRI

  10. Gamma Knife radiosurgery for treatment of growing vestibular schwannomas in patients with neurofibromatosis Type 2: a matched cohort study with sporadic vestibular schwannomas.

    Science.gov (United States)

    Kruyt, Ivo J; Verheul, Jeroen B; Hanssens, Patrick E J; Kunst, Henricus P M

    2018-01-01

    OBJECTIVE Neurofibromatosis Type 2 (NF2) is a tumor syndrome characterized by an autosomal dominant pattern of inheritance. The hallmark of NF2 is the development of bilateral vestibular schwannomas (VSs), generally by 30 years of age. One of the first-line treatment options for small to medium-large VSs is radiosurgery. Although radiosurgery shows excellent results in sporadic VS, its use in NF2-related VS is still a topic of dispute. The aim of this study was to evaluate long-term tumor control, hearing preservation rates, and factors influencing outcome of optimally dosed, contemporary Gamma Knife radiosurgery (GKRS) for growing VSs in patients with NF2 and compare the findings to data obtained in patients with sporadic VS also treated by means of GKRS. METHODS The authors performed a retrospective analysis of 47 growing VSs in 34 NF2 patients who underwent GKRS treatment performed with either the Model C or Perfexion Leksell Gamma Knife, with a median margin dose of 11 Gy. Actuarial tumor control rates were estimated using the Kaplan-Meier method. For patient- and treatment-related factors, a Cox proportional hazards model was used to identify predictors of outcome. Trigeminal, facial, and vestibulocochlear nerve function were assessed before and after treatment. NF2-related VS patients were matched 1:1 with sporadic VS patients who were treated in the same institute, and the same indications for treatment, definitions, and dosimetry were used in order to compare outcomes. RESULTS Actuarial tumor control rates in NF2 patients after 1, 3, 5, and 8 years were 98%, 89%, 87%, and 87%, respectively. Phenotype and tumor volume had significant hazard rates of 0.086 and 22.99, respectively, showing that Feiling-Gardner phenotype and a tumor volume not exceeding 6 cm 3 both were associated with significantly better outcome. Actuarial rates of serviceable hearing preservation after 1, 3, 5, and 7 years were 95%, 82%, 59%, and 33%, respectively. None of the patients

  11. Primary intracranial leiomyoma in renal transplant recipient

    Directory of Open Access Journals (Sweden)

    Upasana Patel

    2017-01-01

    Full Text Available Leiomyoma, the benign tumor of smooth muscle cell origin, is commonly seen in genitourinary and gastrointestinal tracts. Primary intracranial leiomyoma, however, is extremely rare occurrence. We hereby report a case of Epstein-Barr negative primary intracranial leiomyoma in a middle-aged renal transplant recipient, which mimicked left frontal parasagittal meningioma on neuroimaging. The tumor was completely excised and diagnosis of leiomyoma was clinched on pathological analysis with immunohistochemistry. The patient improved after tumor removal, and no evidence of tumor recurrence was noted on follow-up study after 10 months postsurgically.

  12. Cancer driver-passenger distinction via sporadic human and dog cancer comparison: a proof of principle study with colorectal cancer

    Science.gov (United States)

    Tang, Jie; Li, Yaping; Lyon, Kenneth; Camps, Jordi; Dalton, Stephen; Ried, Thomas; Zhao, Shaying

    2014-01-01

    Herein we report a proof of principle study illustrating a novel dog-human comparison strategy that addresses a central aim of cancer research, namely cancer driver–passenger distinction. We previously demonstrated that sporadic canine colorectal cancers (CRCs) share similar molecular pathogenesis mechanisms as their human counterparts. In this study, we compared the genome-wide copy number abnormalities between 29 human- and 10 canine sporadic CRCs. This led to the identification of 73 driver candidate genes (DCGs), altered in both species and with 27 from the whole genome and 46 from dog-human genomic rearrangement breakpoint (GRB) regions, as well as 38 passenger candidate genes (PCGs), altered in humans only and located in GRB regions. We noted that DCGs significantly differ from PCGs in every analysis conducted to assess their cancer relevance and biological functions. Importantly, while PCGs are not enriched in any specific functions, DCGs possess significantly enhanced functionality closely associated with cell proliferation and death regulation, as well as with epithelial cell apicobasal polarity establishment/maintenance. These observations support the notion that, in sporadic CRCs of both species, cell polarity genes not only contribute in preventing cancer cell invasion and spreading, but also likely serve as tumor suppressors by modulating cell growth. This pilot study validates our novel strategy and has uncovered four new potential cell polarity and colorectal tumor suppressor genes (RASA3, NUPL1, DENND5A, and AVL9). Expansion of this study would make more driver-passenger distinctions for cancers with large genomic amplifications or deletions, and address key questions regarding the relationship between cancer pathogenesis and epithelial cell polarity control in mammals. PMID:23416983

  13. Mediastinal tumor

    Science.gov (United States)

    Thymoma - mediastinal; Lymphoma - mediastinal ... mediastinal tumors in adults occur in the anterior mediastinum. They are usually cancerous (malignant) lymphomas, germ cell tumors, or thymomas. These tumors are ...

  14. Wunderlich's syndrome secondary to renal angiomyolipoma: CT diagnosis

    International Nuclear Information System (INIS)

    Sabate, J.M.; Gimenez, A.M.; Parellada, J.A.; Franquet, T.; Reig, C.

    1996-01-01

    We present three cases of renal angiomyolipoma with spontaneous perineal bleeding. The diagnosis was based on computerized tomography which disclosed the fatty component of the tumor and defined the retroperitoneal extension of the bleeding. (Author) 16 refs

  15. Reference genes for gene expression analysis by real-time reverse transcription polymerase chain reaction of renal cell carcinoma

    DEFF Research Database (Denmark)

    Bjerregaard, Henriette; Pedersen, Shona; Kristensen, Søren Risom

    2011-01-01

    Differentiation between malignant renal cell carcinoma and benign oncocytoma is of great importance to choose the optimal treatment. Accurate preoperative diagnosis of renal tumor is therefore crucial; however, existing imaging techniques and histologic examinations are incapable of providing an ...

  16. Strong Expression of Chemokine Receptor CXCR4 by Renal Cell Carcinoma Correlates with Advanced Disease

    Directory of Open Access Journals (Sweden)

    Thomas C. Wehler

    2008-01-01

    Full Text Available Diverse chemokines and their receptors have been associated with tumor growth, tumor dissemination, and local immune escape. In different tumor entities, the level of chemokine receptor CXCR4 expression has been linked with tumor progression and decreased survival. The aim of this study was to evaluate the influence of CXCR4 expression on the progression of human renal cell carcinoma. CXCR4 expression of renal cell carcinoma was assessed by immunohistochemistry in 113 patients. Intensity of CXCR4 expression was correlated with both tumor and patient characteristics. Human renal cell carcinoma revealed variable intensities of CXCR4 expression. Strong CXCR4 expression of renal cell carcinoma was significantly associated with advanced T-status (P=.039, tumor dedifferentiation (P = .0005, and low hemoglobin (P = .039. In summary, strong CXCR4 expression was significantly associated with advanced dedifferentiated renal cell carcinoma.

  17. Tumor-induced osteomalacia.

    Science.gov (United States)

    Jan de Beur, Suzanne M

    2005-09-14

    Tumor-induced osteomalacia (TIO) is a rare paraneoplastic form of renal phosphate wasting that results in severe hypophosphatemia, a defect in vitamin D metabolism, and osteomalacia. This debilitating disorder is illustrated by the clinical presentation of a 55-year-old woman with progressive fatigue, weakness, and muscle and bone pain with fractures. After a protracted clinical course and extensive laboratory evaluation, tumor-induced osteomalacia was identified as the basis of her clinical presentation. In this article, the distinctive clinical characteristics of this syndrome, the advances in diagnosis of TIO, and new insights into the pathophysiology of this disorder are discussed.

  18. Maxillary sporadic Burkitt′s lymphoma associated with neuro-orbital involvement in an Indian male

    Directory of Open Access Journals (Sweden)

    Rakesh Kumar Manne

    2014-01-01

    Full Text Available Burkitt′s lymphoma (BL is the fastest growing malignancy of the lymphoreticular system to affect humans and has a potential ability to double in size every day. A case of maxillary sporadic BL (sBL associated with neuro-orbital involvement in an Indian male is presented. sBL initially presented as maxillary swelling with no obvious dental and periodontal changes. Histological specimen from incisional biopsy revealed a round cell malignant tumor and immunohistochemistry reactions favored nonHodgkin′s lymphoma consistent with BL. Four weeks later, patient presented with orbital involvement as diplopia, sixth cranial nerve palsy, and medial rectus palsy. Chemotherapy regimen according to LMB 89 protocol was started. During chemotherapy regimen patient showed bradycardia and Babinski response, suggestive of central nervous system involvement. sBL associated with orbital involvement is extremely rare and only seven cases have been reported. Our case showed unusual presentation; despite the aggressive tumor did not show any common clinical, radiological, and hematological findings. We also discussed the role of oral medicine specialist, importance of early diagnosis, and prompt referral in management of maxillary sBL.

  19. Outcome and renal function following salvage surgery for bilateral ...

    African Journals Online (AJOL)

    Objective: The aim of this study was to determine the surgical outcomes and renal function following salvage surgery for bilateral Wilms tumor (BWT). Summary background data The challenge for the surgeon treating BWT lies in striking a fine balance between renal preservation and oncological clearance. Methods: This is ...

  20. Primary renal leiomyosarcoma: Case report and literature review ...

    African Journals Online (AJOL)

    Primary leimyosarcoma of the kidney is extremely rare. The management is unclear due to the limited literature on the subject. The diagnosis should be considered when imaging shows a relatively hypovascular tumor involving one renal pole, with atypical features on microscopy. We report a renal leiomyosarcoma in a 68 ...

  1. Outcome and renal function following salvage surgery for bilateral ...

    African Journals Online (AJOL)

    Objective The aim of this study was to determine the surgical outcomes and renal function following salvage surgery for bilateral Wilms tumor (BWT). Summary background data The challenge for the surgeon treating BWT lies in striking a fine balance between renal preservation and oncological clearance. Methods This is a ...

  2. Endothelial protein C receptor in renal tubular epithelial cells and ...

    African Journals Online (AJOL)

    This study aims to investigate EPCR expression in renal tubular epithelial cells and related influencing factors. EPCR expression was assessed by flow cytometry in renal tubular epithelial cells. The effects of some reagents (high glucose, tumor necrosis factor–α and interleukin-1β) were measured by RT-PCR. The results ...

  3. Sporadic versus Radiation-Associated Angiosarcoma: A Comparative Clinicopathologic and Molecular Analysis of 48 Cases

    Directory of Open Access Journals (Sweden)

    Jennifer Hung

    2013-01-01

    Full Text Available Angiosarcomas are aggressive tumors of vascular endothelial origin, occurring sporadically or in association with prior radiotherapy. We compared clinicopathologic and biologic features of sporadic angiosarcomas (SA and radiation-associated angiosarcomas (RAA. Methods. From a University of Michigan institutional database, 37 SA and 11 RAA were identified. Tissue microarrays were stained for p53, Ki-67, and hTERT. DNA was evaluated for TP53 and ATM mutations. Results. Mean latency between radiotherapy and diagnosis of RAA was 11.9 years: 6.7 years for breast RAA versus 20.9 years for nonbreast RAA (P=0.148. Survival after diagnosis did not significantly differ between SA and RAA (P=0.590. Patients with nonbreast RAA had shorter overall survival than patients with breast RAA (P=0.03. The majority of SA (86.5% and RAA (77.8% were classified as high-grade sarcomas (P=0.609. RAA were more likely to have well-defined vasoformative areas (55.6% versus 27%, P=0.127. Most breast SA were parenchymal in origin (80%, while most breast RAA were cutaneous in origin (80%. TMA analysis showed p53 overexpression in 25.7% of SA and 0% RAA, high Ki-67 in 35.3% of SA and 44.4% RAA, and hTERT expression in 100% of SA and RAA. TP53 mutations were detected in 13.5% of SA and 11.1% RAA. ATM mutations were not detected in either SA or RAA. Conclusions. SA and RAA are similar in histology, immunohistochemical markers, and DNA mutation profiles and share similar prognosis. Breast RAA have a shorter latency period compared to nonbreast RAA and a significantly longer survival.

  4. Renal tuberculosis

    Directory of Open Access Journals (Sweden)

    Džamić Zoran

    2016-01-01

    Full Text Available Tuberculosis is still a significant health problem in the world, mostly in developing countries. The special significance lies in immunocompromised patients, particularly those suffering from the HIV. Urogenital tuberculosis is one of the most common forms of extrapulmonary tuberculosis, while the most commonly involved organ is the kidney. Renal tuberculosis occurs by hematogenous dissemination of mycobacterium tuberculosis from a primary tuberculosis foci in the body. Tuberculosis is characterized by the formation of pathognomonic lesions in the tissues - granulomata. These granulomata may heal spontaneously or remain stable for years. In certain circumstances in the body associated with immunosuppression, the disease may be activated. Central caseous necrosis occurs within tuberculoma, leading to formation of cavities that destroy renal parenchyma. The process may gain access to the collecting system, forming the caverns. In this way, infection can be spread distally to renal pelvis, ureter and bladder. Scaring of tissue by tuberculosis process may lead to development of strictures of the urinary tract. The clinical manifestations are presented by nonspecific symptoms and signs, so tuberculosis can often be overlooked. Sterile pyuria is characteristic for urinary tuberculosis. Dysuric complaints, flank pain or hematuria may be presented in patients. Constitutional symptoms of fever, weight loss and night sweats are presented in some severe cases. Diagnosis is made by isolation of mycobacterium tuberculosis in urine samples, by cultures carried out on standard solid media optimized for mycobacterial growth. Different imaging studies are used in diagnostics - IVU, CT and NMR are the most important. Medical therapy is the main modality of tuberculosis treatment. The first line anti-tuberculosis drugs include isoniazid, rifampicin, pyrazinamide and ethambutol. Surgical treatment is required in some cases, to remove severely damaged kidney, if

  5. Drug-induced renal injury

    African Journals Online (AJOL)

    induced renal toxicity into four major renal syndromes: • acute renal failure. • chronic renal failure. • glomerulonephritis. • tubulopathies. These major renal syndromes are discussed in further detail below (see summary in Table I). Acute renal failure. Drugs can cause acute renal failure by causing pre-renal, intrinsic or.

  6. Integrated genomic characterization reveals novel, therapeutically relevant drug targets in FGFR and EGFR pathways in sporadic intrahepatic cholangiocarcinoma.

    Directory of Open Access Journals (Sweden)

    Mitesh J Borad

    2014-02-01

    Full Text Available Advanced cholangiocarcinoma continues to harbor a difficult prognosis and therapeutic options have been limited. During the course of a clinical trial of whole genomic sequencing seeking druggable targets, we examined six patients with advanced cholangiocarcinoma. Integrated genome-wide and whole transcriptome sequence analyses were performed on tumors from six patients with advanced, sporadic intrahepatic cholangiocarcinoma (SIC to identify potential therapeutically actionable events. Among the somatic events captured in our analysis, we uncovered two novel therapeutically relevant genomic contexts that when acted upon, resulted in preliminary evidence of anti-tumor activity. Genome-wide structural analysis of sequence data revealed recurrent translocation events involving the FGFR2 locus in three of six assessed patients. These observations and supporting evidence triggered the use of FGFR inhibitors in these patients. In one example, preliminary anti-tumor activity of pazopanib (in vitro FGFR2 IC50≈350 nM was noted in a patient with an FGFR2-TACC3 fusion. After progression on pazopanib, the same patient also had stable disease on ponatinib, a pan-FGFR inhibitor (in vitro, FGFR2 IC50≈8 nM. In an independent non-FGFR2 translocation patient, exome and transcriptome analysis revealed an allele specific somatic nonsense mutation (E384X in ERRFI1, a direct negative regulator of EGFR activation. Rapid and robust disease regression was noted in this ERRFI1 inactivated tumor when treated with erlotinib, an EGFR kinase inhibitor. FGFR2 fusions and ERRFI mutations may represent novel targets in sporadic intrahepatic cholangiocarcinoma and trials should be characterized in larger cohorts of patients with these aberrations.

  7. [Case report of rare co-occurrence of renal cell carcinoma and crossed renal dystopia (L-shaped kidney)].

    Science.gov (United States)

    Bakov, V N; Los, M S

    2017-10-01

    L-shaped kidney refers to a rare anomaly of the relative kidney positioning. Due to low prevalence, the literature on the co-occurrence of this anomaly with malignancy is lacking. And, if the diagnosis of a renal anomaly does not present difficulties, if a tumor is detected in such a kidney, even MSCT does not always help differentiate a pelvic tumor from a tumor of the renal parenchyma spreading to the pelvicalyceal system. This has important implications for choosing an appropriate surgical strategy. A feature of the presented clinical observation is the co-occurrence of the rare anomaly of kidney position and locally advanced renal cell carcinoma spreading to the renal pelvis. Due to the massive spread of the tumor, an organ-sparing surgery was not feasible. Due to the suspicion of tumor spread to the renal pelvis, the patient underwent nephrureterectomy of the L-shaped kidney. Introduction to renoprival state with transfer to chronic hemodialysis became the only option to maintain homeostasis and extend the patients life. Histological examination revealed clear cell renal cell carcinoma with invasion of the pelvis and renal capsule, with no clear demarcation between the fused kidneys.

  8. A Ten-Year Review of Childhood Renal Admissions into the Lagos ...

    African Journals Online (AJOL)

    A Ten-Year Review of Childhood Renal Admissions into the Lagos University Teaching Hospital, Nigeria. ... Wilms Tumor (15.3%), acute renal failure (7.4%), urinary tract infection (4.9%), posterior urethral valves 3.4%), and chronic renal failure (0.5%) in order of ranking. Mortality was 17.7% and deaths were mainly from ...

  9. MR Imaging of papillary renal neoplasms: potential application for characterization of small renal masses

    International Nuclear Information System (INIS)

    Roy, Catherine; Sauer, Benoit; Lindner, Veronique; Lang, Herve; Saussine, Christian; Jacqmin, Didier

    2007-01-01

    The purpose of our study was to evaluate the role of MRI in demonstrating the precise nature of papillary renal tumors (P RCC) and its potential application to select patients for partial surgery. Ninety-seven tumors less than or equal to 3 cm in size [55 papillary renal cell carcinoma - 42 clear cell renal carcinoma (CC RCC)] were preoperatively evaluated by MRI. Imaging findings were assessed with a special focus on the aspect of the tumoral process. Correlations were performed with pathologic staging after surgery. At pathology, 92 tumors were established to be staged p T1 and 5 were p T3 (3 cases of CC RCC and 2 cases of P RCC). Ninety-four percent of papillary tumors exhibited low signal intensity with homogeneous pattern on T2-weighted images. All clear cell carcinoma were hyperintense and heterogeneous on T2-weighted sequence. Enhancement was lower and delayed in the papillary type in comparison with the clear cell type. MRI is accurate enough to predict the 'histologic' nature of papillary renal carcinoma. It is an additional argument to propose that the tumor can be removed by partial surgery. (orig.)

  10. Renal calculus

    CERN Document Server

    Pyrah, Leslie N

    1979-01-01

    Stone in the urinary tract has fascinated the medical profession from the earliest times and has played an important part in the development of surgery. The earliest major planned operations were for the removal of vesical calculus; renal and ureteric calculi provided the first stimulus for the radiological investigation of the viscera, and the biochemical investigation of the causes of calculus formation has been the training ground for surgeons interested in metabolic disorders. It is therefore no surprise that stone has been the subject of a number of monographs by eminent urologists, but the rapid development of knowledge has made it possible for each one of these authors to produce something new. There is still a technical challenge to the surgeon in the removal of renal calculi, and on this topic we are always glad to have the advice of a master craftsman; but inevitably much of the interest centres on the elucidation of the causes of stone formation and its prevention. Professor Pyrah has had a long an...

  11. Prognostic factors of the therapeutic efficacy of mTOR and VEGFR inhibitors in patients with metastatic renal cell carcinoma

    Directory of Open Access Journals (Sweden)

    Е. А. Voroshilova

    2015-01-01

    Full Text Available Background. Thorough study of the molecular genetic alterations in patients with hereditary and sporadic renal cell carcinoma (RCC enabled to reveal potential therapeutic targets - vascular endothelial growth factor (VEGF, platelet-derived growth factor (PDGF, growth factor receptors (VEGFR, PDGFR, EGFR, FGFR, mTOR signaling protein. Advances in targeted therapy treatment in the current therapeutic practice have brought a problem of its rational use and ultimately effective outcomes. The main solution of solving this problem is to establish independent clinical and laboratory prognostic factors and molecular markers which could predict the efficacy of targeted therapy.Objective – optimization of targeted therapy in patients with RCC by using both molecular and genetic prognostic factors as predictors of the treatment efficacy.Materials and methods. We assessed the level of mRNA expression of 13 potential target genes in primary tumor and metastatic site of patients suffering from metastatic RCC (n = 43 and evaluated the influence of the selected genes’ expression on the therapeutic efficacy of mTOR inhibitors and VEGFR inhibitors.Conclusion. VEGFR1 mRNA overexpression in metastatic site as well as mTOR and/or PI3K mRNA overexpression could be assessed as potential biomarkers in predicting the treatment efficacy of VEGFR inhibitors and mTOR inhibitors respectively. The higher expression of RAF1 mRNA and mTOR signaling pathway are not typical molecular alterations in patients with mRCC. RAF1 mRNA overexpression in metastatic site as well as activation of the alternative signaling pathway (RAS-RAF-MAPK in tumor cell are negative prognostic factors of the efficacy of targeted therapy. Activation of the signaling RAS-RAF-MAPK pathway in tumor cells is probably an alternative independent mechanism that “drives” tumor development in certain groups of patients.

  12. Metastatic renal cell carcinoma without evidence of a renal primary

    Science.gov (United States)

    Costantino, Corey; Thomas, George V.; Ryan, Christopher; Coakley, Fergus V.; Troxell, Megan L.

    2016-01-01

    Purpose Metastatic renal cell carcinoma (RCC), without an identified kidney primary, has been reported rarely. We report a patient with RCC metastatic to bilateral adrenal glands and liver, without an apparent renal primary. We detail the immunohistochemical and molecular studies employed to substantiate the diagnosis of RCC and direct therapy. Methods Histopathologic findings were correlated with imaging data and supplemented by a panel of immunohistochemical stains, as well as tumor sequence analysis. Results Despite the presence of bilateral adrenal masses and lack of tumor within kidney parenchyma, the diagnosis of RCC was substantiated by immunohistochemistry (RCC+/PAX2+/PAX8+/Melan-A−/SF-1− among others) and molecular genetic analysis, harboring mutations in VHL, TP53, KDM5C, and PBRM1. After debulking surgery, based on the diagnosis of RCC and the molecular profile, the patient was treated with a tyrosine kinase inhibitor (sunitinib), resulting in stablilization of disease. Conclusions This case illustrates the role of mutational analysis in carcinomas with rare or unusual presentations, such as metastatic RCC without a renal primary. PMID:26527083

  13. Application of quantitative DTI metrics in sporadic CJD

    Directory of Open Access Journals (Sweden)

    E. Caverzasi

    2014-01-01

    Full Text Available Diffusion Weighted Imaging is extremely important for the diagnosis of probable sporadic Jakob–Creutzfeldt disease, the most common human prion disease. Although visual assessment of DWI MRI is critical diagnostically, a more objective, quantifiable approach might more precisely identify the precise pattern of brain involvement. Furthermore, a quantitative, systematic tracking of MRI changes occurring over time might provide insights regarding the underlying histopathological mechanisms of human prion disease and provide information useful for clinical trials. The purposes of this study were: 1 to describe quantitatively the average cross-sectional pattern of reduced mean diffusivity, fractional anisotropy, atrophy and T1 relaxation in the gray matter (GM in sporadic Jakob–Creutzfeldt disease, 2 to study changes in mean diffusivity and atrophy over time and 3 to explore their relationship with clinical scales. Twenty-six sporadic Jakob–Creutzfeldt disease and nine control subjects had MRIs on the same scanner; seven sCJD subjects had a second scan after approximately two months. Cortical and subcortical gray matter regions were parcellated with Freesurfer. Average cortical thickness (or subcortical volume, T1-relaxiation and mean diffusivity from co-registered diffusion maps were calculated in each region for each subject. Quantitatively on cross-sectional analysis, certain brain regions were preferentially affected by reduced mean diffusivity (parietal, temporal lobes, posterior cingulate, thalamus and deep nuclei, but with relative sparing of the frontal and occipital lobes. Serial imaging, surprisingly showed that mean diffusivity did not have a linear or unidirectional reduction over time, but tended to decrease initially and then reverse and increase towards normalization. Furthermore, there was a strong correlation between worsening of patient clinical function (based on modified Barthel score and increasing mean diffusivity.

  14. Imaging and clinical characteristics of sporadic Creutzfeldt-Jakob disease

    Directory of Open Access Journals (Sweden)

    HAN Shun-chang

    2013-04-01

    Full Text Available Five patients with sporadic Creutzfeldt-Jakob disease (sCJD presented rapidly progressive dementia which were subacute onset from 1 to 4 months. Among these cases, periodic synchronous discharge (PSD of electroencephalography (EEG was seen in 2 patients. Besides, 4 patients obtained positive results in cerebrospinal fluid (CSF analysis for 14-3-3 protein. The cranial MRI examination showed symmetrical or asymmetrical colored-ribbon-shaped high signals in cerebral cortex or basal ganglia by diffusion weighted imaging (DWI, suggesting that DWI had high sensitivity and specificity for the diagnosis of sCJD as a preferred method in the clinical examination of sCJD.

  15. Diffusion MR imaging in sporadic Creutzfeldt-Jakob disease

    Directory of Open Access Journals (Sweden)

    Burcak Cakir Pekoz

    2014-08-01

    Full Text Available Creutzfeldt-Jakob disease (CJD is a rare dementing disease and is thought to caused by a prion. It is characterized by rapidly progressive dementia, ataxia, myoclonus, akinetic mutism and eventual death. Brain biopsy or autopsy is required for a definitive diagnosis of CJD. Diffusion-weighted imaging became an important tool for early diagnosis of CJD because of the high sensitivity. We present 59-year-old female patient diagnosed as sporadic CJD with typical MR imagings. [Cukurova Med J 2014; 39(4.000: 880-883

  16. The monster sporadic group and a theory underlying superstring models

    International Nuclear Information System (INIS)

    Chapline, G.

    1996-09-01

    The pattern of duality symmetries acting on the states of compactified superstring models reinforces an earlier suggestion that the Monster sporadic group is a hidden symmetry for superstring models. This in turn points to a supersymmetric theory of self-dual and anti-self-dual K3 manifolds joined by Dirac strings and evolving in a 13 dimensional spacetime as the fundamental theory. In addition to the usual graviton and dilaton this theory contains matter-like degrees of freedom resembling the massless states of the heterotic string, thus providing a completely geometric interpretation for ordinary matter. 25 refs

  17. TRANSPLANTE RENAL

    Directory of Open Access Journals (Sweden)

    Soraia Geraldo Rozza Lopes

    2014-01-01

    Full Text Available El objetivo del estudio fue comprender el significado de espera del trasplante renal para las mujeres en hemodiálisis. Se trata de un estudio cualitativo-interpretativo, realizado con 12 mujeres en hemodiálisis en Florianópolis. Los datos fueron recolectados a través de entrevistas en profundidad en el domicilio. Fue utilizado el software Etnografh 6.0 para la pre-codificación y posterior al análisis interpretativo emergieron dos categorías: “las sombras del momento actual”, que mostró que las dificultades iniciales de la enfermedad están presentes, pero las mujeres pueden hacer frente mejor a la enfermedad y el tratamiento. La segunda categoría, “la luz del trasplante renal”, muestra la esperanza impulsada por la entrada en la lista de espera para un trasplante.

  18. [Hypertension and renal disease

    DEFF Research Database (Denmark)

    Kamper, A.L.; Pedersen, E.B.; Strandgaard, S.

    2009-01-01

    Renal mechanisms, in particular the renin-angiotensin system and renal salt handling, are of major importance in blood pressure regulation. Co-existence of hypertension and decreased renal function may be due to nephrosclerosis secondary to hypertension, or primary renal disease with secondary...

  19. Slower Dynamics and Aged Mitochondria in Sporadic Alzheimer's Disease

    Science.gov (United States)

    Gargini, Ricardo; García, Esther; Perry, George

    2017-01-01

    Sporadic Alzheimer's disease corresponds to 95% of cases whose origin is multifactorial and elusive. Mitochondrial dysfunction is a major feature of Alzheimer's pathology, which might be one of the early events that trigger downstream principal events. Here, we show that multiple genes that control mitochondrial homeostasis, including fission and fusion, are downregulated in Alzheimer's patients. Additionally, we demonstrate that some of these dysregulations, such as diminished DLP1 levels and its mitochondrial localization, as well as reduced STOML2 and MFN2 fusion protein levels, take place in fibroblasts from sporadic Alzheimer's disease patients. The analysis of mitochondrial network disruption using CCCP indicates that the patients' fibroblasts exhibit slower dynamics and mitochondrial membrane potential recovery. These defects lead to strong accumulation of aged mitochondria in Alzheimer's fibroblasts. Accordingly, the analysis of autophagy and mitophagy involved genes in the patients demonstrates a downregulation indicating that the recycling mechanism of these aged mitochondria might be impaired. Our data reinforce the idea that mitochondrial dysfunction is one of the key early events of the disease intimately related with aging. PMID:29201274

  20. FISIOPATOLOGÍA DE LA INSUFICIENCIA RENAL AGUDA POR CISPLATINO

    Directory of Open Access Journals (Sweden)

    Rodriguez Macías EL

    2013-09-01

    Full Text Available Cisplatin is one of the major antineoplastic used for treatment of tumors such as testicular, ovarian, cervical, lung, bladder and head, among others. It has been described various types of toxicities induced by cisplatin, but the renal is the main one. This toxicity consist sof an acute reduction in renal plasma flow and a decline in glomerular filtration and installation of a tubular necrosis, with predominant involvement of the distal tubules and accumulation of cellular debris (lumen obstruction. Other mechanisms involved in renal injury are microangiopathy and pro-inflammatory mediators. We conclude that the nephrotoxicity caused by cisplatin can induce renal failure in which tubular structural damage predominates.

  1. Microwave treatment of renal cell carcinoma adjacent to renal sinus.

    Science.gov (United States)

    Gao, Yongyan; Liang, Ping; Yu, Xiaoling; Yu, Jie; Cheng, Zhigang; Han, Zhiyu; Duan, Shaobo; Huang, Hui

    2016-11-01

    To evaluate the efficacy and safety of ultrasound (US)-guided percutaneous microwave ablation (MWA) for renal cell carcinoma (RCC) adjacent to renal sinus. This retrospective study included 41 patients who underwent US-guided percutaneous MWA of 41 RCCs adjacent to the renal sinus from April 2006 to December 2015. Contrast-enhanced images of US and computed tomography (CT) or magnetic resonance (MR) imaging were performed at pre-ablation and 1day, 1 month, 3 months, and every 6 months after ablation. Initial ablation success (IAS), disease-free survival (DFS), RCC-related survival (RRS), and overall survival (OS) were recorded at the follow-up visits. IAS was achieved in 92.7% (38/41) of the study subjects. The IAS significantly differed between patients with RCCs ≤4cm (100%, 29/29) and RCCs >4cm (75%, 9/12, p=0.021). During the median follow-up of 37.6 (range, 3.0-97.3) months, the estimated 1-, 3-, and 5-year DFS of patients with an initial tumor of ≤4cm were 100%, 89.7%, and 81.5%, respectively. The 1-, 3-, and 5-year RRS were 100%, 93.3%, and 93.3%, respectively. The 1-, 3-, and 5-year OS were 97.1%, 87.8%, and 83.6%, respectively. The multivariate analysis using the Cox proportional hazard model revealed no independent predictor of recurrence among all the variables. There were no MWA-related deaths among the study subjects. One patient developed a retroperitoneal abscess after ablation. US-guided percutaneous MWA appears to be a promising method for RCCs adjacent to renal sinus, especially for tumors ≤4cm. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  2. Giant renal artery pseudoaneurysm caused by rupture of renal angiomyolipoma following pregnancy: Endovascular treatment and review of the literature

    Directory of Open Access Journals (Sweden)

    Ilkay S Idilman

    2014-01-01

    Full Text Available Renal angiomyolipoma is a hamartomatous, benign tumor composed of blood vessels, fatty tissue and smooth muscle cells, and is often detected incidentally. It can also be associated with the tuberous-sclerosis complex (TSC. Pregnancy and use of oral contraceptives are known to be associated with an increased risk of tumoral rupture and bleeding. Herein, we report a unique case of renal angiomyolipoma associated with TSC who presented with hypovolemic shock as a result of spontaneous rupture of a giant renal pseudoaneurysm, immediately after pregnancy. Emergency endovascular treatment was successful with sparing of most of the affected kidney as demonstrated by follow-up computed tomography imaging.

  3. End Stage and Chronic Kidney Disease: Associations with Renal Cancer

    International Nuclear Information System (INIS)

    Russo, Paul

    2012-01-01

    There is a well known association between end stage renal disease and the development of kidney cancer in the native kidney of patients requiring renal replacement therapy. There is now emerging evidence that lesser degrees of renal insufficiency (chronic kidney disease, CKD) are also associated with an increased likelihood of cancer in general and kidney cancer in particular. Nephropathological changes are commonly observed in the non-tumor bearing portions of kidney resected at the time of partial and radical nephrectomy (RN). In addition, patients with renal cancer are more likely to have CKD at the time of diagnosis and treatment than the general population. The exact mechanism by which renal insufficiency transforms normal kidney cells into tumor cells is not known. Possible mechanisms include uremic immune inhibition or increased exposure to circulating toxins not adequately cleared by the kidneys. Surgeons managing kidney tumors must have an increased awareness of their patient’s renal functional status as they plan their resection. Kidney sparing approaches, including partial nephrectomy (PN) or active surveillance in older and morbidly ill patients, can prevent CKD or delay the further decline in renal function which is well documented with RN. Despite emerging evidence that PN provides equivalent local tumor control to RN while at the same time preventing CKD, this operation remains under utilized in the United States and abroad. Increased awareness of the bi directional relationship between kidney function and kidney cancer is essential in the contemporary management of kidney cancer.

  4. Molecular characteristics of mismatch repair genes in sporadic colorectal tumors in Czech patients

    Czech Academy of Sciences Publication Activity Database

    Vymetálková, Veronika; Slyšková, Jana; Korenková, Vlasta; Bielik, Ludovít; Langerová, Lucie; Procházka, Pavel; Rejhová, Alexandra; Schwarzová, L.; Pardini, B.; Naccarati, Alessio; Vodička, Pavel

    2014-01-01

    Roč. 15, č. 1 (2014), s. 17 ISSN 1471-2350 R&D Projects: GA AV ČR IAA500200917; GA ČR(CZ) GPP304/11/P715 Grant - others:GA MŠk(CZ) Prvouk-P27/LF1/1 Institutional support: RVO:68378041 ; RVO:86652036 Keywords : colorectal cancer * mismatch repair genes * expression levels Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 2.083, year: 2014

  5. A Mouse Model for the Cloning of a Tumor Suppressor Gene Mutated in Sporadic Breast Cancer

    Science.gov (United States)

    1998-07-01

    identification as part of a phophatase derived from the full-length sequence. Wnt-3a Wnit-4 Wnt interacting clone: full-length mPPT : 5’--PR repeats...phosphatasedoma’ Fig. 2 mPPT interacting domain. The above mPPT protein subfragment, including the TPR motifs, represents the mPPT domain the four Wnt baits...activity. PPT exhibits phosphatase activity when the TPR region is deleted (32). The putative dominant activating and negative murine PPT ( mPPT ) forms

  6. Genome-Wide Uniparental Disomy and Copy Number Variations in Renal Cell Carcinomas Associated with Birt-Hogg-Dubé Syndrome.

    Science.gov (United States)

    Iribe, Yasuhiro; Yao, Masahiro; Tanaka, Reiko; Kuroda, Naoto; Nagashima, Yoji; Nakatani, Yukio; Furuya, Mitsuko

    2016-02-01

    Birt-Hogg-Dubé syndrome is an inherited disorder caused by germline mutations of the folliculin gene (FLCN). The affected patients are prone to developing renal cell carcinomas (RCCs). Most mutant FLCN-associated RCCs (mFLCN-RCCs) are histologically chromophobe RCCs and hybrid oncocytic/chromophobe tumors. It is incompletely understood whether mFLCN-RCCs have different chromosomal abnormalities compared with their sporadic histological counterparts. Herein, we describe somatic mutations of FLCN and DNA-copy number abnormalities using a high-density, whole-genome, single-nucleotide polymorphism array. The histological types included chromophobe RCC (n = 12), hybrid oncocytic/chromophobe tumor (n = 5), and clear-cell RCC (n = 2). Of 19 tumors, 8 had pathological somatic mutations of FLCN. Among 11 mFLCN-RCCs investigated by single-nucleotide polymorphism array, 8 showed balanced genomic profiles, 2 had gains in chromosome 3q, and 1 had gains in chromosomes 1q and 7. All had copious numbers of loss of heterozygosity in a wide range of chromosomes. The common loss-of-heterozygosity regions were chromosomes 3p24, 8q11, 16q11, Xp22-21, Xp11, Xq11, Xq13, and Xq23. Most of the loss of heterozygosity was because of uniparental disomy. Common uniparental disomy patterns in chromophobe RCCs and hybrid oncocytic/chromophobe tumors indicated that these types were relatively similar in cytogenetic events. Two clear-cell RCCs also shared several uniparental disomy regions with chromophobe RCCs and hybrid oncocytic/chromophobe tumors. mFLCN-RCCs may have common therapeutic targets among different histological types. Copyright © 2016 American Society for Investigative Pathology. Published by Elsevier Inc. All rights reserved.

  7. Genetic abnormalities in sporadic parathyroid adenomas: Loss of heterozygosity for chromosome 3q markers flanking the calcium receptor locus

    Energy Technology Data Exchange (ETDEWEB)

    Thompson, D.B.; Samowitz, W.S.; Davis, K. [Univ. of Utah School of Medicine, Salt Lake City, UT (United States)] [and others

    1995-10-01

    Inactivating mutations of the parathyroid cell calcium receptor (CaR) gene cause one form of familial benign/hypocalciuric hypercalcemia, and in homozygous form, cause neonatal severe primary hyperparathyroidism with parathyroid hyperplasia. Thus, we postulated that partial or total loss of CaR function might contribute to calcium insensitivity or even stimulate cell proliferation in sporadic parathyroid adenomas (PAds). To examine this possibility, we sought loss of heterozygosity (LOH) for markers flanking the CaR locus (3cen-3q21) in 35 PAds. We used 16 highly-polymorphic PCR-based markers in paired normal and tumor DNA, extracted from archived surgical specimens. Nineteen to twenty-four of the DNA pairs were informative with at least one marker. In two informative pairs, we found LOH for markers D3S1303, D3S1267, or D3S1269, which are tightly-linked with and flank the CaR locus. In one tumor, deletion mapping confined the lost area between D3S1271 and D3S1238 (41.7 centimorgans, cM). In the other tumor, LOH spanned most of chromosome 3, ranging at least from D3S1307 to D3S1311 (271.4 cM). LOH was confirmed by repetition of the experiments and quantified by phosphorimaging. Thus, we found LOH encompassing the CaR locus in approximately 10% of sporadic PAds. These data are consistent with the hypothesis that loss of CaR function may occur in PAds, with functional consequences for calcium sensitivity and cell proliferation. 20 refs., 2 figs.

  8. A case of lymphoma presented with acute renal failure

    Directory of Open Access Journals (Sweden)

    Mustafa Yaprak

    2017-03-01

    Full Text Available Acute renal failure (ARF in patients with malignancy occurs due to causes such as prerenal, renal and post renal as in normal population. Tumor infiltration of kidneys is usually uncommon. However, renal function may be impaired in fast-growing hematological malignancies such as acute leukemia or lymphoma, depending on tumor involvement. Herein, we presented a case of ARF and later diagnosed as B-cell Non-Hodgkin's lymphoma. 54-year-old male patient was admitted due to ARF. Although development of ARF due to tumor infiltration is rare, in cases who did not have risk factors for development of ARF, leukemic or lymphomatous infiltration should be considered. [Cukurova Med J 2017; 42(1.000: 168-171

  9. Microwave treatment of renal cell carcinoma adjacent to renal sinus

    International Nuclear Information System (INIS)

    Gao, Yongyan; Liang, Ping; Yu, Xiaoling; Yu, Jie; Cheng, Zhigang; Han, Zhiyu; Duan, Shaobo; Huang, Hui

    2016-01-01

    Highlights: • This study shows US-guided microwave ablation appears to be a promising method to treat renal cell carcinoma adjacent to renal sinus. • The estimated 1-, 3- and 5-year RCC-related survival were 100%, 93.3% and 93.3%, respectively. • The estimated 1-, 3- and 5-year overall survival were 97.1%, 87.8%, 83.6%, respectively. • For patients with RCC ≤4 cm, initial ablation success was 100% (29/29) and the estimated 5-year disease-free survival were 81.5%. - Abstract: Purpose: To evaluate the efficacy and safety of ultrasound (US)-guided percutaneous microwave ablation (MWA) for renal cell carcinoma (RCC) adjacent to renal sinus. Materials and methods: This retrospective study included 41 patients who underwent US-guided percutaneous MWA of 41 RCCs adjacent to the renal sinus from April 2006 to December 2015. Contrast-enhanced images of US and computed tomography (CT) or magnetic resonance (MR) imaging were performed at pre-ablation and 1 day, 1 month, 3 months, and every 6 months after ablation. Initial ablation success (IAS), disease-free survival (DFS), RCC-related survival (RRS), and overall survival (OS) were recorded at the follow-up visits. Results: IAS was achieved in 92.7% (38/41) of the study subjects. The IAS significantly differed between patients with RCCs ≤4 cm (100%, 29/29) and RCCs >4 cm (75%, 9/12, p = 0.021). During the median follow-up of 37.6 (range, 3.0–97.3) months, the estimated 1-, 3-, and 5-year DFS of patients with an initial tumor of ≤4 cm were 100%, 89.7%, and 81.5%, respectively. The 1-, 3-, and 5-year RRS were 100%, 93.3%, and 93.3%, respectively. The 1-, 3-, and 5-year OS were 97.1%, 87.8%, and 83.6%, respectively. The multivariate analysis using the Cox proportional hazard model revealed no independent predictor of recurrence among all the variables. There were no MWA-related deaths among the study subjects. One patient developed a retroperitoneal abscess after ablation. Conclusion: US-guided percutaneous MWA

  10. Microwave treatment of renal cell carcinoma adjacent to renal sinus

    Energy Technology Data Exchange (ETDEWEB)

    Gao, Yongyan, E-mail: gaoyongyan7@163.com [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853 (China); Department of Ultrasound, The General Hospital of Chinese People’s Armed Police Forces, 69 Yongding Road, Beijing, 100039 (China); Liang, Ping, E-mail: liangping301@hotmail.com [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853 (China); Yu, Xiaoling, E-mail: 784107477@qq.com [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853 (China); Yu, Jie, E-mail: 1411495161@qq.com [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853 (China); Cheng, Zhigang, E-mail: 13691367317@163.com [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853 (China); Han, Zhiyu, E-mail: hanzhiyu122@163.com [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853 (China); Duan, Shaobo, E-mail: Dustin2662@163.com [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853 (China); Huang, Hui, E-mail: 309hh@sina.com [Department of Interventional Ultrasound, Chinese PLA General Hospital, 28 Fuxing Road, Beijing, 100853 (China)

    2016-11-15

    Highlights: • This study shows US-guided microwave ablation appears to be a promising method to treat renal cell carcinoma adjacent to renal sinus. • The estimated 1-, 3- and 5-year RCC-related survival were 100%, 93.3% and 93.3%, respectively. • The estimated 1-, 3- and 5-year overall survival were 97.1%, 87.8%, 83.6%, respectively. • For patients with RCC ≤4 cm, initial ablation success was 100% (29/29) and the estimated 5-year disease-free survival were 81.5%. - Abstract: Purpose: To evaluate the efficacy and safety of ultrasound (US)-guided percutaneous microwave ablation (MWA) for renal cell carcinoma (RCC) adjacent to renal sinus. Materials and methods: This retrospective study included 41 patients who underwent US-guided percutaneous MWA of 41 RCCs adjacent to the renal sinus from April 2006 to December 2015. Contrast-enhanced images of US and computed tomography (CT) or magnetic resonance (MR) imaging were performed at pre-ablation and 1 day, 1 month, 3 months, and every 6 months after ablation. Initial ablation success (IAS), disease-free survival (DFS), RCC-related survival (RRS), and overall survival (OS) were recorded at the follow-up visits. Results: IAS was achieved in 92.7% (38/41) of the study subjects. The IAS significantly differed between patients with RCCs ≤4 cm (100%, 29/29) and RCCs >4 cm (75%, 9/12, p = 0.021). During the median follow-up of 37.6 (range, 3.0–97.3) months, the estimated 1-, 3-, and 5-year DFS of patients with an initial tumor of ≤4 cm were 100%, 89.7%, and 81.5%, respectively. The 1-, 3-, and 5-year RRS were 100%, 93.3%, and 93.3%, respectively. The 1-, 3-, and 5-year OS were 97.1%, 87.8%, and 83.6%, respectively. The multivariate analysis using the Cox proportional hazard model revealed no independent predictor of recurrence among all the variables. There were no MWA-related deaths among the study subjects. One patient developed a retroperitoneal abscess after ablation. Conclusion: US-guided percutaneous MWA

  11. Immunotherapy of renal cell carcinoma.

    Science.gov (United States)

    Gouttefangeas, Cécile; Stenzl, Arnulf; Stevanović, Stefan; Rammensee, Hans-Georg

    2007-01-01

    Carcinomas of the kidney generally have a poor prognosis and respond minimally to classical radiotherapy or chemotherapy. Immunotherapy constitutes an interesting alternative to these established forms of treatment, and indeed, cytokine-based therapies have been used for many years, leading to favorable clinical responses in a small subset of patients. During the past few years, immunotherapeutical trials targeting renal cell tumor-associated antigens have also been reported, with diverse passive or active approaches using antibodies or aimed at activating tumor-directed T lymphocytes. The following review presents the results and the progress made in the field, including classical cytokine treatments, non-myeloablative stem cell transplantation and antigen specific-based trials, with special focus on T-cell studies. In consideration of the few specific molecular targets described so far for this tumor entity, current strategies which can lead to the identification of new relevant antigens will be discussed. Hopefully these will very soon contribute to an improvement in renal cell carcinoma specific immunotherapy and its evaluation.

  12. Mutations of codon 918 in the RET proto-oncogene correlate to poor prognosis in sporadic medullary thyroid carcinomas

    Energy Technology Data Exchange (ETDEWEB)

    Zedenius, J.; Svensson, A.; Baeckdahl, M.; Wallin, G. [Karolinska Hospital, Stockholm (Sweden)] [and others

    1995-10-01

    The hereditary multiple endocrine neoplasia syndromes types 2A and B (MEN 2A and B) were recently linked to germline mutations in the RET proto-oncogene, altering one of five cysteine residues in exon 10 or 11 (MEN 2A), or substituting a methionine for a threonine at codon 918 in exon 16 (MEN 2B). The latter mutation also occurs somatically in some sporadic medullary thyroid carcinomas (MTC), and has in a previous study been correlated with a less favorable clinical outcome. In the present study, 46 MTCs were selected for investigation of the codon 918 mutation. The mutation was found in 29 tumors (63%), and was significantly correlated with a poor outcome, with regard to distant metastasis or tumor recurrence (p<10{sup 4}). Two tumors showed multifocal growth and C-cell hyperplasia, and these patients were therefore also investigated for germline mutations in exons 10, 11 and 16. The codon 918 mutation was found only in the tumors, thus of somatic origin. The RET codon 918 mutation may have prognostic impact, and therefore preoperative assessment may influence decision-making in the treatment of patients suffering from MTC. 13 refs., 1 fig., 1 tab.

  13. Alternaria infectoria phaeohyphomycosis in a renal transplant patient.

    NARCIS (Netherlands)

    Nulens, E.; Laere, E. De; Vandevelde, H.; Hilbrands, L.B.; Rijs, A.J.M.M.; Melchers, W.J.G.; Verweij, P.E.

    2006-01-01

    A male renal transplant patient developed a tumor on the dorsum of his right hand. After excision, histological examination of the tumor showed hyphal structures, but growth developed very slowly. Therapy consisted of surgery alone. A definitive identification of Alternaria infectoria was only

  14. PRIMARY RENAL MASSES IN CHILDREN IN CAMEROON: A PLEA ...

    African Journals Online (AJOL)

    Fifteen of 21 patients (71.4%) with nephro-blastoma survived past 5 years. Conclusion While nephroblastoma is the most common tumor cell type, lymphomas and adenocarcinomas occur in over 20% of children with renal tumors. Therefore, prior to chemotherapy and radiotherapy, it is imperative to make a histologic ...

  15. Metastatic renal cell carcinoma from a native kidney of a renal transplant patient diagnosed by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA biopsy

    Directory of Open Access Journals (Sweden)

    Yaseen Alastal

    2015-04-01

    Full Text Available Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA biopsy sampling of enlarged lymph nodes is increasingly used to diagnose metastatic tumors, especially of the gastrointestinal tract and the lungs. Herein, we describe the diagnosis of metastatic renal cell carcinoma from a native kidney of a 54 year-old male patient, who had a 5-years history of renal transplant, by EUS-FNA of mediastinal and celiac lymph nodes. Histological and immunohistochemical findings confirmed the origin of metastatic tumor. EUS-FNA with proper cytological evaluation can be useful in the diagnosis of metastatic renal cell carcinoma in renal transplant patients. 

  16. The Prognostic Impact of p53 Expression on Sporadic Colorectal Cancer Is Dependent on p21 Status

    International Nuclear Information System (INIS)

    Kruschewski, Martin; Mueller, Kathrin; Lipka, Sybille; Budczies, Jan; Noske, Aurelia; Buhr, Heinz Johannes; Elezkurtaj, Sefer

    2011-01-01

    The prognostic value of p53 and p21 expression in colorectal cancer is still under debate. We hypothesize that the prognostic impact of p53 expression is dependent on p21 status. The expression of p53 and p21 was immunohistochemically investigated in a prospective cohort of 116 patients with UICC stage II and III sporadic colorectal cancer. The results were correlated with overall and recurrence-free survival. The mean observation period was 51.8 ± 2.5 months. Expression of p53 was observed in 72 tumors (63%). Overall survival was significantly better in patients with p53-positive carcinomas than in those without p53 expression (p = 0.048). No differences were found in recurrence-free survival (p = 0.161). The p53+/p21− combination was seen in 68% (n = 49), the p53+/p21+ combination in 32% (n = 23). Patients with p53+/p21− carcinomas had significantly better overall and recurrence-free survival than those with p53+/p21+ (p < 0.0001 resp. p = 0.003). Our data suggest that the prognostic impact of p53 expression on sporadic colorectal cancer is dependent on p21 status

  17. Linfoma renal: espectro de imagens na tomografia computadorizada Renal lymphoma: spectrum of computed tomography findings

    Directory of Open Access Journals (Sweden)

    Carol Pontes de Miranda Maranhão

    2005-04-01

    Full Text Available O acometimento renal no linfoma é raro, uma vez que o rim não possui tecido linfóide. O envolvimento secundário é mais freqüente, ocorrendo em até um terço das autópsias dos portadores de linfoma. Alguns autores acreditam que esta seja a única forma de acometimento renal, questionando a existência do linfoma primário. O linfoma renal representaria metástases hematogênicas ou invasão direta do tumor ocorrendo no espaço perirrenal. A partir destes tipos de envolvimento decorrem as formas de apresentação do linfoma renal: múltiplos nódulos, massa solitária, invasão renal por doença retroperitoneal contígua, doença perirrenal e infiltração difusa. Neste trabalho são discutidas e apresentadas imagens destas diferentes formas de acometimento.Isolated renal lymphoma is rare due to the absence of lymphoid tissue in kidneys. Secondary involvement occurs more frequently and is reported in up to 1/3 of the autopsies of patients who died from lymphoma. Some authors believe this is actually the only existing form of renal lymphoma. The involvement of the kidney by lymphoma would occur through hematogenic metastasis or direct tumor invasion of the perirenal space. These different types of involvement determine the several forms of renal lymphoma presentation: multiple nodules, solitary mass, renal invasion from contiguous retroperitoneal disease, perirenal disease and diffuse infiltration. In this study the imaging findings features of the different forms of involvement are presented and discussed.

  18. Effects of radiofrequency ablation on individual renal function. Assessment by technetium-99m mercaptoacetyltriglycine renal scintigraphy

    International Nuclear Information System (INIS)

    Mukai, Takashi; Sato, Shuhei; Iguchi, Toshihiro

    2006-01-01

    We quantitatively evaluated total and individual renal function by technetium-99m mercaptoacetyltriglycine (Tc-99m MAG3) renal scintigraphy before and after radiofrequency ablation (RFA) of renal tumors. Eleven patients who underwent Tc-99m MAG3 renal scintigraphy 1 week before and after RFA were evaluated (7 men and 4 women; age range: 23-83 years; mean age: 60.6 years). Five patients had solitary kidneys, and five had normally or minimally functioning contralateral kidneys. One patient had a renal cell carcinoma in the contralateral kidney. One patient with a solitary kidney underwent RFA a second time for a residual tumor. In patients with a solitary kidney, MAG3 clearance decreased after 5 of 6 RFAs, and in patients with a normally functioning contralateral kidney, MAG3 clearance decreased after 4 of 5 REAs, but no significant differences were observed between before and after treatments. In addition to the total MAG3 clearance, the split MAG3 clearance was evaluated in patients with a normally functioning contralateral kidney. MAG3 clearance decreased in 4 of 5 treated kidneys, while it adversely increased in the contralateral kidneys after 4 of 5 RFAs. No significant differences, however, were observed between before and after treatments. The results of our study revealed no significant differences in sCr, BUN, CCr, or MAG3 clearance between pre- and post-RFA values. These results support data regarding the functional impact and safety of renal RFA in published reports. We evaluated total and individual renal function quantitatively using Tc-99m MAG3 renal scintigraphy before and after treatment. This scintigraphy was very useful in assessing the effects of RFA on renal function. (author)

  19. Sporadic naturally occurring melanoma in dogs as a preclinical model for human melanoma.

    Science.gov (United States)

    Simpson, R Mark; Bastian, Boris C; Michael, Helen T; Webster, Joshua D; Prasad, Manju L; Conway, Catherine M; Prieto, Victor M; Gary, Joy M; Goldschmidt, Michael H; Esplin, D Glen; Smedley, Rebecca C; Piris, Adriano; Meuten, Donald J; Kiupel, Matti; Lee, Chyi-Chia R; Ward, Jerrold M; Dwyer, Jennifer E; Davis, Barbara J; Anver, Miriam R; Molinolo, Alfredo A; Hoover, Shelley B; Rodriguez-Canales, Jaime; Hewitt, Stephen M

    2014-01-01

    Melanoma represents a significant malignancy in humans and dogs. Different from genetically engineered models, sporadic canine melanocytic neoplasms share several characteristics with human disease that could make dogs a more relevant preclinical model. Canine melanomas rarely arise in sun-exposed sites. Most occur in the oral cavity, with a subset having intra-epithelial malignant melanocytes mimicking the in situ component of human mucosal melanoma. The spectrum of canine melanocytic neoplasia includes benign lesions with some analogy to nevi, as well as invasive primary melanoma, and widespread metastasis. Growing evidence of distinct subtypes in humans, differing in somatic and predisposing germ-line genetic alterations, cell of origin, epidemiology, relationship to ultraviolet radiation and progression from benign to malignant tumors, may also exist in dogs. Canine and human mucosal melanomas appear to harbor BRAF, NRAS, and c-kit mutations uncommonly, compared with human cutaneous melanomas, although both species share AKT and MAPK signaling activation. We conclude that there is significant overlap in the clinical and histopathological features of canine and human mucosal melanomas. This represents opportunity to explore canine oral cavity melanoma as a preclinical model. © 2013 The Authors. Pigment Cell & Melanoma Research published by John Wiley & Sons Ltd.

  20. SMARCB1/INI1 germline mutations contribute to 10% of sporadic schwannomatosis

    Directory of Open Access Journals (Sweden)

    Bourdon Violaine

    2011-01-01

    Full Text Available Abstract Background Schwannomatosis is a disease characterized by multiple non-vestibular schwannomas. Although biallelic NF2 mutations are found in schwannomas, no germ line event is detected in schwannomatosis patients. In contrast, germline mutations of the SMARCB1 (INI1 tumor suppressor gene were described in familial and sporadic schwannomatosis patients. Methods To delineate the SMARCB1 gene contribution, the nine coding exons were sequenced in a series of 56 patients affected with a variable number of non-vestibular schwannomas. Results Nine variants scattered along the sequence of SMARCB1 were identified. Five of them were classified as deleterious. All five patients carrying a SMARCB1 mutation had more multiple schwannomas, corresponding to 10.2% of patients with schwannomatosis. They were also diagnosed before 35 years of age. Conclusions These results suggest that patients with schwannomas have a significant probability of carrying a SMARCB1 mutation. Combined with data available from other studies, they confirm the clinical indications for genetic screening of the SMARCB1 gene.

  1. SMARCB1/INI1 germline mutations contribute to 10% of sporadic schwannomatosis.

    Science.gov (United States)

    Rousseau, Guillaume; Noguchi, Tetsuro; Bourdon, Violaine; Sobol, Hagay; Olschwang, Sylviane

    2011-01-24

    Schwannomatosis is a disease characterized by multiple non-vestibular schwannomas. Although biallelic NF2 mutations are found in schwannomas, no germ line event is detected in schwannomatosis patients. In contrast, germline mutations of the SMARCB1 (INI1) tumor suppressor gene were described in familial and sporadic schwannomatosis patients. To delineate the SMARCB1 gene contribution, the nine coding exons were sequenced in a series of 56 patients affected with a variable number of non-vestibular schwannomas. Nine variants scattered along the sequence of SMARCB1 were identified. Five of them were classified as deleterious. All five patients carrying a SMARCB1 mutation had more multiple schwannomas, corresponding to 10.2% of patients with schwannomatosis. They were also diagnosed before 35 years of age. These results suggest that patients with schwannomas have a significant probability of carrying a SMARCB1 mutation. Combined with data available from other studies, they confirm the clinical indications for genetic screening of the SMARCB1 gene.

  2. Prokineticin 1 protein expression is a useful new prognostic factor for human sporadic colorectal cancer.

    Science.gov (United States)

    Nakazawa, Toshiyuki; Goi, Takanori; Hirono, Yasuo; Yamaguchi, Akio

    2015-05-01

    Hematogenous metastasis, regarded as closely related to angiogenic growth factors, is associated with colorectal cancer prognosis. The angiogenic growth factor prokineticin 1 (PROK1) has been cloned from endocrine cells. However, its protein expression in human malignant tumors has not been studied. The current study established the anti-PROK1 monoclonal antibody (mAb) and examined the relationship between the expression of PROK1 protein and human colorectal cancer. The expression of PROK1 protein was assessed in 620 resected sporadic colorectal cancer tissue samples by immunohistochemical staining with in-house-developed human PROK1 mAb to investigate the relationship of PROK1 expression to clinicopathologic factors, recurrence, and survival rate and to evaluate its prognostic significance. The expression of PROK1 protein was detected in 36 % (223/620) of human primary colorectal cancer lesions but no in the healthy mucosa adjacent to the colorectal cancer lesions. According to the clinicopathologic examinations, the frequency of positive PROK1 expression was significantly higher in cases with serosal invasion, lymphatic invasion, venous invasion, lymph node metastasis, liver metastasis, hematogenous metastasis, and higher stage disease. The recurrence rate and prognosis for patients with PROK1 expression-positive lesions were significantly worse. In the Cox proportional hazard model, PROK1 expression was an independent prognostic factor. The expression of PROK1 protein was identified for the first time as a new prognostic factor in colorectal cancer.

  3. Percutaneous and laparoscopic assisted cryoablation of small renal cell carcinomas

    DEFF Research Database (Denmark)

    Nielsen, Tommy Kjærgaard; Østraat, Øyvind; Borre, Michael

    Aim: To evaluate the complication rate and short term oncological outcome of small renal cell carcinomas treated with cryoablation. Materials and methods: 91 biopsy verified renal cell carcinomas were cryoablated between 2006-11. Patients treated had primarily T1a tumors, but exceptions were made...... Medical® was used. Treatment was considered successful when tumors gradually shrunk and showed no sign of contrast enhancement, assessed by CT or MRI. Results: Mean patient age and tumor size was 65 yr [17 - 83] and 26 mm [10 - 62], respectively [min-max]. Treatment modalities consisted of percutaneous...

  4. A Unifying Hypothesis for Familial and Sporadic Alzheimer's Disease

    Directory of Open Access Journals (Sweden)

    Carole J. Proctor

    2012-01-01

    Full Text Available Alzheimer's disease (AD is characterised by the aggregation of two quite different proteins, namely, amyloid-beta (Aβ, which forms extracellular plaques, and tau, the main component of cytoplasmic neurofibrillary tangles. The amyloid hypothesis proposes that Aβ plaques precede tangle formation but there is still much controversy concerning the order of events and the linkage between Aβ and tau alterations is still unknown. Mathematical modelling has become an essential tool for generating and evaluating hypotheses involving complex systems. We have therefore used this approach to discover the most probable pathway linking Aβ and tau. The model supports a complex pathway linking Aβ and tau via GSK3β, p53, and oxidative stress. Importantly, the pathway contains a cycle with multiple points of entry