Outcome of pregnancy in survivors of Wilms' tumor

International Nuclear Information System (INIS)

Li, F.P.; Gimbrere, K.; Gelber, R.D.; Sallan, S.E.; Flamant, F.; Green, D.M.; Heyn, R.M.; Meadows, A.T.

1987-01-01

Outcome of pregnancy was reported by 99 patients who were cured of childhood Wilms' tumor at seven pediatric cancer centers during 1931 to 1979. These patients carried or sired 191 singleton pregnancies of at least 20 weeks in duration. Among the 114 pregnancies in women who had received abdominal radiotherapy for Wilms' tumor, an adverse outcome occurred in 34 (30%). There were 17 perinatal deaths (five in premature low-birth-weight infants) and 17 other low-birth-weight infants. Compared with white women in the United States, the irradiated women had an increased perinatal mortality rate (relative risk, 7.9) and an excess of low-birth-weight infants (relative risk, 4.0). In contrast, an adverse outcome was found in two (3%) of the 77 pregnancies in nonirradiated female patients with Wilms' tumor and wives of male patients. The high risk of adverse pregnancy outcome should be considered in the counseling and prenatal care of women who have received abdominal radiotherapy for Wilms' tumor

Biomarkers for Wilms Tumor: a Systematic Review

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Cone, Eugene B.; Dalton, Stewart S.; Van Noord, Megan; Tracy, Elizabeth T.; Rice, Henry E.; Routh, Jonathan C.

2016-01-01

Purpose Wilms tumor is the most common childhood renal malignancy and the fourth most common childhood cancer. Many biomarkers have been studied but there has been no comprehensive summary. We systematically reviewed the literature on biomarkers in Wilms Tumor with the objective of quantifying the prognostic implication of the presence of individual tumor markers. Methods We searched for English language studies from 1980–2015 performed on children with Wilms Tumor under 18 years old with prognostic data. The protocol was conducted as per PRISMA guidelines. Two reviewers abstracted data in duplicate using a standard evaluation form. We performed descriptive statistics, then calculated relative risks and 95% confidence intervals for markers appearing in multiple level 2 or 3 studies. Results 40 studies were included examining 32 biomarkers in 7381 Wilms patients. Studies had a median of 61 patients with 24 biomarker positive patients per study, and a median follow-up of 68.4 months. Median percent of patients in Stage 1, 2, 3, 4, and 5 were 28.5%, 26.4%, 24.5%, 14.1%, and 1.7%, with 10.2% anaplasia. The strongest negative prognostic association was loss of heterozygosity on 11p15, with a risk of recurrence of 5.00, although loss of heterozygosity on 1p and gain of function on 1q were also strongly linked to increased recurrence (2.93 and 2.86 respectively). Conclusions Several tumor markers are associated with an increased risk of recurrence or a decreased risk of overall survival in Wilms Tumor. These data suggest targets for development of diagnostic tests and potential therapies. PMID:27259655

Wilms tumor in a child with trisomy 13.

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Sweeney, H; Pelegano, J

2000-01-01

A 4-year-old black boy with trisomy 13, a history of frequent urinary tract infections, and a horseshoe kidney with painless gross hematuria was examined. An abdominal mass was detected and surgically resected. Examination of the surgical specimen revealed a Wilms tumor. Given the concurrence of trisomy 13 and Wilms tumor and the presence of another such case in the literature, there may be just cause to suspect a locus on chromosome 13 that affects the probability of developing Wilms tumor. Given the increasingly longer survival of patients with trisomy 13, clinicians may need to be aware of the possibility of renal malignant disease in this population of patients.

Radiological diagnostics and radiotherapy in Wilms' tumor

Energy Technology Data Exchange (ETDEWEB)

Kutzner, J

1981-01-01

The possibilities of diagnosing Wilms' tumor correctly have been greatly extended by the introduction of computerised tomography and ultrasonic examination. In view of the fact that Wilms' tumor is subjected to combined treatment involving chemotherapy, surgery and radiotherapy, it appears justified to reduce the dose to 20-30 Gy, depending upon the age of the child and the extension of the tumor. It is believed that preoperative radiotherapy will yield better surgical possibilities in large tumours. Radiotherapy can be omitted in infants in the stages I and II as well as in children in stage I.

Treatment-independent miRNA signature in blood of wilms tumor patients

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Schmitt Jana

2012-08-01

Full Text Available Abstract Background Blood-born miRNA signatures have recently been reported for various tumor diseases. Here, we compared the miRNA signature in Wilms tumor patients prior and after preoperative chemotherapy according to SIOP protocol 2001. Results We did not find a significant difference between miRNA signature of both groups. However both, Wilms tumor patients prior and after chemotherapy showed a miRNA signature different from healthy controls. The signature of Wilms tumor patients prior to chemotherapy showed an accuracy of 97.5% and of patients after chemotherapy an accuracy of 97.0%, each as compared to healthy controls. Conclusion Our results provide evidence for a blood-born Wilms tumor miRNA signature largely independent of four weeks preoperative chemotherapy treatment.

Lin28 sustains early renal progenitors and induces Wilms tumor

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Urbach, Achia; Yermalovich, Alena; Zhang, Jin; Spina, Catherine S.; Zhu, Hao; Perez-Atayde, Antonio R.; Shukrun, Rachel; Charlton, Jocelyn; Sebire, Neil; Mifsud, William; Dekel, Benjamin; Pritchard-Jones, Kathy; Daley, George Q.

2014-01-01

Wilms Tumor, the most common pediatric kidney cancer, evolves from the failure of terminal differentiation of the embryonic kidney. Here we show that overexpression of the heterochronic regulator Lin28 during kidney development in mice markedly expands nephrogenic progenitors by blocking their final wave of differentiation, ultimately resulting in a pathology highly reminiscent of Wilms tumor. Using lineage-specific promoters to target Lin28 to specific cell types, we observed Wilms tumor only when Lin28 is aberrantly expressed in multiple derivatives of the intermediate mesoderm, implicating the cell of origin as a multipotential renal progenitor. We show that withdrawal of Lin28 expression reverts tumorigenesis and markedly expands the numbers of glomerulus-like structures and that tumor formation is suppressed by enforced expression of Let-7 microRNA. Finally, we demonstrate overexpression of the LIN28B paralog in a significant percentage of human Wilms tumor. Our data thus implicate the Lin28/Let-7 pathway in kidney development and tumorigenesis. PMID:24732380

Tumor de Wilms extrarrenal: Un caso inusual Extrarenal Wilm's tumor: An unusual case

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Caridad Verdecia Cañizares

2004-09-01

Full Text Available El tumor de Wilms extrarrenal es extremadamente raro en la infancia. Se reporta el caso de una niña de 4 años de edad a la que se le realizó diagnóstico en nuestro hospital de esta variante hística de localización retroperitoneal, con buena evaluación posoperatoria, y fue el objetivo dar a conocer esta localización inusual y el valor de la biopsia espirativa con aguja fina en el diagnóstico de esta afección.Extrarenal Wilms' tumor is extremely rare in children. It is reported the case of a 4-year-old girl that was diagnosed this histic variant of retroperitoneal localization in our hospital with a good postoperative evaluation. The objective of this paper was to make known this unsual localization and the value ot the fine needle aspiration biopsy in the diagnosis of this affection.

Drugs Approved for Wilms Tumor

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This page lists cancer drugs approved by the Food and Drug Administration (FDA) for Wilms tumor and other childhood kidney cancers. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

Innovations in the management of Wilms' tumor.

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Gleason, Joseph M; Lorenzo, Armando J; Bowlin, Paul R; Koyle, Martin A

2014-08-01

Advances in the management of Wilms' tumor have been dramatic over the past half century, not in small part due to the institution of multimodal therapy and the formation of collaborative study groups. While different opinions exist in the management of Wilms' tumors depending on where one lives and practices, survival rates have surpassed 90% across the board in Western societies. With more children surviving into adulthood, the concerns about morbidity have reached the forefront and now represent as much a consideration as oncologic outcomes these days. Innovations in treatment are on the horizon in the form of potential tumor markers, molecular biological means of testing for chemotherapeutic responsiveness, and advances in the delivery of chemotherapy for recurrent or recalcitrant tumors. Other technological innovations are being applied to childhood renal tumors, such as minimally invasive and nephron-sparing approaches. Risk stratification also allows for children to forego potentially unnecessary treatments and their associated morbidities. Wilms' tumor stands as a great example of the gains that can be made through protocol-driven therapy with strenuous outcomes analyses. These gains continue to spark interest in minimization of morbidity, while avoiding any compromise in oncologic efficacy. While excitement and innovation are important in the advancement of treatment delivery, we must continue to temper this enthusiasm and carefully evaluate options in order to continue to provide the highest standard of care in the management of this now highly curable disease.

Immunohistochemical Expression of Ki67 and p53 in Wilms Tumor and Its Relationship with Tumor Histology and Stage at Presentation

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Krishna, O. H. Radhika; Kayla, Geetha; Abdul Aleem, Mohammed; Malleboyina, Ramani; Reddy Kota, Ramesh

2016-01-01

Aim. Evaluate tumor proliferation marker (Ki67) and p53 tumor suppressor marker in Wilms tumor and correlate with histology, anaplasia, and staging. Design. Prospective, hospital based study conducted at a tertiary pediatric referral centre in south India. Setting. Wilms tumor is the most common childhood renal malignancy worldwide. Anaplasia on histology is associated with treatment resistance but not with aggressiveness clinical presentation. Chemotherapy for Wilms tumor is based on histology and staging. Most patients respond to current chemotherapy protocol. However, a small fraction relapses or metastasizes. Affordable prognostic markers are needed for histopathological evaluation of this tumor. Subjects. Cases of histologically confirmed Wilms tumor over five years. Cases after chemotherapy were excluded as the immunostaining was inconsistent in necrotic areas. Methods. The clinical and radiological findings of 31 cases of Wilms tumor were documented at a tertiary pediatric referral hospital over five years. In addition to Hematoxylin and Eosin staining, Ki67 proliferation index and p53 expression were correlated with tumor histology and staging. Results. Age incidence was 3–8 years with female preponderance. Significant correlation was noted between Ki67 proliferation index and tumor staging. p53 expression was not useful in stratification of Wilms tumor. Conclusion. Ki67 was cost-effective immunohistochemical marker for prognostication of pediatric Wilms tumor. PMID:26904359

Immunohistochemical Expression of Ki67 and p53 in Wilms Tumor and Its Relationship with Tumor Histology and Stage at Presentation

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O. H. Radhika Krishna

2016-01-01

Full Text Available Aim. Evaluate tumor proliferation marker (Ki67 and p53 tumor suppressor marker in Wilms tumor and correlate with histology, anaplasia, and staging. Design. Prospective, hospital based study conducted at a tertiary pediatric referral centre in south India. Setting. Wilms tumor is the most common childhood renal malignancy worldwide. Anaplasia on histology is associated with treatment resistance but not with aggressiveness clinical presentation. Chemotherapy for Wilms tumor is based on histology and staging. Most patients respond to current chemotherapy protocol. However, a small fraction relapses or metastasizes. Affordable prognostic markers are needed for histopathological evaluation of this tumor. Subjects. Cases of histologically confirmed Wilms tumor over five years. Cases after chemotherapy were excluded as the immunostaining was inconsistent in necrotic areas. Methods. The clinical and radiological findings of 31 cases of Wilms tumor were documented at a tertiary pediatric referral hospital over five years. In addition to Hematoxylin and Eosin staining, Ki67 proliferation index and p53 expression were correlated with tumor histology and staging. Results. Age incidence was 3–8 years with female preponderance. Significant correlation was noted between Ki67 proliferation index and tumor staging. p53 expression was not useful in stratification of Wilms tumor. Conclusion. Ki67 was cost-effective immunohistochemical marker for prognostication of pediatric Wilms tumor.

Wilms' tumor in adults: apropos of a case

International Nuclear Information System (INIS)

Izquierdo-Gonzalez, Marlen

2009-01-01

Wilms tumor accounts for 8% of solid tumors in children, making up over 80% of tumors genitourinary children under 15 years. In the United States of America reported 350 new cases each year and Cuba 14 a year, usually unilateral, but in 5-10% involvement is bilateral and also identifies cases extrarenal, but more isolated. The presentation of this tumor outside the pediatric age exceptional. But as recent cases reported in the literature. According to statistics reported in Europe and United States, its frequency is 0.2 cases per million adults. The aim of this study is to analyze the elements allowed the diagnosis of Wilms tumor in a patient adult of 43 years and the treatment he was subjected in Medical Surgical Research Center in Havana Cuba and clinical elements of its evolution and correlation with necropsy findings. (Author)

Wilms Tumor Treatment Outcomes: Perspectives From a Low-Income Setting

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Festus Njuguna

2017-10-01

Full Text Available Purpose: Wilms tumor is the commonest renal malignancy in childhood. Survival in high-income countries is approximately 90%, whereas in low-income countries, it is less than 50%. This study assessed treatment outcomes of patients with Wilms tumor at a Kenyan academic hospital. Patients and Methods: We conducted a retrospective medical record review of all children diagnosed with Wilms tumor between 2010 and 2012. Data on treatment outcomes and various sociodemographic and clinical characteristics were collected. Results: Of the 39 patients with Wilms tumor, 41% had event-free survival, 31% abandoned treatment, 23% died, and 5% had progressive or relapsed disease. Most patients presented at an advanced stage: stage I (0%, II (7%, III (43%, IV (40%, or V (10%. The most likely treatment outcome in patients with low-stage (I to III disease was event-free survival (67%, whereas in those with high-stage (IV to V disease, it was death (40%. No deaths or instances of progressive or relapsed disease were recorded among patients with low-stage disease; their only reason for treatment failure was abandonment of treatment. Stage of disease significantly affected treatment outcomes (P = .014 and event-free survival estimates (P < .001. Age at diagnosis, sex, duration of symptoms, distance to hospital, and health insurance status did not statistically significantly influence treatment outcomes or event-free survival estimates. Conclusion: Survival of patients with Wilms tumor in Kenya is lower compared with that in high-income countries. Treatment abandonment is the most common cause of treatment failure. Stage of disease at diagnosis statistically significantly affects treatment outcomes and survival.

Wilms Tumor Treatment Outcomes: Perspectives From a Low-Income Setting.

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Njuguna, Festus; Martijn, Hugo A; Kuremu, Robert Tenge; Saula, Peter; Kirtika, Patel; Olbara, Gilbert; Langat, Sandra; Martin, Steve; Skiles, Jodi; Vik, Terry; Kaspers, Gertjan J L; Mostert, Saskia

2017-10-01

Wilms tumor is the commonest renal malignancy in childhood. Survival in high-income countries is approximately 90%, whereas in low-income countries, it is less than 50%. This study assessed treatment outcomes of patients with Wilms tumor at a Kenyan academic hospital. We conducted a retrospective medical record review of all children diagnosed with Wilms tumor between 2010 and 2012. Data on treatment outcomes and various sociodemographic and clinical characteristics were collected. Of the 39 patients with Wilms tumor, 41% had event-free survival, 31% abandoned treatment, 23% died, and 5% had progressive or relapsed disease. Most patients presented at an advanced stage: stage I (0%), II (7%), III (43%), IV (40%), or V (10%). The most likely treatment outcome in patients with low-stage (I to III) disease was event-free survival (67%), whereas in those with high-stage (IV to V) disease, it was death (40%). No deaths or instances of progressive or relapsed disease were recorded among patients with low-stage disease; their only reason for treatment failure was abandonment of treatment. Stage of disease significantly affected treatment outcomes ( P = .014) and event-free survival estimates ( P abandonment is the most common cause of treatment failure. Stage of disease at diagnosis statistically significantly affects treatment outcomes and survival.

Transcript profiling of Wilms tumors reveals connections to kidney morphogenesis and expression patterns associated with anaplasia.

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Li, Wenliang; Kessler, Patricia; Williams, Bryan R G

2005-01-13

Anaplasia (unfavorable histology) is associated with therapy resistance and poor prognosis of Wilms tumor, but the molecular basis for this phenotype is unclear. Here, we used a cDNA array with 9240 clones relevant to cancer biology and/or kidney development to examine the expression profiles of 54 Wilms tumors, five normal kidneys and fetal kidney. By linking genes differentially expressed between fetal kidney and Wilms tumors to kidney morphogenesis, we found that genes expressed at a higher level in Wilms tumors tend to be expressed more in uninduced metanephrogenic mesenchyme or blastema than in their differentiated structures. Conversely, genes expressed at a lower level in Wilms tumors tend to be expressed less in uninduced metanephrogenic mesenchyme or blastema. We also identified 97 clones representing 76 Unigenes or unclustered ESTs that clearly separate anaplastic Wilms tumors from tumors with favorable histology. Genes in this set provide insight into the nature of the abnormal nuclear morphology of anaplastic tumors and may facilitate identification of molecular targets to improve their responsiveness to treatment.

Irinotecan for relapsed Wilms tumor in pediatric patients

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Hol, Janna A; van den Heuvel-Eibrink, Marry M; Graf, Norbert

2018-01-01

While irinotecan has been studied in various pediatric solid tumors, its potential role in Wilms tumor (WT) is less clear. We evaluated response and outcome of irinotecan-containing regimens in relapsed WT and compared our results to the available literature. Among 14 evaluable patients, one...

Nuclear medicine and ultrasonography in Wilms' tumor diagnosis

International Nuclear Information System (INIS)

Serson, D.; Donoso, M.C.P.; Bianchi, A.; Schmillevitch, J.; Antoneli, C.B.G.; Andrea, M.L.M. de; Petrilli, A.S.

1983-01-01

Renal scintigrams are analysed, as well as isotopic nephrograms and ultra-sound in 20 patients with Wilms' tumors. It is concluded that the methods above mentioned have great value to the study of the morphology and functional state of these renal tumors. (Author) [pt

Acute lymphoblastic leukemia mimicking Wilms tumor at presentation.

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Singh, Amitabh; Mandal, Anirban; Guru, Vijay; Seth, Rachna

2016-09-01

Acute lymphoblastic leukemia (ALL), the commonest malignancy of childhood, is known to manifest with a myriad of atypical presentations. Nephromegaly is a rare presentation of childhood ALL with hepatic mass being even rarer. We present a 3 year-old child with unilateral renal mass and hepatic mass lesion with normal blood counts, initially suspected to have metastatic Wilms tumor based on clinical, radiological and WT1 positivity on immunocytochemistry of renal mass. He was later diagnosed as ALL with peripheral blood flowcytometry and bone marrow examination. Renomegaly at presentation of acute leukemia is not necessarily due to leukemic infiltration and rarely leads to renal impairment. The radiological differential of such a renal mass includes both benign and malignant entities including metastasis. Over-expression of WT1 mRNA has been found in a number of solid tumors and hematological malignancies and is far from being diagnostic of Wilms tumor. Again, a small number of children with acute leukemia may have a deceptively normal complete blood count at presentation. Though, initial all (clinical, radiological, hematological, and immunocytological) parameters pointed towards a diagnosis of Wilms tumor in our case, the subsequent development of thrombocytopenia and lymphocytic leukocytosis prompted further investigation and final diagnosis of ALL. WT1 positivity is a known phenomenon in childhood ALL and undifferentiated lymphoblasts may be positive for WT1 and negative for Leucocyte common antigen. Acute leukemia with renal and hepatic mass with normal blood counts at presentation is a diagnostic challenge.

A Children's Oncology Group and TARGET initiative exploring the genetic landscape of Wilms tumor.

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Gadd, Samantha; Huff, Vicki; Walz, Amy L; Ooms, Ariadne H A G; Armstrong, Amy E; Gerhard, Daniela S; Smith, Malcolm A; Auvil, Jaime M Guidry; Meerzaman, Daoud; Chen, Qing-Rong; Hsu, Chih Hao; Yan, Chunhua; Nguyen, Cu; Hu, Ying; Hermida, Leandro C; Davidsen, Tanja; Gesuwan, Patee; Ma, Yussanne; Zong, Zusheng; Mungall, Andrew J; Moore, Richard A; Marra, Marco A; Dome, Jeffrey S; Mullighan, Charles G; Ma, Jing; Wheeler, David A; Hampton, Oliver A; Ross, Nicole; Gastier-Foster, Julie M; Arold, Stefan T; Perlman, Elizabeth J

2017-10-01

We performed genome-wide sequencing and analyzed mRNA and miRNA expression, DNA copy number, and DNA methylation in 117 Wilms tumors, followed by targeted sequencing of 651 Wilms tumors. In addition to genes previously implicated in Wilms tumors (WT1, CTNNB1, AMER1, DROSHA, DGCR8, XPO5, DICER1, SIX1, SIX2, MLLT1, MYCN, and TP53), we identified mutations in genes not previously recognized as recurrently involved in Wilms tumors, the most frequent being BCOR, BCORL1, NONO, MAX, COL6A3, ASXL1, MAP3K4, and ARID1A. DNA copy number changes resulted in recurrent 1q gain, MYCN amplification, LIN28B gain, and MIRLET7A loss. Unexpected germline variants involved PALB2 and CHEK2. Integrated analyses support two major classes of genetic changes that preserve the progenitor state and/or interrupt normal development.

A Children's Oncology Group and TARGET initiative exploring the genetic landscape of Wilms tumor

KAUST Repository

Gadd, Samantha

2017-08-21

We performed genome-wide sequencing and analyzed mRNA and miRNA expression, DNA copy number, and DNA methylation in 117 Wilms tumors, followed by targeted sequencing of 651 Wilms tumors. In addition to genes previously implicated in Wilms tumors (WT1, CTNNB1, AMER1, DROSHA, DGCR8, XPO5, DICER1, SIX1, SIX2, MLLT1, MYCN, and TP53), we identified mutations in genes not previously recognized as recurrently involved in Wilms tumors, the most frequent being BCOR, BCORL1, NONO, MAX, COL6A3, ASXL1, MAP3K4, and ARID1A. DNA copy number changes resulted in recurrent 1q gain, MYCN amplification, LIN28B gain, and MIRLET7A loss. Unexpected germline variants involved PALB2 and CHEK2. Integrated analyses support two major classes of genetic changes that preserve the progenitor state and/or interrupt normal development.

A Children's Oncology Group and TARGET initiative exploring the genetic landscape of Wilms tumor

KAUST Repository

Gadd, Samantha; Huff, Vicki; Walz, Amy L; Ooms, Ariadne H A G; Armstrong, Amy E; Gerhard, Daniela S; Smith, Malcolm A; Auvil, Jaime M Guidry; Meerzaman, Daoud; Chen, Qing-Rong; Hsu, Chih Hao; Yan, Chunhua; Nguyen, Cu; Hu, Ying; Hermida, Leandro C; Davidsen, Tanja; Gesuwan, Patee; Ma, Yussanne; Zong, Zusheng; Mungall, Andrew J; Moore, Richard A; Marra, Marco A; Dome, Jeffrey S; Mullighan, Charles G; Ma, Jing; Wheeler, David A; Hampton, Oliver A; Ross, Nicole; Gastier-Foster, Julie M; Arold, Stefan T.; Perlman, Elizabeth J

2017-01-01

We performed genome-wide sequencing and analyzed mRNA and miRNA expression, DNA copy number, and DNA methylation in 117 Wilms tumors, followed by targeted sequencing of 651 Wilms tumors. In addition to genes previously implicated in Wilms tumors (WT1, CTNNB1, AMER1, DROSHA, DGCR8, XPO5, DICER1, SIX1, SIX2, MLLT1, MYCN, and TP53), we identified mutations in genes not previously recognized as recurrently involved in Wilms tumors, the most frequent being BCOR, BCORL1, NONO, MAX, COL6A3, ASXL1, MAP3K4, and ARID1A. DNA copy number changes resulted in recurrent 1q gain, MYCN amplification, LIN28B gain, and MIRLET7A loss. Unexpected germline variants involved PALB2 and CHEK2. Integrated analyses support two major classes of genetic changes that preserve the progenitor state and/or interrupt normal development.

Focal versus diffuse anaplasia in Wilms tumor--new definitions with prognostic significance: a report from the National Wilms Tumor Study Group.

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Faria, P; Beckwith, J B; Mishra, K; Zuppan, C; Weeks, D A; Breslow, N; Green, D M

1996-08-01

Anaplasia, defined by the presence of extreme nuclear and mitotic atypia, is a potent marker of adverse prognosis in Wilms tumor (WT). Anaplastic WT cells apparently have increased resistance to therapy rather than increased aggressiveness. The distribution of anaplasia should therefore have critical prognostic relevance. The original definitions for focal anaplasia (FA) and diffuse anaplasia (DA) were based on quantitative rather than topographical criteria and lacked prognostic significance. A new definition was developed based on the distribution of anaplastic changes within the tumor: FA applies only to tumors with anaplasia confined to one or a few discrete loci within the primary tumor, with no anaplasia or marked nuclear atypia elsewhere. This revised definition was evaluated in 165 cases with anaplastic WT entered on the third and fourth National Wilms Tumor Study. Only three relapses and one death occurred among 39 cases with FA, regardless of tumor stage, a result comparable to that for nonanaplastic WT. Eight children with metastases at diagnosis and FA in the primary tumor were alive and free of relapse; 22 of 23 children with stage IV DA WT died of tumor. This new definition reinforces the importance of carefully documenting the exact site from which each tumor section is obtained.

Anaplasia and drug selection-independent overexpression of the multidrug resistance gene, MDR1, in Wilms' tumor.

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Re, G G; Willingham, M C; el Bahtimi, R; Brownlee, N A; Hazen-Martin, D J; Garvin, A J

1997-02-01

One reason for the failure of chemotherapy is the overexpression of the multidrug resistance gene, MDR1. The product of this gene is the multidrug transporter P-glycoprotein, an ATP-dependent pump that extrudes drugs from the cytoplasm. Some tumors inherently express P-glycoprotein, whereas others acquire the ability to do so after exposure to certain chemotherapeutic agents, often by the mechanism of gene amplification. Classical Wilms' tumors (nephroblastoma) typically respond to therapy and have a good prognosis. On the contrary, anaplastic Wilms' tumors are generally refractory to chemotherapy. These anaplastic variants are rare (4.5% of all Wilms' tumors reported in the United States), aggressive, and often fatal forms of tumor, which are commonly thought to result from the progression of classical Wilms' tumors. To investigate the basis for this differential response to therapy, we examined a number of classical and anaplastic Wilms' tumors for the expression of the MDR1 gene by immunohistochemical and mRNA analysis. Classical Wilms' tumors consistently did not express P-glycoprotein except in areas of tubular differentiation, as in normal kidney. Similarly, two of three anaplastic tumors failed to show P-glycoprotein expression. In contrast, cultured cells derived from a third anaplastic tumor, W4, exhibited strong P-glycoprotein expression and were drug resistant in vitro. Southern analysis revealed that W4 cells contained a single copy of the MDR1 gene per haploid genome similar to normal cells, demonstrating that the overexpression of MDR1 was not caused by gene amplification. Transcriptional activation of the MDR1 gene would be in keeping with the concept that p53 might act as a transcriptional repressor of the MDR1 gene.

A Children's Oncology Group and TARGET initiative exploring the genetic landscape of Wilms tumor. | Office of Cancer Genomics

Science.gov (United States)

We performed genome-wide sequencing and analyzed mRNA and miRNA expression, DNA copy number, and DNA methylation in 117 Wilms tumors, followed by targeted sequencing of 651 Wilms tumors. In addition to genes previously implicated in Wilms tumors (WT1, CTNNB1, AMER1, DROSHA, DGCR8, XPO5, DICER1, SIX1, SIX2, MLLT1, MYCN, and TP53), we identified mutations in genes not previously recognized as recurrently involved in Wilms tumors, the most frequent being BCOR, BCORL1, NONO, MAX, COL6A3, ASXL1, MAP3K4, and ARID1A.

Dyspnea, Tachycardia, and New Onset Seizure as a Presentation of Wilms Tumor: A Case Report

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Linda Li

2014-01-01

Full Text Available Wilms tumor is found in 1 in 10,000 children and most commonly presents in asymptomatic toddlers whose care givers notice a nontender abdominal mass in the right upper quadrant. This case of Wilms tumor presented as a critically ill eleven-year old with significant tachypnea, dyspnea, vague abdominal pain, intermittent emesis, new onset seizure, metabolic acidosis, and hypoxemia. This is the first case in the literature of Wilms Tumor with cavoatrial involvement and seizure and pulmonary embolism resulting in aggressive resuscitation and treatment. Treatment included anticoagulation, chemotherapy, nephrectomy, and surgical resection of thrombi, followed by adjunctive chemotherapy with pulmonary radiation.

Extrarenal retroperitoneal Wilms' tumor with subsequent pulmonary and peritoneal metastasis in a 4 year-old girl: A case report and review of literature

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Jinyoung Park

2016-05-01

Full Text Available This report describes an extremely rare case of extrarenal Wilms' tumor in a 4 year-old girl who presented with abdominal mass and pain. Computed tomography (CT scanning revealed a well-defined mass lesion measuring 10 cm on the right side of her lower abdomen and pelvic cavity. The mass was surgically removed. Histologically, the tumor showed a triphasic pattern, consisting of stromal, epithelial and blastemal components. Immunohistochemistry showed that the tumor was positive for cytokeratin, vimentin and CD99. The histopathological diagnosis was extrarenal Wilms' tumor arising in the retroperitoneum and inguinal canal. The patient was administered adjuvant chemoradiotherapy due to subsequent pulmonary and peritoneal metastases. Follow-up 4 years later showed that she was doing well, with no recurrence of the tumor.

Wilms tumor arising in extracoelomic paravertebral soft tissues.

LENUS (Irish Health Repository)

Mulligan, Linda

2012-02-01

Extrarenal Wilms tumor (ERWT) is a well-established entity which most commonly arises within the genitourinary tract, including intracoelomic paranephric soft tissue. Rarely, ERWT arises within teratoma, and it tends to occur predominantly in distinct settings, such as females with spinal defects and males with testicular teratomas. We report a unique ERWT arising within an extracoelomic teratoma of the paraspinal musculature, thereby expanding the range of reported locations for this unusual tumor.

Portal Hypertension in Children With Wilms' Tumor: A Report From the National Wilms' Tumor Study Group

International Nuclear Information System (INIS)

Warwick, Anne B.; Kalapurakal, John A.; Ou, San-San; Green, Daniel M.; Norkool, Pat A.; Peterson, Susan M.; Breslow, Norman E.

2010-01-01

Purpose: This analysis was undertaken to determine the cumulative risk of and risk factors for portal hypertension (PHTN) in patients with Wilms' tumor (WT). Methods and Materials: Medical records were reviewed to identify cases of PHTN identified with late liver/spleen/gastric toxicities in a cohort of 5,195 patients treated with National Wilms' Tumor Studies (NWTS) protocols 1 to 4. A nested case control study (5 controls/case) was conducted to determine relationships among doxorubicin, radiation therapy (RT) dose to the liver, patient gender, and PHTN. Conditional logistic regression was used to estimate adjusted hazard ratios (HR) of PHTN associated with these factors. Results: Cumulative risk of PHTN at 6 years from WT diagnosis was 0.7% for patients with right-sided tumors vs. 0.1% for those with left-sided tumors (p = 0.002). Seventeen of 19 cases were evaluable for RT. The majority of cases (16/17 [94%]) received right-flank RT either alone or as part of whole-abdomen RT and received >15 Gy to the liver. Fifteen of 17 (88%) patients received a higher dose to the liver than they would have with modern WT protocols. Controlling for RT dose, the HR was 3.0 for patients who received doxorubicin (p = 0.32) and 2.8 for females (p = 0.15). Controlling for doxorubicin, the 95% lower confidence bound on the HR associating PHTN with a minimum liver RT dose of >15 Gy vs. ≤15 Gy was 2.5 (p = 0.001); it was 2.4 for a maximum liver dose of >15 Gy vs. ≤15 Gy (p = 0.001). Conclusions: There was a strong association between higher doses of liver RT (>15 Gy) and the development of PHTN among WT patients.

Nuclear morphometry and prognosis in favorable histology Wilms' tumor: A prospective reevaluation.

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Breslow, N E; Partin, A W; Lee, B R; Guthrie, K A; Beckwith, J B; Green, D M

1999-07-01

This study was designed to evaluate the ability of a previously published nuclear morphometry discriminant function to predict disease-free survival in patients with Wilms' tumor. We identified 218 patients with stage I-IV Wilms' tumor of favorable histology who were entered onto the National Wilms' Tumor Study (NWTS) between January 1, 1990 and April 15, 1994. The nuclear morphometry score was calculated for each patient as follows: MV(f) = (0.02 x AGE) + (1.17 x SNRF) + (90.6 x LEFD) - 94, with AGE denoting age at diagnosis in months, SNRF the skewness of the nuclear roundness factor, and LEFD the lowest value of nuclear ellipticity as measured by the feret diameter method. Relative risks of relapse were estimated for the total score and for each of its components. Sensitivity and specificity were determined for the criterion of "MV(f) is greater than -0.35" as a predictor of relapse. By contrast with previously published results, neither the SNRF nor the LEFD made any contribution to the prediction of disease-free survival. Sensitivity and specificity of the criterion of "MV(f) is greater than -0.35" were 71% and 56%, respectively. Re-evaluation of a published nuclear morphometry score showed that it did not predict disease-free survival in patients with Wilms' tumor. The earlier study very likely overestimated the predictive power of nuclear morphometry by using the same data set both to develop the score and to evaluate its properties. Because of the huge number of combinations of nuclear morphometry measurements that may enter into the multivariate discriminant function, use of appropriate statistical methods is essential to estimate accurately the sensitivity and specificity.

Erroneously diagnosed Wilm's tumors in the SIOP material. An analysis of 21 radiological failures

International Nuclear Information System (INIS)

Ostrem, L.; Ehklef, U.; Ringertts, Kh.

1986-01-01

Available films of 21 cases of erroneously diagnosed Wilms' tumor in the European Wilms' material have been studied. The diagnosis has been reassessed and the reasons for agreement in 6 cases and disagreement in the remaining 15 are discussed. General diagnostic recommendations are given to help secure optimal diagnostic information

The Management of Synchronous Bilateral Wilms Tumor: A Report from the National Wilms Tumor Study Group

Science.gov (United States)

Hamilton, Thomas E.; Ritchey, Michael L.; Haase, Gerald M.; Argani, Pedram; Peterson, Susan M.; Anderson, James R.; Green, Daniel M.; Shamberger, Robert C.

2013-01-01

Objective To provide guidelines for future trials, we reviewed the outcomes of children with synchronous bilateral Wilms tumors (BWT) treated on National Wilms Tumor Study-4 (NWTS-4). Methods NWTS-4 enrolled 3,335 patients (pts) including 188 pts with BWT (5.6%). Treatment and outcome data were collected. Results Among 188 BWT pts registered with NWTS-4, 195 kidneys in 123 patients had initial open biopsy, 44 kidneys in 31 pts had needle biopsies. Although pre-resection chemotherapy was recommended, 87 kidneys in 83 pts were managed with primary resection: Complete nephrectomy 48 in 48 pts, 31 partial/wedge nephrectomies in 27 pts, enucleations 8 in 8 pts. No initial surgery was performed in 45 kidneys in 43 pts, 5 kidneys in 3 pts not coded. Anaplasia was diagnosed after completion of the initial course of chemotherapy in 14 pts (initial surgical procedure: 9 open biopsies, 4 needle biopsies, 1 partial nephrectomy). The average number of days from the start of chemotherapy to diagnosis of anaplasia was 390 (range 44–1,925 days). Relapse or progression of disease occurred in 54 children. End stage renal failure occurred in 23 children, 6 of whom had bilateral nephrectomies. The 8 year event free survival (EFS) for BWT with favorable histology was 74%, and overall survival (OS) was 89%; while the EFS for BWT with unfavorable histology was 40%, OS was 45%. Conclusion The current analysis of patients with BWT treated on NWTS-4 shows that preservation of renal parenchyma is possible in many pts following initial preoperative chemotherapy. The incidence of end-stage renal disease remains significantly higher in children with BWT. Future studies are warranted to address the need for earlier biopsy in non-responsive tumors and earlier definitive surgery to recognize unfavorable histology in these high risk patients. PMID:21394016

Cytogenetics and molecular genetics of Wilms' tumor of childhood

NARCIS (Netherlands)

Slater, R. M.; Mannens, M. M.

1992-01-01

We describe the way in which application of cytogenetic and molecular genetic techniques to the study of Wilms' tumor (WT) of the kidney and the associated congenital disorders, such as sporadic aniridia and the Beckwith-Wiedemann syndrome, has led to identification of two regions on the short arm

Radiation therapy for favorable histology Wilms tumor: Prevention of flank recurrence did not improve survival on National Wilms Tumor Studies 3 and 4

International Nuclear Information System (INIS)

Breslow, Norman E.; Beckwith, J. Bruce; Haase, Gerald M.; Kalapurakal, John A.; Ritchey, Michael L.; Shamberger, Robert C.; Thomas, Patrick; D'Angio, Giulio J.; Green, Daniel M.

2006-01-01

Purpose: To determine whether radiation therapy (RT) of patients with Wilms tumor of favorable histology prevented flank recurrence and thereby improved the survival outcomes. Methods and Materials: Recurrence and mortality risks were compared among groups of patients with Stage I-IV/favorable histology Wilms tumor enrolled in the third (n = 1,640) and fourth (n = 2,066) National Wilms Tumor Study Group studies. Results: Proportions of patients with flank recurrence were 0 of 513 = 0.0% for 20 Gy, 12 of 805 = 1.5% for 10 Gy, and 44 of 2,388 = 1.8% for no flank RT (p trend 0.001 adjusted for stage and doxorubicin); for intra-abdominal (including flank) recurrence they were 5 of 513 = 1.0%, 30 of 805 = 3.7%, and 58 of 2,388 = 2.4%, respectively (p trend = 0.02 adjusted). Survival percentages at 8 years after intra-abdominal recurrence were 0 of 5 = 0% for 20 Gy, 10 of 30 = 33% for 10 Gy, and 34 of 58 = 56% for no RT (p trend = 0.0001). NWTS-4 discontinued use of 20 Gy RT, and the 8-year flank recurrence risk increased to 2.1% from 1.0% on NWTS-3 (p = 0.013). However, event-free survival was unaltered (88% vs. 86%, p = 0.39), and overall survival was better (93.8% vs. 90.8%, p = 0.036) on NWTS-4. Conclusions: Partly because of lower postrecurrence mortality among nonirradiated patients, prevention of flank recurrence by RT did not improve survival. It is important to evaluate entire treatment policies with regard to long-term outcomes

Significance of TP53 mutation in Wilms tumors with diffuse anaplasia : A report from the Children's Oncology Group

NARCIS (Netherlands)

Ooms, Ariadne H A G; Gadd, Samantha; Gerhard, Daniela S.; Smith, Malcolm A.; Guidry Auvil, Jaime M.; Meerzaman, Daoud; Chen, Qing Rong; Hsu, Chih Hao; Yan, Chunhua; Nguyen, Cu; Hu, Ying; Ma, Yussanne; Zong, Zusheng; Mungall, Andrew J.; Moore, Richard A.; Marra, Marco A.; Huff, Vicki; Dome, Jeffrey S.; Chi, Yueh Yun; Tian, Jing; Geller, James I.; Mullighan, Charles G.; Ma, Jing; Wheeler, David A.; Hampton, Oliver A.; Walz, Amy L.; Van Den Heuvel-Eibrink, Marry M.; De Krijger, Ronald R.; Ross, Nicole; Gastier-Foster, Julie M.; Perlman, Elizabeth J.

2016-01-01

Purpose: To investigate the role and significance of TP53 mutation in diffusely anaplastic Wilms tumors (DAWTs). Experimental Design: All DAWTs registered on National Wilms Tumor Study-5 (n = 118) with available samples were analyzed for TP53 mutations and copy loss. Integrative genomic analysis was

Pattern, clincal presentation and management of Wilms' Tumor in ...

African Journals Online (AJOL)

Patients and Methods: This is a retrospective analysis of the hospital records of children with a diagnosis of Wilms' tumor treated from June 1996 to May 2005 at the ... have a dismal prognosis, with treatment outcomes at levels where it was before the advent of chemotherapy and radiation therapy in more advanced centers.

Glypican-3 mRNA expression level in Wilms tumor: correlation with histological type, stage, and outcome.

Science.gov (United States)

Wari, Md Nahidul; Vallonthaiel, Archana George; Ahmed, Aijaz; Saxena, Deepali; Iyer, Venkateswaran K; Mathur, Sandeep R; Agarwala, Sandeep; Bakhshi, Sameer; Srinivas, V; Chattopadhyaya, P; Sharma, Arundhati; Gupta, S Datta; Dinda, Amit

2017-06-01

To correlate expression of Glypican-3 in Wilms tumor with histopathology, stage, and outcome. Glypican-3 mRNA expression by real-time PCR on tumor and normal germline samples from 75 fresh nephrectomies for Wilms tumor with fold change after normalization against GAPDH was compared. Survival analysis for event-free and overall survival (EFS, OS) with 2-year follow-up for Glypican-3 overexpression (>1.5 times) and clinicopathological parameters was performed. Glypican-3 was overexpressed in 37/75 (49.3%). It was overexpressed in 77% (10/13) cases with blastema predominance or anaplastic histology, as compared to 44% of other histologies (27/62) (p = 0.03). OS was 73 and 93%, respectively (p = 0.016), for those with and without GPC-3 overexpression. EFS was not significantly different with Glypican-3 overexpression (p = 0.11). All 5 deaths among blastema predominant tumors and 4/5 deaths among triphasic tumors had overexpressed Glypican-3. Most deaths in Stage IV, Stage III, and Stage I + II (5/7, 3/3, 1/1) had GPC-3 overexpression. On multivariate analysis, only histology and stage were found to have independent prognostic value. Glypican-3 overexpression in Wilms tumor correlates with poor OS on univariate analysis. However, only histology and stage have independent prognostic value. Glypican-3 levels may help to stratify intermediate outcome histology (triphasic) and Stage III Wilms tumors.

[3D-visualization by MRI for surgical planning of Wilms tumors].

Science.gov (United States)

Schenk, J P; Waag, K-L; Graf, N; Wunsch, R; Jourdan, C; Behnisch, W; Tröger, J; Günther, P

2004-10-01

To improve surgical planning of kidney tumors in childhood (Wilms tumor, mesoblastic nephroma) after radiologic verification of the presumptive diagnosis with interactive colored 3D-animation in MRI. In 7 children (1 boy, 6 girls) with a mean age of 3 years (1 month to 11 years), the MRI database (DICOM) was processed with a raycasting-based 3D-volume-rendering software (VG Studio Max 1.1/Volume Graphics). The abdominal MRI-sequences (coronal STIR, coronal T1 TSE, transverse T1/T2 TSE, sagittal T2 TSE, transverse and coronal T1 TSE post contrast) were obtained with a 0.5T unit in 4 - 6 mm slices. Additionally, a phase-contrast-MR-angiography was applied to delineate the large abdominal and retroperitoneal vessels. A notebook was used to demonstrate the 3D-visualization for surgical planning before surgery and during the surgical procedure. In all 7 cases, the surgical approach was influenced by interactive 3D-animation and the information found useful for surgical planning. Above all, the 3D-visualization demonstrates the mass effect of the Wilms tumor and its anatomical relationship to the renal hilum and to the rest of the kidney as well as the topographic relationship of the tumor to the critical vessels. One rupture of the tumor capsule occurred as a surgical complication. For the surgeon, the transformation of the anatomical situation from MRI to the surgical situs has become much easier. For surgical planning of Wilms tumors, the 3D-visualization with 3D-animation of the situs helps to transfer important information from the pediatric radiologist to the pediatric surgeon and optimizes the surgical preparation. A reduction of complications is to be expected.

3D-visualization by MRI for surgical planning of Wilms tumors

International Nuclear Information System (INIS)

Schenk, J.P.; Wunsch, R.; Jourdan, C.; Troeger, J.; Waag, K.-L.; Guenther, P.; Graf, N.; Behnisch, W.

2004-01-01

Purpose: To improve surgical planning of kidney tumors in childhood (Wilms tumor, mesoblastic nephroma) after radiologic verification of the presumptive diagnosis with interactive colored 3D-animation in MRI. Materials and Methods: In 7 children (1 boy, 6 girls) with a mean age of 3 years (1 month to 11 years), the MRI database (DICOM) was processed with a raycasting-based 3D-volume-rendering software (VG Studio Max 1.1/Volume Graphics). The abdominal MRI-sequences (coronal STIR, coronal T1 TSE, transverse T1/T2 TSE, sagittal T2 TSE, transverse and coronal T1 TSE post contrast) were obtained with a 0.5T unit in 4-6 mm slices. Additionally, phase-contrast-MR-angiography was applied to delineate the large abdominal and retroperitoneal vessels. A notebook was used to demonstrate the 3D-visualization for surgical planning before surgery and during the surgical procedure. Results: In all 7 cases, the surgical approach was influenced by interactive 3D-animation and the information found useful for surgical planning. Above all, the 3D-visualization demonstrates the mass effect of the Wilms tumor and its anatomical relationship to the renal hilum and to the rest of the kidney as well as the topographic relationship of the tumor to the critical vessels. One rupture of the tumor capsule occurred as a surgical complication. For the surgeon, the transformation of the anatomical situation from MRI to the surgical situs has become much easier. Conclusion: For surgical planning of Wilms tumors, the 3D-visualization with 3D-animation of the situs helps to transfer important information from the pediatric radiologist to the pediatric surgeon and optimizes the surgical preparation. A reduction of complications is to be expected. (orig.)

Birth characteristics and Wilms tumors in children in the Nordic countries

DEFF Research Database (Denmark)

Schüz, Joachim; Schmidt, Lisbeth Samsø; Kogner, Per

2011-01-01

Little is known about causes of Wilms tumor. Because of the young age at diagnosis, several studies have looked at various birth characteristics. We conducted a registry-based case-control study involving 690 cases of Wilms tumor aged 0-14 years, occurring in Denmark, Finland, Norway or Sweden...... during 1985-2006, individually matched to five controls drawn randomly from the Nordic childhood population. Information on birth characteristics was obtained from the population-based medical birth registries. We estimated odds ratios (ORs) and 95% confidence intervals (CIs) using conditional logistic......-for-gestational age girls also had a higher risk (2.48, 1.51-4.05), whereas no effect was seen for boys (1.12, 0.60-2.07). An association was seen with Apgar score at 5 min birth order. In our large-scale, registry...

Local behavior and lymph node metastases of Wilms' tumor: accuracy of computed tomography; Comportamento local e metastases linfonodais do tumor de Wilms: acuracia da tomografia computadorizada

Energy Technology Data Exchange (ETDEWEB)

Silva, Eduardo Just da Costa e, E-mail: eduardojust@oi.com.br [Universidade Federal de Pernambuco (UFPE), Recife, PE (Brazil); Instituto Materno Infantil de Pernambuco (IMIP), Recife, PE (Brazil); Silva, Giselia Alves Pontes da [Universidade Federal de Pernambuco (UFPE), Recife, PE (Brazil). Dept. Maternal Infantil

2014-01-15

Objective: to evaluate the accuracy of computed tomography for local and lymph node staging of Wilms' tumor. Materials and methods: each case of Wilms' tumor was evaluated for the presence of abdominal lymph nodes by a radiologist. Signs of capsule and adjacent organ invasion were analyzed. Surgical and histopathological results were taken as the gold standard. Results: sensitivity was 100% for both mesenteric and retroperitoneal lymph nodes detection, and specificity was, respectively, 12% and 33%, with positive predictive value of 8% and 11% and negative predictive value of 100%. Signs of capsular invasion presented sensitivity of 87%, specificity of 77%, positive predictive value of 63% and negative predictive value of 93%. Signs of adjacent organ invasion presented sensitivity of 100%, specificity of 78%, positive predictive value of 37% and negative predictive value of 100%. Conclusion: computed tomography tumor showed low specificity and low positive predictive value in the detection of lymph node dissemination. The absence of detectable lymph nodes makes their presence unlikely, and likewise regarding the evaluation of local behavior of tumors. (author)

Btryoid Wilm's tumor in a child presenting with gross hematuria: A case report

Energy Technology Data Exchange (ETDEWEB)

Park, Chae Jung; Im, Young Jae; Shin, Hyun Joo; Kim, Myung Joon; Lee, Mi Jung [Severance Children' s Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of)

2016-09-15

We report a unique case of botryoid Wilms' tumor with its characteristic imaging findings in a 5-month-old boy presenting with gross hematuria. In our case, ultrasonography revealed lobulated hyperechoic lesions filling the pelvicalyceal system without parenchymal invasion, mimicking a blood clot. However, magnetic resonance imaging (MRI) demonstrated the exact extent of the lesion with diffusion restriction and delayed enhancement suggestive of a tumor. Despite their rarity, botryoid Wilms' tumors should be included in the differential diagnosis of lobulated renal pelvic lesions presenting as gross hematuria in children, and MRI can suggest the diagnosis.

Recurrent DGCR8, DROSHA, and SIX Homeodomain Mutations in Favorable Histology Wilms Tumors

NARCIS (Netherlands)

A.L. Walz (Amy L.); A.H.A.G. Ooms (Ariadne ); S. Gadd (Samantha); D.S. Gerhard (Daniela S.); M.A. Smith (Malcolm A.); J.M. GuidryAuvil (Jamie M.); D. Meerzaman (Daoud); Q.-R. Chen (Qing-Rong); C. Hsu (ChihHao); C. Yan (Chunhua); C. Nguyen (Cu); Y. Hu (Ying); R. Bowlby (Reanne); D. Brooks (Denise); Y. Ma (Yussanne); A.A. Mungall (Andrew J.); R.A. Moore (Richard A.); J. Schein (Jacqueline); M.A. Marra (Marco A.); V. Huff (Vicki); J.S. Dome (Jeffrey); Y.-Y. Chi (Yueh-Yun); C.G. Mullighan (Charles); J. Ma (Jing); D.A. Wheeler (David A.); O.A. Hampton (Oliver A.); N. Jafari (Nadereh); N. Ross (Nicole); J.M. Gastier-Foster (Julie); E.J. Perlman (Elizabeth J.)

2015-01-01

textabstractWe report the most common single-nucleotide substitution/deletion mutations in favorable histology Wilms tumors (FHWTs) to occur within SIX1/. 2 (7% of 534 tumors) and microRNA processing genes (miRNAPGs) DGCR8 and DROSHA (15% of 534 tumors). Comprehensive analysis of 77 FHWTs indicates

Visceral larval migrans masquerading as metastatic disease in a toddler with Wilms tumor

Energy Technology Data Exchange (ETDEWEB)

Anderson, Andrew; Fordham, Lynn Ansley; Bula, Melania L. [University of North Carolina School of Medicine, Department of Radiology, Chapel Hill, NC (United States); Blatt, Julie [University of North Carolina School of Medicine, Department of Pediatrics, Chapel Hill, NC (United States)

2006-03-15

A 22-month-old girl with a renal mass had multiple small pulmonary nodules on CT at her initial presentation. After biopsy and neoadjuvant chemotherapy, a Wilms tumor was resected and the pulmonary nodules were shown to have regressed on CT. Follow-up imaging 4 months after initial diagnosis demonstrated multiple new liver lesions and new pulmonary nodules with peripheral eosinophilia. Lung biopsy revealed granuloma formation with prominent eosinophils. The serum antibody titers for Toxocara canis were elevated. This case illustrates that toxocariasis should be considered as a rare differential diagnostic possibility for multiple liver lesions and multifocal peripheral pulmonary opacities in young children with Wilms tumor. (orig.)

Noncirrhotic portal fibrosis after Wilms' tumor therapy

International Nuclear Information System (INIS)

Barnard, J.A.; Marshall, G.S.; Neblett, W.W.; Gray, G.; Ghishan, F.K.

1986-01-01

A 9-yr-old girl developed massive hemorrhage from esophageal varices 2 yr after combined modality therapy for Wilms' tumor. Evaluation showed a patent extrahepatic portal venous system and an elevated splenic pulp pressure. In contrast to previous reports of hepatopathy after irradiation injury, histologic sections of the liver did not demonstrate occlusion of the central veins, but rather a diffuse obliteration of intrahepatic portal venous radicles. This pattern of noncirrhotic portal fibrosis has not been described following antitumor therapy

Surveillance Recommendations for Children with Overgrowth Syndromes and Predisposition to Wilms Tumors and Hepatoblastoma

NARCIS (Netherlands)

Kalish, Jennifer M.; Doros, Leslie; Helman, Lee J.; Hennekam, Raoul C.; Kuiper, Roland P.; Maas, Saskia M.; Maher, Eamonn R.; Nichols, Kim E.; Plon, Sharon E.; Porter, Christopher C.; Rednam, Surya; Schultz, Kris Ann P.; States, Lisa J.; Tomlinson, Gail E.; Zelley, Kristin; Druley, Todd E.

2017-01-01

A number of genetic syndromes have been linked to increased risk for Wilms tumor (WT), hepatoblastoma (HB), and other embryonal tumors. Here, we outline these rare syndromes with at least a 1% risk to develop these tumors and recommend uniform tumor screening recommendations for North America.

Evaluation of nuclear unrest and p53 immunostaining in Wilms' tumor.

Science.gov (United States)

Salama, Asmaa; Kamel, Ahmad

2011-03-01

Nuclear unrest is a term applied to Wilms' tumors (WT) that show nuclear abnormalities close to anaplasia but without abnormal mitoses. p53 is claimed to be associated with anaplasia and poor prognosis. This study was undertaken to evaluate the clinical significance of nuclear unrest and p53 immunostaining in Wilms' tumor. This is a retrospective study of 63 patients who presented at NCI with Wilms' tumors, and underwent preoperative chemotherapy followed by nephrectomy. Histopathologic assessment and p53 immunohistochemistry were done. WT with nuclear unrest grade III closely resembled anaplastic tumors and both of them (group 1) constituted 19% of cases. Group 1 constituted 29% of cases showing blastema dominant morphology compared to 9.4% of cases without blastema dominant morphology with significant statistical difference (p=0.047). Almost 83% of cases that achieved 1st complete remission were stages I, II and III, while 17% were stages IV and V with significant statistical difference (p<0.001). Stage affected the 3-year relapse-free-survival (RFS) significantly (p=0.014) as it was more in stages I, II and III than in stages IV and V (75.4% versus 50%). Blastema dominant morphology and high risk state significantly lowered the 3-year overall survival (OS) into 54.8% in comparison to 80.9% for cases with non-blastema dominant morphology (p=0.042). Regarding p53 immunohistochemistry, group 1 tumors showed positive p53 more than group 2 with significant statistical difference (p=0.014). p53 Positive immunostaining was significantly associated with high risk nephroblastoma (p=0.004). Tumor stage and blastema dominant morphology are potent prognostic factors. p53 is linked to blastema dominant morphology. WT with nuclear unrest grade III closely resembles anaplastic WT. It may be appropriate to group tumors with nuclear unrest grade III with anaplastic histology regarding treatment stratification. Copyright © 2011. Published by Elsevier B.V.

Evaluation of nuclear unrest and p53 immunostaining in Wilms' tumor

International Nuclear Information System (INIS)

Salama, A.; Kamel, A.

2011-01-01

Nuclear unrest is a term applied to Wilms' tumors (WT) that show nuclear abnormalities close to anaplasia but without abnormal mitoses. p53 is claimed to be associated with anaplasia and poor prognosis. This study was undertaken to evaluate the clinical significance of nuclear unrest and p53 immunostaining in Wilms' tumor. Material and methods: This is a retrospective study of 63 patients who presented at NCI with Wilms' tumors, and underwent preoperative chemotherapy followed by nephrectomy. Histopathologic assessment and p53 immunohistochemistry were done. Results: WT with nuclear unrest grade III closely resembled anaplastic tumors and both of them (group 1) constituted 19% of cases. Group 1 constituted 29% of cases showing blastema dominant morphology compared to 9.4% of cases without blastema dominant morphology with significant statistical difference (p = 0.047). Almost 83% of cases that achieved 1st complete remission were stages I, II and III, while 17% were stages IV and V with significant statistical difference (p < 0.001). Stage affected the 3-year relapse-free-survival (RFS) significantly (p = 0.014) as it was more in stages I, II and III than in stages IV and V (75.4% versus 50%). Blastema dominant morphology and high risk state significantly lowered the 3-year overall survival (OS) into 54.8% in comparison to 80.9% for cases with non-blastema dominant morphology (p = 0.042). Regarding p53 immunohistochemistry, group 1 tumors showed positive p53 more than group 2 with significant statistical difference (p = 0.014). p53 Positive immunostaining was significantly associated with high risk nephroblastoma (p = 0.004). Conclusion: Tumor stage and blastema dominant morphology are potent prognostic factors. p53 is linked to blastema dominant morphology. WT with nuclear unrest grade III closely resembles anaplastic WT. It may be appropriate to group tumors with nuclear unrest grade III with anaplastic histology regarding treatment stratification

Nephron sparing surgery (NSS) for unilateral wilms tumor (UWT): the SIOP 2001 experience

NARCIS (Netherlands)

Wilde, Jim C. H.; Aronson, Daniel C.; Sznajder, Beata; van Tinteren, Harm; Powis, Mark; Okoye, Bruce; Cecchetto, Giovanni; Audry, Georges; Fuchs, Jörg; Schweinitz, Dietrich Von; Heij, Hugo; Graf, Norbert; Bergeron, Christophe; Pritchard-Jones, Kathy; van den Heuvel-Eibrink, Marry; Carli, Modesto; Oldenburger, Foppe; Sandstedt, Bengt; de Kraker, Jan; Godzinski, Jan

2014-01-01

Total nephrectomy (TN) remains the standard treatment of unilateral Wilms tumors (uWT). The SIOP WT-2001 protocol allowed Nephron Sparing Surgery (NSS) for polar or peripherally non-infiltrating tumors. Inventory of the current SIOP NSS-experience. 2,800 patients with a unilateral, localized or

Optimal duration of preoperative therapy in unilateral and nonmetastatic Wilms' tumor in children older than 6 months: results of the Ninth International Society of Pediatric Oncology Wilms' Tumor Trial and Study

NARCIS (Netherlands)

Tournade, M. F.; Com-Nougué, C.; de Kraker, J.; Ludwig, R.; Rey, A.; Burgers, J. M.; Sandstedt, B.; Godzinski, J.; Carli, M.; Potter, R.; Zucker, J. M.

2001-01-01

To determine the optimal duration of preoperative chemotherapy to further increase the proportion of stage I tumors by comparison of two regimens in the treatment of patients older than 6 months who have unilateral Wilms' tumor. Eligible patients (n = 382) initially received four weekly doses of

EVALUATION OF LATE ADVERSE EVENTS IN LONG-TERM WILMS' TUMOR SURVIVORS

NARCIS (Netherlands)

van Dijk, Irma W. E. M.; Oldenburger, Foppe; Cardous-Ubbink, Mathilde C.; Geenen, Maud M.; Heinen, Richard C.; de Kraker, Jan; van Leeuwen, Flora E.; van der Pal, Helena J. H.; Caron, Huib N.; Koning, Caro C. E.; Kremer, Leontien C. M.

2010-01-01

Purpose: To evaluate the prevalence and severity of adverse events (AEs) and treatment-related risk factors in long-term Wilms' tumor (WT) survivors, with special attention to radiotherapy. Methods and Materials: The single-center study cohort consisted of 185 WT survivors treated between 1966 and

3D-visualization by MRI for surgical planning of Wilms tumors; 3-D-Visualisierung in der MRT zur Operationsplanung von Wilms-Tumoren

Energy Technology Data Exchange (ETDEWEB)

Schenk, J.P.; Wunsch, R.; Jourdan, C.; Troeger, J. [Universitaetsklinik Heidelberg (Germany). Abteilung Paediatrische Radiologie; Waag, K.-L.; Guenther, P. [Universitaetsklinik Heidelberg (Germany). Abteilung Kinderchirurgie; Graf, N. [Universitaetsklinik Homburg (Germany). Abteilung Paediatrische Haematologie und Onkologie; Behnisch, W. [Universitaetsklinik Heidelberg (Germany). Abteilung Paediatrische Haematologie und Onkologie

2004-10-01

Purpose: To improve surgical planning of kidney tumors in childhood (Wilms tumor, mesoblastic nephroma) after radiologic verification of the presumptive diagnosis with interactive colored 3D-animation in MRI. Materials and Methods: In 7 children (1 boy, 6 girls) with a mean age of 3 years (1 month to 11 years), the MRI database (DICOM) was processed with a raycasting-based 3D-volume-rendering software (VG Studio Max 1.1/Volume Graphics). The abdominal MRI-sequences (coronal STIR, coronal T1 TSE, transverse T1/T2 TSE, sagittal T2 TSE, transverse and coronal T1 TSE post contrast) were obtained with a 0.5T unit in 4-6 mm slices. Additionally, phase-contrast-MR-angiography was applied to delineate the large abdominal and retroperitoneal vessels. A notebook was used to demonstrate the 3D-visualization for surgical planning before surgery and during the surgical procedure. Results: In all 7 cases, the surgical approach was influenced by interactive 3D-animation and the information found useful for surgical planning. Above all, the 3D-visualization demonstrates the mass effect of the Wilms tumor and its anatomical relationship to the renal hilum and to the rest of the kidney as well as the topographic relationship of the tumor to the critical vessels. One rupture of the tumor capsule occurred as a surgical complication. For the surgeon, the transformation of the anatomical situation from MRI to the surgical situs has become much easier. Conclusion: For surgical planning of Wilms tumors, the 3D-visualization with 3D-animation of the situs helps to transfer important information from the pediatric radiologist to the pediatric surgeon and optimizes the surgical preparation. A reduction of complications is to be expected. (orig.)

Birth characteristics and Wilms tumors in children in the Nordic countries: a register-based case-control study.

Science.gov (United States)

Schüz, Joachim; Schmidt, Lisbeth Samsø; Kogner, Per; Lähteenmäki, Päivi M; Pal, Niklas; Stokland, Tore; Schmiegelow, Kjeld

2011-05-01

Little is known about causes of Wilms tumor. Because of the young age at diagnosis, several studies have looked at various birth characteristics. We conducted a registry-based case-control study involving 690 cases of Wilms tumor aged 0-14 years, occurring in Denmark, Finland, Norway or Sweden during 1985-2006, individually matched to five controls drawn randomly from the Nordic childhood population. Information on birth characteristics was obtained from the population-based medical birth registries. We estimated odds ratios (ORs) and 95% confidence intervals (CIs) using conditional logistic regression analysis. We observed a distinct association between Wilms tumor and high birth weight (≥4 kg) for girls (OR 1.97, CI 1.50-2.59) but not for boys (1.04, 0.78-1.38); overall, the OR was 1.43 (1.17-1.74). Among girls, risk increased by 28% (15-42%) per 500 g increase in birth weight. Large-for-gestational age girls also had a higher risk (2.48, 1.51-4.05), whereas no effect was seen for boys (1.12, 0.60-2.07). An association was seen with Apgar score at 5 min birth order. In our large-scale, registry-based study, we confirmed earlier observations of an association between high birth weight and risk of Wilms tumor, but we found an effect only in girls. The higher risk of infants with low Apgar score might reflect hypoxia causing cell damage, adverse side effects of neonatal treatment or reverse causation as low Apgar score might indicate the presence of a tumor. Copyright © 2010 UICC.

Tumor de Wilms: Hallazgo coincidente

Directory of Open Access Journals (Sweden)

Jaime Galindo López

2006-01-01

Full Text Available El tumor de Wilms o nefroblastoma representa el 6% de los cánceres infantiles y se considera la formación abdominal y renal maligna más frecuente en la edad pediátrica. En Estados Unidos se diagnostican aproximadamente 500 nuevos casos por año, siendo más frecuente el hallazgo a los 36 meses de edad, aunque es posible encontrarlo en momentos tan tempranos como el nacimiento, e incluso hasta los 15 años de edad, con igual probabilidad de encontrarlo en niñas y niños a cualquier edad. Es un tumor agresivo, tiene la capacidad de alcanzar gran tamaño e incluso hacer metástasis a distancia. Se presenta por su inusual manifestación inicial el caso de un paciente de 28 meses de edad, sexo masculino, que consultó al servicio de urgencias por dolor en rodilla izquierda de 24 horas de evolución, acompañado de imposibilidad para la marcha. Al examen físico inicial se encontró presencia de masa abdominal.

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.

Nephrogenic rests mimicking Wilms' tumor on CT

International Nuclear Information System (INIS)

Subhas, Naveen; Siegelman, Stanley S.; Argani, Pedram; Gearhart, John P.

2004-01-01

Nephrogenic rests (NR) are persistent benign remnants of embryonic renal tissue. A small percentage of these may develop into Wilms' tumor (WT). Radiologic imaging is relied upon to differentiate between these entities, with the hallmark of malignant transformation being growth on serial imaging studies. There is, however, considerable overlap in their imaging characteristics. The authors present a case of two biopsy-proven NR in a 2-year-old girl with sporadic aniridia that were indistinguishable from WT on initial radiologic studies. One of the NR grew on serial imaging studies mimicking a WT, but after resection was confirmed to be a benign hyperplastic NR on pathologic examination. (orig.)

Vincristine, Irinotecan, and Bevacizumab in Relapsed Wilms Tumor With Diffuse Anaplasia.

Science.gov (United States)

Schiavetti, Amalia; Varrasso, Giulia; Collini, Paola; Clerico, Anna

2018-05-01

The prognosis of relapsed Wilms tumor (WT) with diffuse anaplasia is dismal, therefore, novel therapeutic strategies need to be explored. We reported on 2 consecutive cases with relapsed anaplastic WT who presented a partial response after 2 courses of vincristine, irinotecan, and bevacizumab association. This regimen may have a role in the treatment of patients with anaplastic advanced WT.

Children with Idiopathic Hemihypertrophy and Beckwith-Wiedemann Syndrome Have Different Constitutional Epigenotypes Associated with Wilms Tumor

OpenAIRE

Niemitz, Emily L. ; Feinberg, Andrew P. ; Brandenburg, Sheri A. ; Grundy, Paul E. ; DeBaun, Michael R. 

2005-01-01

Idiopathic hemihypertrophy (IH) is a congenital overgrowth syndrome associated with an increased risk of embryonal cancers in childhood. A related developmental disorder is Beckwith-Wiedemann syndrome (BWS), which increases risk for embryonal cancers, including Wilms tumor. Constitutional epigenetic alterations associated with BWS have been well characterized and include epigenetic alterations of imprinted genes on 11p15. The frequency of hypermethylation of H19 in children with IH and Wilms ...

Molecular analysis of aniridia patients for deletions involving the Wilms' tumor gene

NARCIS (Netherlands)

Drechsler, M.; Meijers-Heijboer, E. J.; Schneider, S.; Schurich, B.; Grond-Ginsbach, C.; Tariverdian, G.; Kantner, G.; Blankenagel, A.; Kaps, D.; Schroeder-Kurth, T.

1994-01-01

A human aniridia candidate (AN) gene on chromosome 11p13 has been cloned and characterized. The AN gene is the second cloned gene of the contiguous genes syndrome WAGR (Wilms' tumor, aniridia, genitourinary malformations, mental retardation) on chromosome 11p13, WT1 being the first gene cloned.

Deletions of 16q in Wilms tumors localize to blastemal-anaplastic cells and are associated with reduced expression of the IRXB renal tubulogenesis gene cluster

NARCIS (Netherlands)

Mengelbier, Linda Holmquist; Karlsson, Jenny; Lindgren, David; Øra, Ingrid; Isaksson, Margareth; Frigyesi, Ildiko; Frigyesi, Attila; Bras, Johannes; Sandstedt, Bengt; Gisselsson, David

2010-01-01

Wilms tumor is the most common pediatric renal neoplasm, but few molecular prognostic markers have been identified for this tumor. Somatic deletion in the long arm of chromosome 16 (16q) is known to predict a less favorable outcome in Wilms tumor, but the underlying molecular mechanisms are not

Physical interaction between Wilms tumor 1 and p73 proteins modulates their functions

NARCIS (Netherlands)

Scharnhorst, V.; Dekker, P.; Eb, van der A.J.; Jochemsen, A.G.

2014-01-01

The WT1 gene, which is heterozygously mutated or deleted in congenital anomaly syndromes and homozygously mutated in about 15% of all Wilms tumors, encodes tissue-specific developmental regulators. Through alternative mRNA splicing, four main WT1 protein isoforms are synthesized. All isoforms can

CT findings in adult Wilms' tumor involving both kidneys

International Nuclear Information System (INIS)

Feijoo, R.; Lasierra, R.; Pina, J.I.; Benito, J.L.

1998-01-01

Wilms' tumor is the most common renal neoplasm during childhood, but it rarely presents in adults and even less frequently involves both kidneys at onset. The preoperative diagnosis is difficult, although it should be suspected (despite the low incidence) in cases of renal masses that contain necrotic tissue, calcification or fat. We report the case of a 32-year-old woman who presented with bilateral kidney mass and lung metastases. The patient had never complained of urinary symptoms prior to this finding. (Author) 12 refs

Late orthopedic effects in children with Wilms' tumor treated with abdominal irradiation

International Nuclear Information System (INIS)

Rate, W.R.; Butler, M.S.; Robertson, W.W. Jr.; D'Angio, G.J.

1991-01-01

Between 1970 and 1984, 31 children with biopsy-proven Wilms' tumor received nephrectomy, chemotherapy, and abdominal irradiation and were followed beyond skeletal maturity. Three patients (10%) developed late orthopedic abnormalities requiring intervention. Ten children received orthovoltage irradiation, and all cases requiring orthopedic intervention or developing a scoliotic curve of greater than 20 degrees were confined to this group, for a complication frequency of 50%. Those children who developed a significant late orthopedic abnormality (SLOA) as defined were treated to a higher median dose (2,890 cGy) and a larger field size (150 cm2) than those who did not (2,580 cGy and 120 cm2). Age at irradiation, sex, and initial stage of disease did not appear to influence the risk of developing an SLOA. No child who received megavoltage irradiation developed an SLOA despite treatment up to 4,000 cGy or to field sizes of 400 cm2. We conclude that modern radiotherapy techniques rarely lead to significant late orthopedic abnormalities previously associated with abdominal irradiation in children with Wilms' tumor

Molecular profiling reveals frequent gain of MYCN and anaplasia-specific loss of 4q and 14q in Wilms tumor.

Science.gov (United States)

Williams, Richard D; Al-Saadi, Reem; Natrajan, Rachael; Mackay, Alan; Chagtai, Tasnim; Little, Suzanne; Hing, Sandra N; Fenwick, Kerry; Ashworth, Alan; Grundy, Paul; Anderson, James R; Dome, Jeffrey S; Perlman, Elizabeth J; Jones, Chris; Pritchard-Jones, Kathy

2011-12-01

Anaplasia in Wilms tumor, a distinctive histology characterized by abnormal mitoses, is associated with poor patient outcome. While anaplastic tumors frequently harbour TP53 mutations, little is otherwise known about their molecular biology. We have used array comparative genomic hybridization (aCGH) and cDNA microarray expression profiling to compare anaplastic and favorable histology Wilms tumors to determine their common and differentiating features. In addition to changes on 17p, consistent with TP53 deletion, recurrent anaplasia-specific genomic loss and under-expression were noted in several other regions, most strikingly 4q and 14q. Further aberrations, including gain of 1q and loss of 16q were common to both histologies. Focal gain of MYCN, initially detected by high resolution aCGH profiling in 6/61 anaplastic samples, was confirmed in a significant proportion of both tumor types by a genomic quantitative PCR survey of over 400 tumors. Overall, these results are consistent with a model where anaplasia, rather than forming an entirely distinct molecular entity, arises from the general continuum of Wilms tumor by the acquisition of additional genomic changes at multiple loci. Copyright © 2011 Wiley Periodicals, Inc.

Intra-Tumor Genetic Heterogeneity in Wilms Tumor: Clonal Evolution and Clinical Implications

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George D. Cresswell

2016-07-01

Full Text Available The evolution of pediatric solid tumors is poorly understood. There is conflicting evidence of intra-tumor genetic homogeneity vs. heterogeneity (ITGH in a small number of studies in pediatric solid tumors. A number of copy number aberrations (CNA are proposed as prognostic biomarkers to stratify patients, for example 1q+ in Wilms tumor (WT; current clinical trials use only one sample per tumor to profile this genetic biomarker. We multisampled 20 WT cases and assessed genome-wide allele-specific CNA and loss of heterozygosity, and inferred tumor evolution, using Illumina CytoSNP12v2.1 arrays, a custom analysis pipeline, and the MEDICC algorithm. We found remarkable diversity of ITGH and evolutionary trajectories in WT. 1q+ is heterogeneous in the majority of tumors with this change, with variable evolutionary timing. We estimate that at least three samples per tumor are needed to detect >95% of cases with 1q+. In contrast, somatic 11p15 LOH is uniformly an early event in WT development. We find evidence of two separate tumor origins in unilateral disease with divergent histology, and in bilateral WT. We also show subclonal changes related to differential response to chemotherapy. Rational trial design to include biomarkers in risk stratification requires tumor multisampling and reliable delineation of ITGH and tumor evolution.

Nephrogenic rests mimicking Wilms' tumor on CT

Energy Technology Data Exchange (ETDEWEB)

Subhas, Naveen; Siegelman, Stanley S. [Russell H. Morgan Department of Radiology, The Johns Hopkins Hospital and School of Medicine, 600 N. Wolfe St., 21287, Baltimore, MD (United States); Argani, Pedram [Department of Pathology, Johns Hopkins Hospital and School of Medicine, 21287, Baltimore, MD (United States); Gearhart, John P. [Department of Pediatric Urology, Brady Urologic Institute, The Johns Hopkins Hospital and School of Medicine, 21287, Baltimore, MD (United States)

2004-02-01

Nephrogenic rests (NR) are persistent benign remnants of embryonic renal tissue. A small percentage of these may develop into Wilms' tumor (WT). Radiologic imaging is relied upon to differentiate between these entities, with the hallmark of malignant transformation being growth on serial imaging studies. There is, however, considerable overlap in their imaging characteristics. The authors present a case of two biopsy-proven NR in a 2-year-old girl with sporadic aniridia that were indistinguishable from WT on initial radiologic studies. One of the NR grew on serial imaging studies mimicking a WT, but after resection was confirmed to be a benign hyperplastic NR on pathologic examination. (orig.)

Loss of 11q and 16q in Wilms tumors is associated with anaplasia, tumor recurrence, and poor prognosis.

Science.gov (United States)

Wittmann, Stefanie; Zirn, Birgit; Alkassar, Muhannad; Ambros, Peter; Graf, Norbert; Gessler, Manfred

2007-02-01

Allele loss of chromosome arms 11q and 16q in Wilms tumors has been associated with different clinical parameters in prior studies. To substantiate these findings in a large collection of tumors treated according to the GPOH/SIOP protocol and to narrow down critical regions, we performed loss of heterozygosity (LOH) analyses of chromosome arms 11q and 16q on 225 Wilms tumors. On chromosome arm 11q an overall rate of allele loss of 19.6% (44 of 225 tumors) was found using eleven markers that were almost evenly distributed along the long arm. Chromosome arm 16q was analyzed with six markers selected from gene-rich regions that identified an LOH rate of 18.4% (41/223). Evaluation of LOH with respect to clinical data revealed significant associations of LOH 11q with histology: LOH 11q was 3-4 times more frequent in mixed type and diffuse anaplastic tumors. In contrast, epithelial as well as stromal type tumors never exhibited allele loss on 11q. Furthermore, a significant correlation with tumor recurrence and death was detected, but only for tumors that lost the entire long arm of chromosome 11. Similarly, LOH 16q was correlated with higher risks of later relapse, especially in tumors with complete loss of the long arm. Hence, analyses of LOH on 11q and 16q appear to be helpful to identify tumors with a higher risk of relapse and adverse outcome, which need adjusted therapeutic approaches. Copyright 2006 Wiley-Liss, Inc.

Significance and management of computed tomography detected pulmonary nodules: a report from the National Wilms Tumor Study Group

International Nuclear Information System (INIS)

Meisel, Jay A.; Guthrie, Katherine A.; Breslow, Norman E.; Donaldson, Sarah S.; Green, Daniel M.

1999-01-01

Purpose: To define the optimal treatment for children with Wilms tumor who have pulmonary nodules identified on chest computed tomography (CT) scan, but have a negative chest radiograph, we evaluated the outcome of all such patients randomized or followed on National Wilms Tumor Study (NWTS)-3 and -4. Patients and Methods: We estimated the event-free and overall survival percentages of 53 patients with favorable histology tumors and pulmonary densities identified only by CT scan (CT-only) who were treated as Stage IV with intensive doxorubicin-containing chemotherapy and whole-lung irradiation, and compared these to the event-free and overall survival percentages of 37 CT-only patients who were treated less aggressively based on the extent of locoregional disease with 2 or 3 drugs, and without whole-lung irradiation. Results: The 4-year event-free and overall survival percentages of the 53 patients with CT-only nodules and favorable histology Wilms tumor who were treated as Stage IV were 89% and 91%, respectively. The 4-year event-free and overall survival percentages for the 37 patients with CT-only nodules and favorable histology who were treated according to the extent of locoregional disease were 80% and 85%, respectively. The differences observed between the 2 groups were not statistically significant. Among the patients who received whole-lung irradiation, there were fewer pulmonary relapses, but more deaths attributable to lung toxicity. Conclusions: The current data raise the possibility that children with Wilms tumor and CT-only pulmonary nodules who receive whole lung irradiation have fewer pulmonary relapses, but a greater number of deaths due to treatment toxicity. The role of whole lung irradiation in the treatment of this group of patients cannot be definitively determined based on the present data. Prolonged follow-up of this group of patients is necessary to accurately estimate the frequency of late, treatment-related mortality

Induction of antiproliferative connective tissue growth factor expression in Wilms' tumor cells by sphingosine-1-phosphate receptor 2.

Science.gov (United States)

Li, Mei-Hong; Sanchez, Teresa; Pappalardo, Anna; Lynch, Kevin R; Hla, Timothy; Ferrer, Fernando

2008-10-01

Connective tissue growth factor (CTGF), a member of the CCN family of secreted matricellular proteins, regulates fibrosis, angiogenesis, cell proliferation, apoptosis, tumor growth, and metastasis. However, the role of CTGF and its regulation mechanism in Wilms' tumor remains largely unknown. We found that the bioactive lipid sphingosine-1-phosphate (S1P) induced CTGF expression in a concentration- and time-dependent manner in a Wilms' tumor cell line (WiT49), whereas FTY720-phosphate, an S1P analogue that binds all S1P receptors except S1P2, did not. Further, the specific S1P2 antagonist JTE-013 completely inhibited S1P-induced CTGF expression, whereas the S1P1 antagonist VPC44116 did not, indicating that this effect was mediated by S1P2. This was confirmed by adenoviral transduction of S1P2 in WiT49 cells, which showed that overexpression of S1P2 increased the expression of CTGF. Induction of CTGF by S1P was sensitive to ROCK inhibitor Y-27632 and c-Jun NH2-terminal kinase inhibitor SP600125, suggesting the requirement of RhoA/ROCK and c-Jun NH2-terminal kinase pathways for S1P-induced CTGF expression. Interestingly, the expression levels of CTGF were decreased in 8 of 10 Wilms' tumor tissues compared with matched normal tissues by quantitative real-time PCR and Western blot analysis. In vitro, human recombinant CTGF significantly inhibited the proliferation of WiT49 cells. In addition, overexpression of CTGF resulted in significant inhibition of WiT49 cell growth. Taken together, these data suggest that CTGF protein induced by S1P2 might act as a growth inhibitor in Wilms' tumor.

Clonal expansion to anaplasia in Wilms` tumors is associated with p53 mutations

Energy Technology Data Exchange (ETDEWEB)

Pelletier, J.; Beckwith, B.; Bardeesy, N. [Loma Linda Univ., CA (United States)]|[McGill Univ., Montreal (Canada)

1994-09-01

The genetics of Wilms` tumor (WT), a pediatric malignancy of the kidney, is complex. Three loci are implicated in WT initiation and include the WT1 tumor suppressor gene (residing at 11p13), an 11p15 locus, and a non-11p locus. As well, allelic loss at 16q24 in {approximately}20% of sporadic WTs suggests the location of (an) additional gene(s) involved in tumor progression. Initiation and progression in WTs is associated with multiple histological variants. Anaplasia is a rare WT subtype associated with poor prognosis and defined by enlarged and multipolar mitotic figures, a threefold nuclear enlargement (compared with adjacent nuclei of the same cell type), and hyperchromasia of the enlarged nuclei. We have previously demonstrated that p53 gene mutations are exclusively associated with anaplastic WTs, being absent from a large number of non-anaplastic WTs analyzed. To determine if such mutations are involved in clonal progression to anaplasia, we performed a retrospective analysis of histologically defined sections from tumor specimens. Six of ten WTs demonstrated p53 mutations by PCR-single stranded conformational polymorphism analysis. Two of these samples were paired, consisting of geographically demarcated anaplastic cells embedded within a non-anaplastic tumor bed. In these cases, p53 mutations were only present in the anaplastic region of the tumor. An overall decrease in the number of apoptotic cells was found associated with the anaplastic tumor region, compared to adjacent non-anaplastic tumor bed. These results indicate that p53 mutations arise during progression to anaplasia late in Wilms` tumor etiology and are associated with a more aggressive form of this cancer.

Wilms Tumor With Metastasis to the Vagina: A Case Report.

Science.gov (United States)

Howe, Adam S; Morganstern, Bradley A; Appelbaum, Heather; Mehta, Sandeep; Palmer, Lane S

2017-03-01

A 12-year-old female presented with abdominal pain, night sweats, weight loss, constipation, dysmenorrhea, menorrhagia, and vaginal discharge. Examination revealed a palpable flank mass and a large tumor adherent to the anterior vaginal wall. Computed tomography scan demonstrated a 23 cm mass in the left kidney, a separate 10.8 cm pelvic mass, and metastatic disease. Biopsies were consistent with Wilms tumor. Neoadjuvant chemotherapy and a left radical nephrectomy were performed for her stage IV disease as the kidney was amiable to complete resection. The patient received radiation and resumed chemotherapy. She was doing well with improved symptoms at follow-up. Copyright © 2016 Elsevier Inc. All rights reserved.

Expression of a possible constitutional hot spot in sperm chromosomes of a patient treated for Wilms' tumor

International Nuclear Information System (INIS)

Genesca, A.; Miro, R.; Caballin, M.R.; Benet, J.; Navarro, J.; Templado, C.; Bonfill, X.; Egozcue, J.

1987-01-01

Sperm chromosomes were studied in a man who was treated for Wilms' tumor with radiotherapy (RT) and chemotherapy (CT) 18 years ago. Human pronuclear sperm chromosomes were obtained after penetration of zona-free hamster eggs. Eighty-nine sperm chromosome complements were analyzed; 12.4% of them showed structural anomalies. This percentage was statistically different from the one found in our laboratory for controls (p less than 0.05). Five of eleven structurally abnormal metaphases had the same aberration: fission of chromosome number1 with the breakpoint at or near the centromere. Breaks and rearrangements of chromosome number1, often involving the centromere region, are among the most frequent anomalies found in Wilms' tumor cells

Adult Wilms' tumour: a case report with review of literature

Directory of Open Access Journals (Sweden)

Gowda KM Srinivasa

2006-12-01

Full Text Available Abstract Background Wilms' tumor is the commonest primary malignant renal tumor in childhood. Rarely, it may present in the adult age group. Case presentation We report a 48-year-old male presenting with flank pain and haematuria. Abdominal ultrasound revealed a right renal mass measuring 11 × 10 cms, and a clinical diagnosis of renal cell carcinoma was made. Nephrectomy was performed, and a final diagnosis of adult Wilms' tumor was made based on the criteria proposed by Kilton et al. Conclusion The possibility of an adult Wilms' tumor should be considered when a patient presents with pain in the flank and a renal mass. Rarity of the tumor favors documentation in literature.

The E7-associated cell-surface antigen: a marker for the 11p13 chromosomal deletion associated with aniridia-Wilms tumor.

OpenAIRE

Scoggin, C H; Fisher, J H; Shoemaker, S A; Morse, H; Leigh, T; Riccardi, V M

1985-01-01

Unbalanced interstitial deletions of the p13 region of human chromosome 11 have been associated with congenital hypoplasia or aplasia of the iris, mental retardation, ambiguous genitalia, and predisposition to Wilms tumor of the kidney. Utilizing somatic cell hybrids containing either the normal or abnormal chromosome 11 from a child with Wilms tumor and aniridia, we previously mapped the E7 cell-surface antigen to the 11p1300-to-11p15.1 region. To localize even further the site of this antig...

The sensitivity testing of Wilms' tumors to cytostatic agents with an autoradiographic in vitro short-term test

International Nuclear Information System (INIS)

Willnow, U.

1984-01-01

Sensitivity of 15 Wilms' tumors in children was tested towards cytostatic agents in vitro by means of an autoradiographic short-term test. Sensitivity was measured as the magnitude of the inhibition of 3 H-thymidine or 3 H-uridine incorporation. The test was performed with Adriamycin, Actinomycin D, Daunomycin, Bleomycin, Cyclophosphamide, Ifosfamide, Trenimon, and Arabinosylcytosine. None of the tumors is resistant to all substances, they are responsive against 2 or more drugs. The most effective drugs tested are Adriamycin, Actinomycin D and Cyclophosphamide. The tumors show a marked individual sensitivity pattern. This behavior is explained mainly by the usually high proliferative activity of Wilms' tumors. The possibilities and limits of long-term and short-term methods for sensitivity testing are discussed critically. For the evaluation of the results of in vitro testing and in vivo effectiveness the close correlation should be considered between the type of cytostatic agent and proliferation kinetics of the tumor, cytostatic agent and effect on tumor metabolism as well as the effect of the cytostatics and the nucleic acid precursors used for the short-term test. Despite the methodological limitations preclinical testing should be preferred to unselected chemotherapy. (author)

Morphoproteomic profiling of the mammalian target of rapamycin (mTOR) signaling pathway in desmoplastic small round cell tumor (EWS/WT1), Ewing's sarcoma (EWS/FLI1) and Wilms' tumor(WT1).

Science.gov (United States)

Subbiah, Vivek; Brown, Robert E; Jiang, Yunyun; Buryanek, Jamie; Hayes-Jordan, Andrea; Kurzrock, Razelle; Anderson, Pete M

2013-01-01

Desmoplastic small round cell tumor (DSRCT) is a rare sarcoma in adolescents and young adults. The hallmark of this disease is a EWS-WT1 translocation resulting from apposition of the Ewing's sarcoma (EWS) gene with the Wilms' tumor (WT1) gene. We performed morphoproteomic profiling of DSRCT (EWS-WT1), Ewing's sarcoma (EWS-FLI1) and Wilms' tumor (WT1) to better understand the signaling pathways for selecting future targeted therapies. This pilot study assessed patients with DSRCT, Wilms' tumor and Ewing's sarcoma. Morphoproteomics and immunohistochemical probes were applied to detect: p-mTOR (Ser2448); p-Akt (Ser473); p-ERK1/2 (Thr202/Tyr204); p-STAT3 (Tyr 705); and cell cycle-related analytes along with their negative controls. In DSRCT the PI3K/Akt/mTOR pathway is constitutively activated by p-Akt (Ser 473) expression in the nuclear compartment of the tumor cells and p-mTOR phosphorylated on Ser 2448, suggesting mTORC2 (rictor+mTOR) as the dominant form. Ewing's sarcoma had upregulated p-Akt and p-mTOR, predominantly mTORC2. In Wilm's tumor, the mTOR pathway is also activated with most tumor cells moderately expressing p-mTOR (Ser 2448) in plasmalemmal and cytoplasmic compartments. This coincides with the constitutive activation of one of the downstream effectors of the mTORC1 signaling pathway, namely p-p70S6K (Thr 389). There was constitutive activation of the Ras/Raf/ERK pathway p-ERK 1/2 (Thr202/Tyr204) expression in the Wilms tumor and metastatic Ewing's sarcoma, but not in the DSRCT. MORPHOPROTEOMIC TUMOR ANALYSES REVEALED CONSTITUTIVE ACTIVATION OF THE MTOR PATHWAY AS EVIDENCED BY: (a) expression of phosphorylated (p)-mTOR, p-p70S6K; (b) mTORC 2 in EWS and DSRCT; (c) ERK signaling was seen in the advanced setting indicating these as resistance pathways to IGF1R related therapies. This is the first morphoproteomic study of such pathways in these rare malignancies and may have potential therapeutic implications. Further study using morphoproteomic

Unusual association of non-anaplastic Wilms tumor and Cornelia de Lange syndrome: case report.

Science.gov (United States)

Santoro, Claudia; Apicella, Andrea; Casale, Fiorina; La Manna, Angela; Di Martino, Martina; Di Pinto, Daniela; Indolfi, Cristiana; Perrotta, Silverio

2016-06-13

Cornelia de Lange syndrome is the prototype for cohesinopathy disorders, which are characterized by defects in chromosome segregation. Kidney malformations, including nephrogenic rests, are common in Cornelia de Lange syndrome. Only one post-mortem case report has described an association between Wilms tumor and Cornelia de Lange syndrome. Here, we describe the first case of a living child with both diseases. Non-anaplastic triphasic nephroblastoma was diagnosed in a patient carrying a not yet reported mutation in NIPBL (c.4920 G > A). The patient had the typical facial appearance and intellectual disability associated with Cornelia de Lange syndrome in absence of limb involvement. The child's kidneys were examined by ultrasound at 2 years of age to exclude kidney abnormalities associated with the syndrome. She underwent pre-operative chemotherapy and nephrectomy. Seven months later she was healthy and without residual detectable disease. The previous report of such co-occurrence, together with our report and previous reports of nephrogenic rests, led us to wonder if there may be any causal relationship between these two rare entities. The wingless/integrated (Wnt) pathway, which is implicated in kidney development, is constitutively activated in approximately 15-20 % of all non-anaplastic Wilms tumors. Interestingly, the Wnt pathway was recently found to be perturbed in a zebrafish model of Cornelia de Lange syndrome. Mutations in cohesin complex genes and regulators have also been identified in several types of cancers. On the other hand, there is no clear evidence of an increased risk of cancer in Cornelia de Lange syndrome, and no other similar cases have been published since the fist one reported by Cohen, and this prompts to think Wilms tumor and Cornelia de Lange syndrome occurred together in our patient by chance.

Unusual association of non-anaplastic Wilms tumor and Cornelia de Lange syndrome: case report

International Nuclear Information System (INIS)

Santoro, Claudia; Apicella, Andrea; Casale, Fiorina; La Manna, Angela; Di Martino, Martina; Di Pinto, Daniela; Indolfi, Cristiana; Perrotta, Silverio

2016-01-01

Cornelia de Lange syndrome is the prototype for cohesinopathy disorders, which are characterized by defects in chromosome segregation. Kidney malformations, including nephrogenic rests, are common in Cornelia de Lange syndrome. Only one post-mortem case report has described an association between Wilms tumor and Cornelia de Lange syndrome. Here, we describe the first case of a living child with both diseases. Non-anaplastic triphasic nephroblastoma was diagnosed in a patient carrying a not yet reported mutation in NIPBL (c.4920 G > A). The patient had the typical facial appearance and intellectual disability associated with Cornelia de Lange syndrome in absence of limb involvement. The child’s kidneys were examined by ultrasound at 2 years of age to exclude kidney abnormalities associated with the syndrome. She underwent pre-operative chemotherapy and nephrectomy. Seven months later she was healthy and without residual detectable disease. The previous report of such co-occurrence, together with our report and previous reports of nephrogenic rests, led us to wonder if there may be any causal relationship between these two rare entities. The wingless/integrated (Wnt) pathway, which is implicated in kidney development, is constitutively activated in approximately 15–20 % of all non-anaplastic Wilms tumors. Interestingly, the Wnt pathway was recently found to be perturbed in a zebrafish model of Cornelia de Lange syndrome. Mutations in cohesin complex genes and regulators have also been identified in several types of cancers. On the other hand, there is no clear evidence of an increased risk of cancer in Cornelia de Lange syndrome, and no other similar cases have been published since the fist one reported by Cohen, and this prompts to think Wilms tumor and Cornelia de Lange syndrome occurred together in our patient by chance

The role of preoperative chemotherapy in the management of Wilms' tumor. The SIOP studies. International Society of Pediatric Oncology

NARCIS (Netherlands)

Graf, N.; Tournade, M. F.; de Kraker, J.

2000-01-01

More than 25 years after introducing preoperative chemotherapy for Wilms' tumor, the benefits of this approach are well known. The preoperative protocol results in easier operations with significantly fewer tumor ruptures during surgery and a favorable stage distribution. Acute toxicity and late

Birth characteristics and Wilms tumors in children in the Nordic countries

DEFF Research Database (Denmark)

Schüz, Joachim; Schmidt, Lisbeth Samsø; Kogner, Per

2011-01-01

during 1985-2006, individually matched to five controls drawn randomly from the Nordic childhood population. Information on birth characteristics was obtained from the population-based medical birth registries. We estimated odds ratios (ORs) and 95% confidence intervals (CIs) using conditional logistic......-based study, we confirmed earlier observations of an association between high birth weight and risk of Wilms tumor, but we found an effect only in girls. The higher risk of infants with low Apgar score might reflect hypoxia causing cell damage, adverse side effects of neonatal treatment or reverse causation...

Spinal Metastasis Of Wilm's Tumuor: An Unusual Occurrence ...

African Journals Online (AJOL)

Background: Metastasis of the Wilm's tumor is usually to surrounding tissue, the lungs and the liver. Rarely is there spread to bone, bone-marrow, spinal canal and other tissues, but this unusual mode of spread sometimes occurs. Objectives: To report a case of Wilm's tumuor complicated by spastic paraplegia consequent to ...

Prognóstico de pacientes com tumor de Wilms unilateral no Rio de Janeiro, 1990-2000 Prognosis for patients with unilateral Wilms' tumor in Rio de Janeiro, Brazil, 1990-2000

Directory of Open Access Journals (Sweden)

Marilia Fornaciari Grabois

2005-10-01

Full Text Available OBJETIVO: Analisar a sobrevida e os principais fatores prognósticos entre os pacientes com tumor de Wilms unilateral. MÉTODOS: A coorte de estudo incluiu 132 casos de tumor de Wilms unilateral em menores de 15 anos de idade matriculados em serviço de oncologia pediátrica, de janeiro de 1990 a dezembro de 2000. Curvas de sobrevida foram confeccionadas utilizando-se o método de Kaplan-Meier e fatores prognósticos foram analisados pelo modelo de riscos proporcionais de Cox. RESULTADOS: A estimativa de sobrevida global em cinco anos foi 84,6%. As probabilidades de sobrevida para os estádios I, II, III e IV foram de 100%; 94,2%; 83,2% e 31,3%, respectivamente. A taxa de sobrevida para os pacientes com: histologia favorável foi de 89,4%, para aqueles com anaplasia focal 66,7 % e com anaplasia difusa 40%. Todos os pacientes com doença em estádio IV e anaplasia difusa foram a óbito (n=4. Todos os pacientes com doença em estádio I, independente da histologia, permaneceram vivos até o final do período de seguimento. CONCLUSÕES: Entre as variáveis escolhidas para o modelo final apenas o estadiamento e a histologia permaneceram associados ao elevado risco de óbito enquanto que os casos na faixa etária entre 24 e 47 meses apresentaram melhor prognóstico que os demais. Esses resultados mostram a importância do diagnóstico em fases iniciais da doença e que a histologia é fundamental para orientar a terapia adequada.OBJECTIVE: To analyze the survival and the main prognostic factors among patients with unilateral Wilms' tumor patients. METHODS: The study cohort included 132 patients with unilateral Wilms' tumor aged under 15 years, who were enrolled in a pediatric oncology service. Survival curves were calculated using the Kaplan-Meier method and the prognostic factors were analyzed using the Cox proportional hazards model. RESULTS: The overall survival rate for five years was 84.6%. The survival probabilities for disease in stages I, II

Role for the Wilms tumor gene in genital development?

International Nuclear Information System (INIS)

van Heyningen, V.; Bickmore, W.A.; Seawright, A.; Fletcher, J.M.; Maule, J.; Hastie, N.D.; Fekete, G.; Gessler, M.; Bruns, G.A.P.; Huerre-Jeanpierre, C.; Junien, C.; Williams, B.R.G.

1990-01-01

Detailed molecular definition of the WAGR region at chromosome 11p13 has been achieved by chromosome breakpoint analysis and long-range restriction mapping. Here the authors describe the molecular detection of a cytogenetically invisible 1-megabase deletion in an individual with aniridia, cryptorchidism, and hypospadias but no Wilms tumor (WT). The region of overlap between this deletion and one associated with WT and similar genital anomalies but no aniridia covers a region of 350-400 kilobases, which is coincident with the extent of homozygous deletion detected in tumor tissue from a sporadic WT. A candidate WT gene located within this region has recently been isolated, suggesting nonpenetrance for tumor expression in the first individual. The inclusion within the overlap region of a gene for WT predisposition and a gene for the best-documented WT-associated genitourinary malformations leads to suggest that both of these anomalies result from a loss-of-function mutation at the same locus. This in turn implies that the WT gene exerts pleiotropic effect on both kidney and genitourinary development, a possibility supported by the observed expression pattern of the WT candidate gene in developing kidney and gonads

Imaging in unilateral Wilms tumour

International Nuclear Information System (INIS)

Brisse, Herve J.; Smets, Anne M.; Kaste, Sue C.; Owens, Catherine M.

2008-01-01

Wilms tumour is one of the most common malignancies in children, with an excellent prognosis after therapy. There is a very diverse approach to treatment according to geographical location. This variation in therapeutic attitude toward Wilms tumour, particularly between the United States and Europe, has consequences for the choice of imaging modality at diagnosis. In Europe, the International Society of Paediatric Oncology (SIOP) treatment protocol is based on chemotherapy followed by surgery. Imaging (US, CT and MRI), clinical history and examination will help predict whether the findings are consistent with Wilms tumour. Furthermore, in the UK preoperative image-guided biopsy is advised to help identify the small group of patients who, despite typical imaging features of Wilms tumour, have other types of neoplasia that require alternative management. In the United States, the National Wilms Tumor Study (NWTS) advises surgery prior to chemo- and radiotherapy. Hence imaging must provide detailed anatomical information for surgical planning. This article discusses the role of imaging at diagnosis and the relative strengths and weaknesses of the available radiological techniques. We also focus on imaging the lung for metastatic disease and the consequences (to the patient's ultimate outcome) of CT-diagnosed small pulmonary nodules and discuss the radiological diagnosis and consequences of tumour rupture present at diagnosis. (orig.)

Hypomethylation and Aberrant Expression of the Glioma Pathogenesis-Related 1 Gene in Wilms Tumors

Directory of Open Access Journals (Sweden)

Laxmi Chilukamarri

2007-11-01

Full Text Available Wilms tumors (WTs have a complex etiology, displaying genetic and epigenetic changes, including loss of imprinting (LOI and tumor suppressor gene silencing. To identify new regions of epigenetic perturbation in WTs, we screened kidney and tumor DNA using CpG island (CGI tags associated with cancer-specific DNA methylation changes. One such tag corresponded to a paralog of the glioma pathogenesis-related 1/related to testis-specific, vespid, and pathogenesis proteins 1 (GLIPR1/RTVP-1 gene, previously reported to be a tumor-suppressor gene silenced by hypermethylation in prostate cancer. Here we report methylation analysis of the GLIPR1/RTVP-1 gene in WTs and normal fetal and pediatric kidneys. Hypomethylation of the GLIPR1/RTVP-1 5'-region in WTs relative to normal tissue is observed in 21/24 (87.5% of WTs analyzed. Quantitative analysis of GLIPR1/RTVP-1 expression in 24 WTs showed elevated transcript levels in 16/24 WTs (67%, with 12 WTs displaying in excess of 20-fold overexpression relative to fetal kidney (FK control samples. Immunohistochemical analysis of FK and WT corroborates the RNA expression data and reveals high GLIPR1/RTVP-1 in WT blastemal cells together with variable levels in stromal and epithelial components. Hypomethylation is also evident in the WT precursor lesions and nephrogenic rests (NRs, supporting a role for GLIPR1/RTVP-1 deregulation early in Wilms tumorigenesis. Our data show that, in addition to gene dosage changes arising from LOI and hypermethylation-induced gene silencing, gene activation resulting from hypomethylation is also prevalent in WTs.

The Perlman syndrome: familial renal dysplasia with Wilms tumor, fetal gigantism and multiple congenital anomalies.

Science.gov (United States)

Neri, G; Martini-Neri, M E; Katz, B E; Opitz, J M

1984-09-01

We describe a familial syndrome of renal dysplasia, Wilms tumor, hyperplasia of the endocrine pancreas, fetal gigantism, multiple congenital anomalies and mental retardation. This condition was previously described by Perlman et al [1973, 1975] and we propose to call it the "Perlman syndrome." It appears to be transmitted as an autosomal recessive trait. The possible relationships between dysplasia, neoplasia and malformation are discussed.

HRAS1-selected chromosome transfer generates markers that colocalize aniridia- and genitourinary dysplasia-associated translocation breakpoints and the Wilms tumor gene within band 11p13.

OpenAIRE

Porteous, D J; Bickmore, W; Christie, S; Boyd, P A; Cranston, G; Fletcher, J M; Gosden, J R; Rout, D; Seawright, A; Simola, K O

1987-01-01

We show that chromosome-mediated gene transfer can provide an enriched source of DNA markers for predetermined, subchromosomal regions of the human genome. Forty-four human DNA recombinants isolated from a HRAS1-selected chromosome-mediated gene transformant map exclusively to chromosome 11, with several sublocalizing to the Wilms tumor region at 11p13. We present a detailed molecular map of the deletion chromosomes 11 from five WAGR (Wilms tumor/aniridia/genitourinary abnormalities/mental re...

Neurofibrosarcoma at irradiation site in a patient with neurofibromatosis and Wilms' tumor

International Nuclear Information System (INIS)

Chu, J.Y.; O'Connor, D.M.; Danis, R.K.

1981-01-01

A female patient with neurofibromatosis had nephrectomy performed because of Wilms' tumor at the age of four and a half. She received radiation therapy and chemotherapy (actinomycin D) after surgery. She had subsequent local recurrence and lung metastasis, which were surgically excised and successfully treated with additional radiation therapy and chemotherapy (vincristine and actinomycin D). However, neurofibrosarcoma at the irradiation site developed seven years after radiation therapy. She died 22 months later because of recurrence and metastasis of neurofibrosarcoma. Radiation therapy's association with malignant transformation of neurofibroma is discussed

Establishment of a Conditionally Immortalized Wilms Tumor Cell Line with a Homozygous WT1 Deletion within a Heterozygous 11p13 Deletion and UPD Limited to 11p15.

Directory of Open Access Journals (Sweden)

Artur Brandt

Full Text Available We describe a stromal predominant Wilms tumor with focal anaplasia and a complex, tumor specific chromosome 11 aberration: a homozygous deletion of the entire WT1 gene within a heterozygous 11p13 deletion and an additional region of uniparental disomy (UPD limited to 11p15.5-p15.2 including the IGF2 gene. The tumor carried a heterozygous p.T41A mutation in CTNNB1. Cells established from the tumor carried the same chromosome 11 aberration, but a different, homozygous p.S45Δ CTNNB1 mutation. Uniparental disomy (UPD 3p21.3pter lead to the homozygous CTNNB1 mutation. The tumor cell line was immortalized using the catalytic subunit of human telomerase (hTERT in conjunction with a novel thermolabile mutant (U19dl89-97tsA58 of SV40 large T antigen (LT. This cell line is cytogenetically stable and can be grown indefinitely representing a valuable tool to study the effect of a complete lack of WT1 in tumor cells. The origin/fate of Wilms tumors with WT1 mutations is currently poorly defined. Here we studied the expression of several genes expressed in early kidney development, e.g. FOXD1, PAX3, SIX1, OSR1, OSR2 and MEIS1 and show that these are expressed at similar levels in the parental and the immortalized Wilms10 cells. In addition the limited potential for muscle/ osteogenic/ adipogenic differentiation similar to all other WT1 mutant cell lines is also observed in the Wilms10 tumor cell line and this is retained in the immortalized cells. In summary these Wilms10 cells are a valuable model system for functional studies of WT1 mutant cells.

Establishment of a Conditionally Immortalized Wilms Tumor Cell Line with a Homozygous WT1 Deletion within a Heterozygous 11p13 Deletion and UPD Limited to 11p15

Science.gov (United States)

Brandt, Artur; Löhers, Katharina; Beier, Manfred; Leube, Barbara; de Torres, Carmen; Mora, Jaume; Arora, Parineeta; Jat, Parmjit S.; Royer-Pokora, Brigitte

2016-01-01

We describe a stromal predominant Wilms tumor with focal anaplasia and a complex, tumor specific chromosome 11 aberration: a homozygous deletion of the entire WT1 gene within a heterozygous 11p13 deletion and an additional region of uniparental disomy (UPD) limited to 11p15.5-p15.2 including the IGF2 gene. The tumor carried a heterozygous p.T41A mutation in CTNNB1. Cells established from the tumor carried the same chromosome 11 aberration, but a different, homozygous p.S45Δ CTNNB1 mutation. Uniparental disomy (UPD) 3p21.3pter lead to the homozygous CTNNB1 mutation. The tumor cell line was immortalized using the catalytic subunit of human telomerase (hTERT) in conjunction with a novel thermolabile mutant (U19dl89-97tsA58) of SV40 large T antigen (LT). This cell line is cytogenetically stable and can be grown indefinitely representing a valuable tool to study the effect of a complete lack of WT1 in tumor cells. The origin/fate of Wilms tumors with WT1 mutations is currently poorly defined. Here we studied the expression of several genes expressed in early kidney development, e.g. FOXD1, PAX3, SIX1, OSR1, OSR2 and MEIS1 and show that these are expressed at similar levels in the parental and the immortalized Wilms10 cells. In addition the limited potential for muscle/ osteogenic/ adipogenic differentiation similar to all other WT1 mutant cell lines is also observed in the Wilms10 tumor cell line and this is retained in the immortalized cells. In summary these Wilms10 cells are a valuable model system for functional studies of WT1 mutant cells. PMID:27213811

Brain metastasis of Wilms tumor with diffuse anaplasia and complex cytogenetic phenotype in a child with neurofibromatosis Type 1.

Science.gov (United States)

Shvartsbeyn, Marianna; Bassani, Luigi; Mikolaenko, Irina; Wisoff, Jeffrey H

2011-10-01

The authors report the first case of a Wilms tumor (WT) with diffuse anaplasia metastatic to the brain in a 13-year-old girl with a history of neurofibromatosis Type 1. At presentation, the metastatic tumor had radiological features that suggested a meningioma. Histologically it was characterized by striking anaplasia and features similar to the patient's previously resected WT with diffuse anaplasia.

Adult Wilms tumor with inferior vena cava thrombus and distal deep vein thrombosis - a case report and literature review.

Science.gov (United States)

Ratajczyk, Krzysztof; Czekaj, Adrian; Rogala, Joanna; Kowal, Pawel

2018-02-23

Adult Wilms tumor (WT, nephroblastoma) is a rare, but well-described renal neoplasm. Although inferior vena cava tumor thrombosis is present in up to 10% of Wilms tumors in childhood, only few cases of this clinical manifestation in adults have been reported. To the best of our knowledge, this is the first case of adult WT infiltrating into inferior vena cava (IVC) with concomitant distal deep vein thrombosis. A 28-year-old male patient with gross hematuria and right flank pain was diagnosed with right kidney tumor penetrating to IVC. Preoperatively, acute distal thrombosis in inferior vena cava and lower extremities veins occurred. Right radical nephrectomy with tumor thrombectomy via cavotomy was performed. In order to prevent pulmonary embolism, IVC was ligated below left renal vein level. Histopathological examination revealed a triphasic nephroblastoma without anaplastic features. Postoperatively, patient was diagnosed with metastatic liver disease, which was treated with two lines of chemotherapy followed by radiotherapy with achievement of complete response. Adult WT occurs usually in young patients, under 40 years of age. Neoadjuvant chemotherapy proved to be effective in children, resulting with tumor shrinkage and venous tumor thrombus regression. Therefore, percutaneous biopsy should be always considered in young patients presenting with renal tumor invading venous system. IVC ligation is a safe treatment option in the event of complete inferior vena cava occlusion due to distal thrombosis concomitant to tumor thrombus, provided collateral venous pathways are well-developed.

Regional localization of DNA probes on the short arm of chromosome 11 using aniridia-Wilms' tumor-associated deletions

NARCIS (Netherlands)

Mannens, M.; Slater, R. M.; Heyting, C.; Geurts van Kessel, A.; Goedde-Salz, E.; Frants, R. R.; van Ommen, G. J.; Pearson, P. L.

1987-01-01

We are interested in the precise localization of various DNA probes on the short arm of chromosome 11 for our research on the aniridia-Wilms' tumor association (AWTA), assigned to region 11p13 (Knudson and Strong 1972; Riccardi et al. 1978). For this purpose we have screened lymphocyte DNA and

New definitions of focal and diffuse anaplasia in Wilms tumor: the International Society of Paediatric Oncology (SIOP) experience

NARCIS (Netherlands)

Vujanić, G. M.; Harms, D.; Sandstedt, B.; Weirich, A.; de Kraker, J.; Delemarre, J. F.

1999-01-01

Unlike the original definitions of focal (FA) and diffuse anaplasia (DA) in Wilms tumor (WT), recently redefined FA and DA proved to be of prognostic significance. The aim of the study was to analyze WT from the SIOP file, the majority of which were treated with preoperative chemotherapy, in order

Relaxation of IGF2/H19 imprinting in Wilms tumour is associated with a switch in DNA methylation

Energy Technology Data Exchange (ETDEWEB)

Reeve, A.E.; Taniguchi, T.; Sullivan, M.J.; Ogawa, O. [Univ. of Otago, Dunedin (New Zealand)

1994-09-01

We and others have recently shown that the normal imprinting of the insulin-like growth factor 2 (IGF2) gene is disrupted in Wilms tumor. The process of relaxation of IGF2 imprinting leads to the activation of transcription of the normally silent maternally inherited IGF2 allele such that both alleles of the IGF2 gene are transcribed. Relaxation of IGF2 imprinting has also been detected as a constitutional event in patients with the Beckwith-Wiedemann syndrom and a patient with gigantism and Wilms tumor. We have now shown that in Wilms tumors in which imprinting is relaxed, IGF2 is transcribed from the maternal allele and there is a concomitant transcriptional inactivation of the H19 maternal allele. Furthermore, the patterns of methylation of the IGF2 and H19 gene are reversed on the maternal chromosome. Relaxation of imprinting in Wilms tumors appear, therefore, to be associated with a switch in gene expression and methylation at the IGF2/H19 locus. The data supports the notion of a disrupted IGF2/H19 imprinting switch in Wilms tumor.

Wilms' tumor in New York State: epidemiology and survivorship.

Science.gov (United States)

Griffel, M

1977-12-01

The outcomes during the period 1950--1972 were compared for Wilms' tumor patients in Erie County, New York (Buffalo and environs) and in a random selection of 23 counties having much smaller populations. For the Erie cohorts of 1967 to 1972 an 87 per cent 7-year survival rate was found as compared with a 50 per cent survival for the corresponding cohorts of the less populous couties. For the years 1960--1966 the 5-year survival rates were respectively 67 and 25 per cent and for the decade 1950--1959, 26 and 23 per cent. The principal conclusion is that within the last 15 years the Erie residents have fared better than residents of the smaller counties. The difference is attributed to the better treatment and care available at some of the hospitals in Buffalo. Data on incidence, age at diagnosis, male/female ratio, and laterality are presented.

Bilateral wilms tumor with TP53-related anaplasia.

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Popov, Sergey D; Vujanic, Gordan M; Sebire, Neil J; Chagtai, Tasnim; Williams, Richard; Vaidya, Sucheta; Pritchard-Jones, Kathy

2013-01-01

Wilms tumor (WT) with diffuse anaplasia has an unfavorable prognosis and is often (>70%) associated with mutations in the TP53 gene. Although most WTs are unilateral, 5-10% are bilateral, and they are almost always present with nephrogenic rests. The latter are considered a precursor of WT. Two cases of bilateral WTs with nephroblastomatosis, in which anaplastic changes were detected over a period of time, were analyzed using clinical, radiological, histopathological, and molecular-genetic data. TP53 was analyzed by direct sequencing of its full coding sequence and intron-exon boundaries in 11 fragments. DNA was extracted from paraffin-embedded or frozen specimens. High-resolution genomic copy number profiling was carried out by UCL Genomics on the Affymetrix Human Mapping 250K Nsp or Genome-Wide Human SNP Array 6.0 platform. Both cases demonstrated a strong association between the appearance of anaplastic clones and TP53 mutations. Synchronous ganglioneuroma was diagnosed in one case. Our cases are unique as they represent a long disease history and demonstrate the difficulties in managing rare cases of bilateral WT with anaplasia. These cases also emphasize the practical importance of modern molecular-genetic techniques and their clinical application. Moreover, they highlight the issue of the adequate sampling needed in order to gather comprehensive, efficient, and sufficient information about genetic events in a single tumor.

Thrombocytopenia and liver damage induced by actinomycin-D following radiotherapy in a patient with Wilms' tumor

International Nuclear Information System (INIS)

Watanabe, Takeshi; Kibe, Norio; Kawano, Yoshifumi; Yazawa, Kenji; Shiraga, Hiroshi; Hosoya, Ryota; Ohya, Tatsuo; Nishimura, Kozo; Yokoyama, Johtaro

1985-01-01

A two-year-old girl with Wilms' tumor received radiotherapy of 25.5 Gy to the right abdomen, followed by vincristine and actinomycin-D (Act). The patient developed general fatigue, anemia, thrombocytopenia, and liver damage associated with ascites. She improved by conservative therapy of two week duration. From the literature, it is suggested that such drug-related liver damage tends to occur when irradiation is given before chemotherapy, including Act. (Namekawa, K.)

Intraoperative Spillage of Favorable Histology Wilms Tumor Cells: Influence of Irradiation and Chemotherapy Regimens on Abdominal Recurrence. A Report From the National Wilms Tumor Study Group

International Nuclear Information System (INIS)

Kalapurakal, John A.; Li, Sierra M.; Breslow, Norman E.; Beckwith, J. Bruce; Ritchey, Michael L.; Shamberger, Robert C.; Haase, Gerald M.; Thomas, Patrick R.M.; Grundy, Paul; Green, Daniel M.; D'Angio, Giulio J.

2010-01-01

Purpose: We undertook this study to determine (1) the frequency with which spilled tumor cells of favorable histology produced intra-abdominal disease in patients treated with differing chemotherapy regimens and abdominal radiation therapy (RT) and (2) the patterns of relapse and outcomes in such patients. Methods and Materials: The influence of RT dose (0, 10, and 20 Gy), RT fields (flank, whole abdomen), and chemotherapy with dactinomycin and vincristine (2 drugs) vs. added doxorubicin (three drugs) on intra-abdominal tumor recurrence rates was analyzed by logistic regression in 450 patients. Each patient was considered at risk for two types of failure: flank and subdiaphragmatic beyond-flank recurrence, with the correlation between the two outcomes accounted for in the analyses. Results: The crude odds ratio for the risk of recurrence relative to no RT was 0.35 (0.15-0.78) for 10Gy and 0.08 (0.01-0.58) for 20Gy. The odds ratio for the risk of recurrence for doxorubicin to two drugs after adjusting for RT was not significant. For Stage II patients (NWTS-4), the 8-year event rates with and without spillage, respectively, were 79% and 87% for relapse-free survival (p = 0.07) and 90% and 95% for overall survival (p = 0.04). Conclusions: Irradiation (10 Gy or 20 Gy) reduced abdominal tumor recurrence rates after tumor spillage. Tumor spillage in Stage II patients reduced relapse-free survival and overall survival, but only the latter was of statistical significance. These data provide a basis for assessing the risks vs. benefits when considering treatment for children with favorable histology Wilms tumor and surgical spillage.

BASP1 is a transcriptional cosuppressor for the Wilms' tumor suppressor protein WT1

DEFF Research Database (Denmark)

Carpenter, Brian; Hill, Kathryn J; Charalambous, Marika

2004-01-01

The Wilms' tumor suppressor protein WT1 is a transcriptional regulator that plays a key role in the development of the kidneys. The transcriptional activation domain of WT1 is subject to regulation by a suppression region within the N terminus of WT1. Using a functional assay, we provide direct...... evidence that this requires a transcriptional cosuppressor, which we identify as brain acid soluble protein 1 (BASP1). WT1 and BASP1 associate within the nuclei of cells that naturally express both proteins. BASP1 can confer WT1 cosuppressor activity in transfection assays, and elimination of endogenous...

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.

Significance of TP53 Mutation in Wilms Tumors with Diffuse Anaplasia: A Report from the Children's Oncology Group.

Science.gov (United States)

Ooms, Ariadne H A G; Gadd, Samantha; Gerhard, Daniela S; Smith, Malcolm A; Guidry Auvil, Jaime M; Meerzaman, Daoud; Chen, Qing-Rong; Hsu, Chih Hao; Yan, Chunhua; Nguyen, Cu; Hu, Ying; Ma, Yussanne; Zong, Zusheng; Mungall, Andrew J; Moore, Richard A; Marra, Marco A; Huff, Vicki; Dome, Jeffrey S; Chi, Yueh-Yun; Tian, Jing; Geller, James I; Mullighan, Charles G; Ma, Jing; Wheeler, David A; Hampton, Oliver A; Walz, Amy L; van den Heuvel-Eibrink, Marry M; de Krijger, Ronald R; Ross, Nicole; Gastier-Foster, Julie M; Perlman, Elizabeth J

2016-11-15

To investigate the role and significance of TP53 mutation in diffusely anaplastic Wilms tumors (DAWTs). All DAWTs registered on National Wilms Tumor Study-5 (n = 118) with available samples were analyzed for TP53 mutations and copy loss. Integrative genomic analysis was performed on 39 selected DAWTs. Following analysis of a single random sample, 57 DAWTs (48%) demonstrated TP53 mutations, 13 (11%) copy loss without mutation, and 48 (41%) lacked both [defined as TP53-wild-type (wt)]. Patients with stage III/IV TP53-wt DAWTs (but not those with stage I/II disease) had significantly lower relapse and death rates than those with TP53 abnormalities. In-depth analysis of a subset of 39 DAWTs showed seven (18%) to be TP53-wt: These demonstrated gene expression evidence of an active p53 pathway. Retrospective pathology review of TP53-wt DAWT revealed no or very low volume of anaplasia in six of seven tumors. When samples from TP53-wt tumors known to contain anaplasia histologically were available, abnormal p53 protein accumulation was observed by immunohistochemistry. These data support the key role of TP53 loss in the development of anaplasia in WT, and support its significant clinical impact in patients with residual anaplastic tumor following surgery. These data also suggest that most DAWTs will show evidence of TP53 mutation when samples selected for the presence of anaplasia are analyzed. This suggests that modifications of the current criteria to also consider volume of anaplasia and documentation of TP53 aberrations may better reflect the risk of relapse and death and enable optimization of therapeutic stratification. Clin Cancer Res; 22(22); 5582-91. ©2016 AACR. ©2016 American Association for Cancer Research.

Significance of TP53 Mutation in Wilms Tumors with Diffuse Anaplasia: A Report from the Children’s Oncology Group

Science.gov (United States)

Ooms, Ariadne H.A.G.; Gadd, Samantha; Gerhard, Daniela S.; Smith, Malcolm A.; Guidry Auvil, Jaime M.; Meerzaman, Daoud; Chen, Qing-Rong; Hsu, Chih Hao; Yan, Chunhua; Nguyen, Cu; Hu, Ying; Ma, Yussanne; Zong, Zusheng; Mungall, Andrew J.; Moore, Richard A.; Marra, Marco A.; Huff, Vicki; Dome, Jeffrey S.; Chi, Yueh-Yun; Tian, Jing; Geller, James I.; Mullighan, Charles G.; Ma, Jing; Wheeler, David A.; Hampton, Oliver A.; Walz, Amy L.; van den Heuvel-Eibrink, Marry M.; de Krijger, Ronald R.; Ross, Nicole; Gastier-Foster, Julie M.; Perlman, Elizabeth J.

2016-01-01

Purpose To investigate the role and significance of TP53 mutation in diffusely anaplastic Wilms tumor (DAWT). Experimental Design All DAWTs registered on National Wilms Tumor Study-5 (n=118) with available samples were analyzed for TP53 mutations and copy loss. Integrative genomic analysis was performed on 39 selected DAWTs. Results Following analysis of a single random sample, 57 DAWT (48%) demonstrated TP53 mutations, 13(11%) copy loss without mutation, and 48(41%) lacked both (defined as TP53-wildtype (wt)). Patients with Stage III/IV TP53-wt DAWTs (but not those with Stage I/II disease) had significantly lower relapse and death rates than those with TP53 abnormalities. In-depth analysis of a subset of 39 DAWT showed 7(18%) to be TP53-wt: these demonstrated gene expression evidence of an active p53 pathway. Retrospective pathology review of TP53-wt DAWT revealed no or very low volume of anaplasia in 6/7 tumors. When samples from TP53-wt tumors known to contain anaplasia histologically were available, abnormal p53 protein accumulation was observed by immunohistochemistry. Conclusion These data support the key role of TP53 loss in the development of anaplasia in WT, and support its significant clinical impact in patients with residual anaplastic tumor following surgery. These data also suggest that most DAWTs will show evidence of TP53 mutation when samples selected for the presence of anaplasia are analyzed. This suggests that modifications of the current criteria to also consider volume of anaplasia and documentation of TP53 aberrations may better reflect the risk of relapse and death and enable optimization of therapeutic stratification. PMID:27702824

Development of oral cancer vaccine using recombinant Bifidobacterium displaying Wilms' tumor 1 protein.

Science.gov (United States)

Kitagawa, Koichi; Oda, Tsugumi; Saito, Hiroki; Araki, Ayame; Gonoi, Reina; Shigemura, Katsumi; Hashii, Yoshiko; Katayama, Takane; Fujisawa, Masato; Shirakawa, Toshiro

2017-06-01

Several types of vaccine-delivering tumor-associated antigens (TAAs) have been developed in basic and clinical research. Wilms' tumor 1 (WT1), identified as a gene responsible for pediatric renal neoplasm, is one of the most promising TAA for cancer immunotherapy. Peptide and dendritic cell-based WT1 cancer vaccines showed some therapeutic efficacy in clinical and pre-clinical studies but as yet no oral WT1 vaccine can be administrated in a simple and easy way. In the present study, we constructed a novel oral cancer vaccine using a recombinant Bifidobacterium longum displaying WT1 protein. B. longum 420 was orally administered into mice inoculated with WT1-expressing tumor cells for 4 weeks to examine anti-tumor effects. To analyze the WT1-specific cellular immune responses to oral B. longum 420, mice splenocytes were isolated and cytokine production and cytotoxic activities were determined. Oral administrations of B. longum 420 significantly inhibited WT1-expressing tumor growth and prolonged survival in mice. Immunohistochemical study and immunological assays revealed that B. longum 420 substantially induced tumor infiltration of CD4 + T and CD8 + T cells, systemic WT1-specific cytokine production, and cytotoxic activity mediated by WT1-epitope specific cytotoxic T lymphocytes, with no apparent adverse effects. Our novel oral cancer vaccine safely induced WT1-specific cellular immunity via activation of the gut mucosal immune system and achieved therapeutic efficacy with several practical advantages over existing non-oral vaccines.

A Retroperitoneal Extra-Renal Wilms' Tumour: A Case Report

African Journals Online (AJOL)

2017-03-06

Mar 6, 2017 ... renal origin might have arisen from totipotent germ cells and hence may consist of ... The exact embryonic origin of extrarenal Wilms' tumor is not certain,[12] but ... Stiller CA, Parkin DM. Human cancer: international variations.

Advances in Wilms Tumor Treatment and Biology: Progress Through International Collaboration.

Science.gov (United States)

Dome, Jeffrey S; Graf, Norbert; Geller, James I; Fernandez, Conrad V; Mullen, Elizabeth A; Spreafico, Filippo; Van den Heuvel-Eibrink, Marry; Pritchard-Jones, Kathy

2015-09-20

Clinical trials in Wilms tumor (WT) have resulted in overall survival rates of greater than 90%. This achievement is especially remarkable because improvements in disease-specific survival have occurred concurrently with a reduction of therapy for large patient subgroups. However, the outcomes for certain patient subgroups, including those with unfavorable histologic and molecular features, bilateral disease, and recurrent disease, remain well below the benchmark survival rate of 90%. Therapy for WT has been advanced in part by an increasingly complex risk-stratification system based on patient age; tumor stage, histology, and volume; response to chemotherapy; and loss of heterozygosity at chromosomes 1p and 16q. A consequence of this system has been the apportionment of patients into such small subgroups that only collaboration between large international WT study groups will support clinical trials that are sufficiently powered to answer challenging questions that move the field forward. This article gives an overview of the Children's Oncology Group and International Society of Pediatric Oncology approaches to WT and focuses on four subgroups (stage IV, initially inoperable, bilateral, and relapsed WT) for which international collaboration is pressing. In addition, biologic insights resulting from collaborative laboratory research are discussed. A coordinated expansion of international collaboration in both clinical trials and laboratory science will provide real opportunity to improve the treatment and outcomes for children with renal tumors on a global level. © 2015 by American Society of Clinical Oncology.

Differences in patterns of allelic loss between two common types of adult cancer, breast and colon carcinoma, and Wilms' tumor of childhood

NARCIS (Netherlands)

Devilee, P.; van den Broek, M.; Mannens, M.; Slater, R.; Cornelisse, C. J.; Westerveld, A.; Khan, P. M.

1991-01-01

Several chromosomal regions exhibit loss of heterozygosity (LOH) in different types of human tumor, and on this basis are presumed to carry-suppressor genes. We studied 7 of such chromosome regions, including 3p, 5q, 11p, 13q, 17p, 18q and 22q, using a selected set of DNA markers in 44 Wilms'

Comparing oncologic outcomes after minimally invasive and open surgery for pediatric neuroblastoma and Wilms tumor.

Science.gov (United States)

Ezekian, Brian; Englum, Brian R; Gulack, Brian C; Rialon, Kristy L; Kim, Jina; Talbot, Lindsay J; Adibe, Obinna O; Routh, Jonathan C; Tracy, Elisabeth T; Rice, Henry E

2018-01-01

Minimally invasive surgery (MIS) has been widely adopted for common operations in pediatric surgery; however, its role in childhood tumors is limited by concerns about oncologic outcomes. We compared open and MIS approaches for pediatric neuroblastoma and Wilms tumor (WT) using a national database. The National Cancer Data Base from 2010 to 2012 was queried for cases of neuroblastoma and WT in children ≤21 years old. Children were classified as receiving open or MIS surgery for definitive resection, with clinical outcomes compared using a propensity matching methodology (two open:one MIS). For children with neuroblastoma, 17% (98 of 579) underwent MIS, while only 5% of children with WT (35 of 695) had an MIS approach for tumor resection. After propensity matching, there was no difference between open and MIS surgery for either tumor for 30-day mortality, readmissions, surgical margin status, and 1- and 3-year survival. However, in both tumors, open surgery more often evaluated lymph nodes and had larger lymph node harvest. Our retrospective review suggests that the use of MIS appears to be a safe method of oncologic resection for select children with neuroblastoma and WT. Further research should clarify which children are the optimal candidates for this approach. © 2017 Wiley Periodicals, Inc.

Wilms' tumour (nephroblastoma)

African Journals Online (AJOL)

Wilms' tumour or nephroblastoma is a cancer of the kidney that ... It may be noticed by parents or it may be an incidental finding ... patients. It may lead to iron deficiency anaemia. Rarely Wilms' tumour may present with acquired von Willebrand's ... the best treatment approach. ... with multimodality therapy in paediatric.

Expression of the Wilms' tumor gene WT1 in the murine urogenital system.

Science.gov (United States)

Pelletier, J; Schalling, M; Buckler, A J; Rogers, A; Haber, D A; Housman, D

1991-08-01

The Wilms' tumor gene WT1 is a recessive oncogene that encodes a putative transcription factor implicated in nephrogenesis during kidney development. In this report we analyze expression of WT1 in the murine urogenital system. WT1 is expressed in non-germ-cell components of the testis and ovaries in both young and adult mice. In situ mRNA hybridization studies demonstrate that WT1 is expressed in the granulosa and epithelial cells of ovaries, the Sertoli cells of the testis, and in the uterine wall. In addition to the 3.1-kb WT1 transcript detected by Northern blotting of RNA from kidney, uterus, and gonads, there is an approximately 2.5-kb WT1-related mRNA species in testis. The levels of WT1 mRNA in the gonads are among the highest observed, surpassing amounts detected in the embryonic kidney. During development, these levels are differentially regulated, depending on the sexual differentiation of the gonad. Expression of WT1 mRNA in the female reproductive system does not fluctuate significantly from days 4 to 40 postpartum. In contrast, WT1 mRNA levels in the tesis increase steadily after birth, reaching their highest expression levels at day 8 postpartum and decreasing slightly as the animal matures. Expression of WT1 in the gonads is detectable as early as 12.5 days postcoitum (p.c.). As an initial step toward exploring the tissue-specific expression of WT1, DNA elements upstream of WT1 were cloned and sequenced. Three putative transcription initiation sites, utilized in testis, ovaries, and uterus, were mapped by S1 nuclease protection assays. The sequences surrounding these sites have a high G + C content, and typical upstream CCAAT and TATAA boxes are not present. These studies allowed us to identify the translation initiation site for WT1 protein synthesis. We have also used an epitope-tagging protocol to demonstrate that WT1 is a nuclear protein, consistent with its role as a transcription factor. Our results demonstrate regulation of WT1 expression

Tumor-specific loss of 11p15.5 alleles in del11p13 Wilms tumor and in familial adrenocortical carcinoma

International Nuclear Information System (INIS)

Henry, I.; Grandjouan, S.; Couillin, P.

1989-01-01

The authors have compared constitutional and tumor genotypes in nine cases of hereditary Wilms tumor (WT) and in three unrelated cases of familial adrenocortical carcinoma (ADCC). Since susceptibility to these tumors can be observed in malformation syndromes associated with a constitutional deletion of band 11p13 (WT) and with a constitutional duplication of band 11p15.5 (WT, ADCC), they investigated these two candidate regions by using 11p polymorphic markers. As expected, somatic chromosomal events, resulting in a loss of heterozygosity limited to region 11p15.5, were observed in the tumor of two familial cases of adrenocortical carcinoma. Surprisingly, however, analysis of the WT of two patients with a constitutional deletion of band 11p13, associated with aniridia, genitourinary abnormalities, and mental retardation (WAGR syndrome), revealed a loss of heterozygosity limited to region 11p15.5. These data therefore suggest that observation of a specific loss of heterozygosity may not necessarily point to the site of the initial germinal mutation. Together with previous similar observations of a loss of heterozygosity limited to 11p15.5 in breast cancer and in rhabdomyosarcoma, the data suggest that region 11p15.5 may carry a non-tissue-specific gene that could be involved in genetic predisposition, in tumor progression, or in both

Combining miRNA and mRNA Expression Profiles in Wilms Tumor Subtypes

Directory of Open Access Journals (Sweden)

Nicole Ludwig

2016-03-01

Full Text Available Wilms tumor (WT is the most common childhood renal cancer. Recent findings of mutations in microRNA (miRNA processing proteins suggest a pivotal role of miRNAs in WT genesis. We performed miRNA expression profiling of 36 WTs of different subtypes and four normal kidney tissues using microarrays. Additionally, we determined the gene expression profile of 28 of these tumors to identify potentially correlated target genes and affected pathways. We identified 85 miRNAs and 2107 messenger RNAs (mRNA differentially expressed in blastemal WT, and 266 miRNAs and 1267 mRNAs differentially expressed in regressive subtype. The hierarchical clustering of the samples, using either the miRNA or mRNA profile, showed the clear separation of WT from normal kidney samples, but the miRNA pattern yielded better separation of WT subtypes. A correlation analysis of the deregulated miRNA and mRNAs identified 13,026 miRNA/mRNA pairs with inversely correlated expression, of which 2844 are potential interactions of miRNA and their predicted mRNA targets. We found significant upregulation of miRNAs-183, -301a/b and -335 for the blastemal subtype, and miRNAs-181b, -223 and -630 for the regressive subtype. We found marked deregulation of miRNAs regulating epithelial to mesenchymal transition, especially in the blastemal subtype, and miRNAs influencing chemosensitivity, especially in regressive subtypes. Further research is needed to assess the influence of preoperative chemotherapy and tumor infiltrating lymphocytes on the miRNA and mRNA patterns in WT.

Teratoid Wilms tumour with chemotherapy resistance

Directory of Open Access Journals (Sweden)

Renuka Gahine

2015-01-01

Full Text Available We present a case of Teratoid Wilms tumour (a rare histologic variant in a 4 year old male who presented with an abdominal lump. Wilms Tumour with paracaval lymphadenopathy and tumour thrombi in right renal vein and inferior vena cava was made radiologically. FNAC report was suggestive of Wilms tumour and patient was subjected to 6 cycles of chemotherapy with not much reduction in size. Post nephrectomy histological diagnosis of Teratoid Wilms tumour was established. Resistance to chemotherapy and radiotherapy is thought to be due to presence of well differentiated histologic appearance. Teratoid Wilms tumour is usually not an aggressive neoplasm and prognosis is comparatively neoplasm and prognosis is comparatively good if the tumour is excised completely thus surgery being the best treatment.

Role of CD56 in Normal Kidney Development and Wilms Tumorigenesis

DEFF Research Database (Denmark)

Yap, Li-Wei; Brok, Jesper; Pritchard-Jones, Kathy

2017-01-01

The cell-surface glycoprotein CD56 has three major isoforms that play important roles in cell adhesion and signaling, which may promote cell proliferation, differentiation, survival, or migration. It is an important molecule in normal kidney development and acts as a key marker in Wilms tumor stem...

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

Constitutional and somatic methylation status of DMRH19 and KvDMR in Wilms tumor patients

Directory of Open Access Journals (Sweden)

Leila C.A. Cardoso

2012-01-01

Full Text Available The most frequent epigenetic alterations in Wilms tumor (WT occur at WT2, assigned to 11p15. WT2 consists of two domains: telomeric domain 1 (DMRH19 that contains the IGF2 gene and an imprinted maternally expressed transcript (H19 and centromeric domain 2 (KvDMR that contains the genes KCNQ1, KCNQ1OT1 and CDKN1C. In this work, we used pyrosequencing and MS-MLPA to compare the methylation patterns of DMRH19/KvDMR in blood and tumor samples from 40 WT patients. Normal constitutional KvDMR methylation indicated that most of the epigenetic alterations in WT occur at DMRH19. Constitutional DMRH19 hypermethylation (HM DMRH19 was observed in two patients with Beckwith-Wiedemann syndrome. Pyrosequencing and MS-MLPA showed HM DMRH19 in 28/34 tumor samples: 16/34 with isolated HM DMRH19 and 12/34 with concomitant HM DMRH19 and KvDMR hypomethylation, indicating paternal uniparental disomy. With the exception of one blood sample, the MS-MLPA and pyrosequencing findings were concordant. Diffuse or focal anaplasia was present in five tumor samples and was associated with isolated somatic HM DMRH19 in four of them. Constitutional 11p15 methylation abnormalities were present in 5% of the samples and somatic abnormalities in the majority of tumors. Combined analysis of DMRH19/KvDMR by pyrosequencing and MS-MLPA is beneficial for characterizing epigenetic anomalies in WT, and MS-MLPA is useful and reliable for estimation of DNA methylation in a clinical setting.

Outcomes of Patients With Revised Stage I Clear Cell Sarcoma of Kidney Treated in National Wilms Tumor Studies 1-5

International Nuclear Information System (INIS)

Kalapurakal, John A.; Perlman, Elizabeth J.; Seibel, Nita L.; Ritchey, Michael; Dome, Jeffrey S.; Grundy, Paul E.

2013-01-01

Purpose: To report the clinical outcomes of children with revised stage I clear cell sarcoma of the kidney (CCSK) using the National Wilms Tumor Study Group (NWTS)-5 staging criteria after multimodality treatment on NWTS 1-5 protocols. Methods and Materials: All CCSK patients enrolled in the National Wilms Tumor Study Group protocols had their pathology slides reviewed, and only those determined to have revised stage I tumors according to the NWTS-5 staging criteria were included in the present analysis. All patients were treated with multimodality therapy according to the NWTS 1-5 protocols. Results: A total of 53 children were identified as having stage I CCSK. All patients underwent primary surgery with radical nephrectomy. The chemotherapy regimens used were as follows: regimen A, C, F, or EE in 4 children (8%); regimen DD or DD4A in 33 children (62%); regimen J in 4 children (8%); and regimen I in 12 children (22%). Forty-six patients (87%) received flank radiation therapy (RT). Seven children (13%) did not receive flank RT. The median delay between surgery and the initiation of RT was 9 days (range, 3-61). The median RT dose was 10.8 Gy (range, 10-36). The flank RT doses were as follows: 10.5 or 10.8 Gy in 25 patients (47%), 11-19.9 Gy in 2 patients (4%), 20-29.9 Gy in 9 patients (17%), and 30-40 Gy in 10 patients (19%). The median follow-up for the entire group was 17 years (range, 2-36). The relapse-free and cancer-specific survival rate was 100% at the last follow-up examination. Conclusions: The present results have demonstrated that children with revised stage I CCSK using the NWTS-5 staging criteria have excellent survival rates despite the use of varying RT doses and chemotherapy regimens in the NWTS 1-5 protocols.

Monitoring therapy with MEK inhibitor U0126 in a novel Wilms tumor model in Wt1 knockout Igf2 transgenic mice using 18F-FDG PET with dual-contrast enhanced CT and MRI: early metabolic response without inhibition of tumor growth.

Science.gov (United States)

Flores, Leo G; Yeh, Hsin-Hsien; Soghomonyan, Suren; Young, Daniel; Bankson, James; Hu, Qianghua; Alauddin, Mian; Huff, Vicki; Gelovani, Juri G

2013-04-01

The understanding of the role of genetic alterations in Wilms tumor development could be greatly advanced using a genetically engineered mouse models that can replicate the development and progression of this disease in human patients and can be monitored using non-invasive structural and molecular imaging optimized for renal tumors. Repetitive dual-contrast computed tomography (CT; intravenous and intraperitoneal contrast), T2-weighted magnetic resonance imaging (MRI), and delayed 2-deoxy-2-[(18)F]fluoro-D-glucose ((18)F-FDG) positron emission tomography (PET) were utilized for characterization of Igf2 biallelic expression/Wt1 knockout mouse model of Wilms tumor. For CT imaging, Ioversol 678 mg/ml in 200 μl was administered i.p. followed by 100 μl injected intravenously at 20 and 15 min prior to imaging, respectively. Static PET imaging studies were acquired at 1, 2, and 3 h after i.v. administration of (18)F-FDG (400 μCi). Coronal and sagittal T1-weighted images (TE/TR 8.5/620 ms) were acquired before and immediately after i.v. injection of 0.4 ml/kg gadopentetate dimeglumine followed by T2-weighted images (TE/TR 60/300 ms). Tumor tissue samples were characterized by histopathology and immunohistochemistry for Glut1, FASN, Ki67, and CD34. In addition, six Wt1-Igf2 mice were treated with a mitogen-activated protein kinase (MEK) inhibitor U0126 (50 μmol/kg i.p.) every 4 days for 6 weeks. (18)F-FDG PET/CT imaging was repeated at different days after initiation of therapy with U0126. The percent change of initial tumor volume and SUV was compared to non-treated historic control animals. Overall, the best tumor-to-adjacent kidney contrast as well as soft tissue contrast for other abdominal organs was achieved using T2-weighted MRI. Delayed (18)F-FDG PET (3-h post (18)F-FDG administration) and dual-contrast CT (intravenous and intraperitoneal contrast) provided a more accurate anatomic and metabolic characterization of Wilms tumors in Wt1-Igf2 mice

Nephron sparing surgery (NSS) for unilateral wilms tumor (UWT): the SIOP 2001 experience.

Science.gov (United States)

Wilde, Jim C H; Aronson, Daniel C; Sznajder, Beata; Van Tinteren, Harm; Powis, Mark; Okoye, Bruce; Cecchetto, Giovanni; Audry, Georges; Fuchs, Jörg; Schweinitz, Dietrich Von; Heij, Hugo; Graf, Norbert; Bergeron, Christophe; Pritchard-Jones, Kathy; Van Den Heuvel-Eibrink, Marry; Carli, Modesto; Oldenburger, Foppe; Sandstedt, Bengt; De Kraker, Jan; Godzinski, Jan

2014-12-01

Total nephrectomy (TN) remains the standard treatment of unilateral Wilms tumors (uWT). The SIOP WT-2001 protocol allowed Nephron Sparing Surgery (NSS) for polar or peripherally non-infiltrating tumors. Inventory of the current SIOP NSS-experience. 2,800 patients with a unilateral, localized or metastatic and an unequivocal surgical technique recorded were included. All had neo-adjuvant chemotherapy and delayed surgery. In 91 (3%) NSS was performed and in 2709 TN. Data was retrieved from the SIOP WT 2001 database. NSS group contained 65% stage I tumours and the TN group 48%. Tumor volume (at diagnosis and surgery) was significantly smaller in the NSS group. Within stage III, after NSS, 7/12 (58%) had positive margins (M +), 5 with tumor negative lymph nodes (LN-). After TN, 355/712 (55%) had M + , 182 were LN-. Treatment of M+ in the NSS group resulted in two conversions to TN (one combined with radiotherapy), three patients had radiotherapy only and in two patients local therapy, if given, was not recorded. After NSS, four recurrences occurred. For localized disease the 5-year overall (OS) and event free survival (EFS) in NSS group was 100 and 94.8 (95% CI:89.9-99.9), respectively, while OS and EFS in the TN group were 94.4 (95% CI: 93.2-95.5, log-rank test P = 0.06) and 86.5 (95% CI:85.0-88.1, log-rank test P = 0.06), respectively. NSS was only performed in 3% of patients with uWT. Despite excellent survival with few relapses, the gain of nephrons needs to be weighed against the risk to induce stage III with intensified therapy. © 2014 Wiley Periodicals, Inc.

Sinusoidal Obstruction Syndrome during Treatment for Wilms' Tumor: A Life-threatening Complication.

Science.gov (United States)

Totadri, Sidharth; Trehan, Amita; Bansal, Deepak; Jain, Richa

2017-01-01

Survival rates exceed 90% in Wilms' tumor (WT). Actinomycin-D (ACT-D) which is indispensable in the management of WT is associated with the development of sinusoidal obstruction syndrome (SOS), a potentially fatal complication. The aim is to study the presentation, management, and outcome of SOS complicating ACT-D administration in WT. Retrospective file review conducted in a Pediatric Hematology-Oncology unit. Patients diagnosed and treated for WT from January 2012 to December 2015 were analyzed. SOS was diagnosed clinically, based on McDonalds criteria, requiring two of the following: jaundice, hepatomegaly and/or right upper quadrant pain, weight gain with or without ascites. Of 104 patients treated, SOS occurred in 5 (4.8%). Age: 6 months to 5 years, 3 were girls. Tumor involved left kidney in 3, right in 1 and a horseshoe kidney in 1. Histopathology was consistent with WT in 4 and clear cell sarcoma kidney in 1. One had pulmonary metastases. Three developed SOS preoperatively and two during adjuvant chemotherapy. None received radiotherapy. Clinical manifestations comprised of jaundice, hepatomegaly, ascites/weight gain, respiratory distress, hypotension, and encephalopathy. Laboratory findings included thrombocytopenia, elevated serum transaminases, and coagulopathy. Treatment included fluid restriction, broad spectrum antibiotics, and transfusional support. Two children received N-acetyl cysteine infusion. Defibrotide was administered to two patients. Four recovered and one succumbed to multi-organ failure. Two patients were safely re-challenged with 50% doses of ACT-D. SOS is a clinical diagnosis. Systematic supportive care can enable complete recovery. Under close monitoring, re-challenge of ACT-D can be performed in gradually escalating doses.

Syndromes and constitutional chromosomal abnormalities associated with Wilms tumour

Science.gov (United States)

Scott, R H; Stiller, C A; Walker, L; Rahman, N

2006-01-01

Wilms tumour has been reported in association with over 50 different clinical conditions and several abnormal constitutional karyotypes. Conclusive evidence of an increased risk of Wilms tumour exists for only a minority of these conditions, including WT1 associated syndromes, familial Wilms tumour, and certain overgrowth conditions such as Beckwith‐Wiedemann syndrome. In many reported conditions the rare co‐occurrence of Wilms tumour is probably due to chance. However, for several conditions the available evidence cannot either confirm or exclude an increased risk, usually because of the rarity of the syndrome. In addition, emerging evidence suggests that an increased risk of Wilms tumour occurs only in a subset of individuals for some syndromes. The complex clinical and molecular heterogeneity of disorders associated with Wilms tumour, together with the apparent absence of functional links between most of the known predisposition genes, suggests that abrogation of a variety of pathways can promote Wilms tumorigenesis. PMID:16690728

SU-F-T-117: A Pilot Study of Organ Dose Reconstruction for Wilms Tumor Patients Treated with Radiation Therapy

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Makkia, R; Pelletier, C; Jung, J [East Carolina University, Greenville, NC (United States); Gopalakrishnan, M [Northwestern Memorial Hospital, Chicago, IL (United States); Lee, C [University of Michigan, Ann Arbor, MI (United States); Mille, M; Lee, C [National Cancer Institute, Rockville, MD (United States); Kalapurakal, J [Northwestern University, Chicago, IL (United States)

2016-06-15

Purpose: To reconstruct major organ doses for the Wilms tumor pediatric patients treated with radiation therapy using pediatric computational phantoms, treatment planning system (TPS), and Monte Carlo (MC) dose calculation methods. Methods: A total of ten female and male pediatric patients (15–88 months old) were selected from the National Wilms Tumor Study cohort and ten pediatric computational phantoms corresponding to the patient’s height and weight were selected for the organ dose reconstruction. Treatment plans were reconstructed on the computational phantoms in a Pinnacle TPS (v9.10) referring to treatment records and exported into DICOM-RT files, which were then used to generate the input files for XVMC MC code. The mean doses to major organs and the dose received by 50% of the heart were calculated and compared between TPS and MC calculations. The same calculations were conducted by replacing the computational human phantoms with a series of diagnostic patient CT images selected by matching the height and weight of the patients to validate the anatomical accuracy of the computational phantoms. Results: Dose to organs located within the treatment fields from the computational phantoms and the diagnostic patient CT images agreed within 2% for all cases for both TPS and MC calculations. The maximum difference of organ doses was 55.9 % (thyroid), but the absolute dose difference in this case was 0.33 Gy which was 0.96% of the prescription dose. The doses to ovaries and testes from MC in out-of-field provided more discrepancy (the maximum difference of 13.2% and 50.8%, respectively). The maximum difference of the 50% heart volume dose between the phantoms and the patient CT images was 40.0%. Conclusion: This study showed the pediatric computational phantoms are applicable to organ doses reconstruction for the radiotherapy patients whose three-dimensional radiological images are not available.

Sinusoidal Obstruction Syndrome during Treatment for Wilms' Tumor: A Life-threatening Complication

Science.gov (United States)

Totadri, Sidharth; Trehan, Amita; Bansal, Deepak; Jain, Richa

2017-01-01

Context: Survival rates exceed 90% in Wilms' tumor (WT). Actinomycin-D (ACT-D) which is indispensable in the management of WT is associated with the development of sinusoidal obstruction syndrome (SOS), a potentially fatal complication. Aims: The aim is to study the presentation, management, and outcome of SOS complicating ACT-D administration in WT. Settings and Design: Retrospective file review conducted in a Pediatric Hematology-Oncology unit. Materials and Methods: Patients diagnosed and treated for WT from January 2012 to December 2015 were analyzed. SOS was diagnosed clinically, based on McDonalds criteria, requiring two of the following: jaundice, hepatomegaly and/or right upper quadrant pain, weight gain with or without ascites. Results: Of 104 patients treated, SOS occurred in 5 (4.8%). Age: 6 months to 5 years, 3 were girls. Tumor involved left kidney in 3, right in 1 and a horseshoe kidney in 1. Histopathology was consistent with WT in 4 and clear cell sarcoma kidney in 1. One had pulmonary metastases. Three developed SOS preoperatively and two during adjuvant chemotherapy. None received radiotherapy. Clinical manifestations comprised of jaundice, hepatomegaly, ascites/weight gain, respiratory distress, hypotension, and encephalopathy. Laboratory findings included thrombocytopenia, elevated serum transaminases, and coagulopathy. Treatment included fluid restriction, broad spectrum antibiotics, and transfusional support. Two children received N-acetyl cysteine infusion. Defibrotide was administered to two patients. Four recovered and one succumbed to multi-organ failure. Two patients were safely re-challenged with 50% doses of ACT-D. Conclusions: SOS is a clinical diagnosis. Systematic supportive care can enable complete recovery. Under close monitoring, re-challenge of ACT-D can be performed in gradually escalating doses. PMID:29333010

The Perlman syndrome: familial renal dysplasia with Wilms tumor, fetal gigantism and multiple congenital anomalies. 1984.

Science.gov (United States)

Neri, Giovanni; Martini-Neri, Maria Enrica; Katz, Ben E; Opitz, John M

2013-11-01

The ensuing paper by Professor Giovanni Neri and colleagues was originally published in 1984, American Journal of Medical Genetics 19:195–207. The original article described a new family with a condition that the authors designated as the Perlman syndrome. This disorder, while uncommon, is an important multiple congenital anomaly and dysplasia syndrome; the causative gene was recently identified. This paper is a seminal work and is graciously republished by Wiley-Blackwell in the Special Festschrift issue honoring Professor Neri. We describe a familial syndrome of renal dysplasia, Wilms tumor, hyperplasia of the endocrine pancreas, fetal gigantism, multiple congenital anomalies and mental retardation. This condition was previously described by Perlman et al. [1973, 1975] and we propose to call it the "Perlman syndrome." It appears to be transmitted as an autosomal recessive trait. The possible relationships between dysplasia, neoplasia and malformation are discussed. © 2013 Wiley Periodicals, Inc.

Genetic changes of two Wilms tumors with anaplasia and a review of the literature suggesting a marker profile for therapy resistance.

Science.gov (United States)

Stock, Cornelia; Ambros, Inge M; Lion, Thomas; Zoubek, Andreas; Amann, Gabriele; Gadner, Helmut; Ambros, Peter F

2002-06-01

Cytogenetic data on Wilms tumors (WT) with anaplasia frequently associated with an unfavorable outcome are scarce. We present cytogenetic changes of two WT with anaplasia (primary tumor material) from nonresponders with a synopsis of the literature. The WT were investigated by cytogenetic analysis, comparative genomic hybridization, fluorescence in situ hybridization, immunofluorescence, and flow cytometric analyses. Both tumors exhibited characteristic genetic changes. One tumor was hypodiploid due to loss of entire chromosome 11; losses of 16p, 16q, 17p, chromosome 19 material, and loss of 22q12-qter. The other tumor was hyperdiploid and triploid, and displayed gain of 1q12-q23 and chromosome 9 material. Moreover, two morphological and genetically distinct cell lines have been established from both tumors, demonstrating underrepresentation of chromosomes 13, 14, 16, and 19. Karyotype descriptions of 120 WT with known clinical data together with data of this report confirm: (1) inter- and intratumor heterogeneity exists; (2) loss or underrepresentation of chromosome material at 11, 13, 14, 16, 17p, 19, and 22q in various combinations presents a new marker profile of resistance to cytotoxic agents regardless of the histological types; and (3) the prognostic impact of gain at 1q12-q23 sequences warrants further validation.

Bloom syndrome in sibs: first reports of hepatocellular carcinoma and Wilms tumor with documented anaplasia and nephrogenic rests.

Science.gov (United States)

Jain, D; Hui, P; McNamara, J; Schwartz, D; German, J; Reyes-Múgica, M

2001-01-01

The triad of small body size, immunodeficiency, and sun-sensitive facial erythema characterizes the phenotype Bloom syndrome (BS), a rare autosomal recessive disorder with a striking predisposition to multiple types of cancers that arise earlier than expected in the general population. Here we report two sibs with BS. The older, a 15-year-old-girl, developed a hepatocellular carcinoma, a neoplasm not yet reported in association with BS. Her younger brother developed an anaplastic Wilms tumor (WT) associated with nephrogenic rests at the age of 31/2 years, and this was followed by a myelodysplastic syndrome. Complex cytogenetic abnormalities were identified in all three neoplasms. These examples expand the spectrum of malignancies occurring in BS to include liver cell neoplasms, and confirm the association of nephrogenic rests with WT, even in the setting of BS.

Wilms Tumor 1b defines a wound-specific sheath cell subpopulation associated with notochord repair

Science.gov (United States)

Lopez-Baez, Juan Carlos; Zeng, Zhiqiang; Brunsdon, Hannah; Salzano, Angela; Brombin, Alessandro; Wyatt, Cameron; Rybski, Witold; Huitema, Leonie F A; Dale, Rodney M; Kawakami, Koichi; Englert, Christoph; Chandra, Tamir; Schulte-Merker, Stefan

2018-01-01

Regenerative therapy for degenerative spine disorders requires the identification of cells that can slow down and possibly reverse degenerative processes. Here, we identify an unanticipated wound-specific notochord sheath cell subpopulation that expresses Wilms Tumor (WT) 1b following injury in zebrafish. We show that localized damage leads to Wt1b expression in sheath cells, and that wt1b+cells migrate into the wound to form a stopper-like structure, likely to maintain structural integrity. Wt1b+sheath cells are distinct in expressing cartilage and vacuolar genes, and in repressing a Wt1b-p53 transcriptional programme. At the wound, wt1b+and entpd5+ cells constitute separate, tightly-associated subpopulations. Surprisingly, wt1b expression at the site of injury is maintained even into adult stages in developing vertebrae, which form in an untypical manner via a cartilage intermediate. Given that notochord cells are retained in adult intervertebral discs, the identification of novel subpopulations may have important implications for regenerative spine disorder treatments. PMID:29405914

Aflac ST0901 CHOANOME - Sirolimus in Solid Tumors

Science.gov (United States)

2018-05-15

Ewing's Sarcoma; Osteosarcoma; Astrocytoma; Atypical Teratoid/Rhabdoid Tumor; Ependymoma; Germ Cell Tumor; Glioma; Medulloblastoma; Rhabdoid Tumor; Retinoblastoma; Clear Cell Sarcoma; Renal Cell Carcinoma; Wilms Tumor; Hepatoblastoma; Neuroblastoma; Rhabdomyosarcoma

Frequency of WT1 and 11p15 constitutional aberrations and phenotypic correlation in childhood Wilms tumour patients.

Science.gov (United States)

Segers, H; Kersseboom, R; Alders, M; Pieters, R; Wagner, A; van den Heuvel-Eibrink, M M

2012-11-01

In 9-17% of Wilms tumour patients a predisposing syndrome is present, in particular WT1-associated syndromes and overgrowth syndromes. Constitutional WT1 mutations or epigenetic changes on chromosome 11p15 have also been described in Wilms tumour patients without phenotypic abnormalities. Thus, the absence of phenotypic abnormalities does not exclude the presence of a genetic predisposition, suggesting that more Wilms tumour patients may have a constitutional abnormality. Therefore, we investigated the frequency of constitutional aberrations in combination with phenotype. Clinical genetic assessment, as well as molecular analysis of WT1 and locus 11p15 was offered to a single-centre cohort of 109 childhood Wilms tumour patients. Twelve patients (11%) had a WT1 aberration and eight patients (8%) had an 11p15 aberration. Of the 12 patients with a WT1 aberration, four had WAGR syndrome (Wilms tumor, aniridia, genitourinary malformations and mental retardation), one had Denys-Drash syndrome, four had genitourinary anomalies without other syndromic features and three had bilateral disease with stromal-predominant histology at young age without congenital anomalies. Of the eight patients with an 11p15 aberration, four had Beckwith-Wiedemann syndrome (BWS), two had minor features of BWS and two had no stigmata of BWS or hemihypertrophy. Constitutional WT1 or 11p15 aberrations are frequent in Wilms tumour patients and careful clinical assessment can identify the majority of these patients. Therefore, we would recommend offering clinical genetic counselling to all Wilms tumour patients, as well as molecular analysis to patients with clinical signs of a syndrome or with features that may indicate a constitutional WT1 or 11p15 aberration. Copyright © 2012 Elsevier Ltd. All rights reserved.

Pattern of malignant tumors in children: a hospital based study

International Nuclear Information System (INIS)

Khan, S.M.; Nasreen, S.; Zai, S.

2001-01-01

From 1990 to 1999 data from 32743 cancer patients (males 18502, females 14241) were analyzed to know the frequency of the most common cancers in local and well as well as afghan refugees. There were 3760 children with biopsy proven cancers 2910 belonged to the north-west frontier province (NWFP), while the remaining 850 were Afghan refugees. Among children of NWFP male were 1945 (67%) and 965(33%) females. In Afghan children, males were 570(67%) and females were 280(33%). The most common tumors in children of NWFP were lymphoid leukemia, lymphoma, tumors of the central nervous system (CNS), myeloid leukemia, soft tissue sarcoma wilms, tumours, retinoblastoma, bone tumor neuroblastoma, and ovarian tumors. Whereas Afghan children had Lymphoid leukemia, lymphoma, myeloid leukemia, wilms, tumor, retinoblastoma, tumors of soft tissue bones CNS, neuroblastoma and ovarian tumors. (author)

Bone metastases in Wilms' tumour - report of three cases and review of literature

International Nuclear Information System (INIS)

Gururangan, S.; Wilimas, J.A.; Fletcher, B.D.

1994-01-01

Bone metastases are extremely rare in patients with classical Wilms' tumor (WT). We describe the clinical and radiologic features, treatment and outcome of three patients with WT (one with favorable histology and two with anaplasia) in whom bone metastases were detected at diagnosis or relapse. Bone metastases were documented by skeletal radiographs, computed tomography and/or bone scintigraphy. The patient with favourable histology WT had no evidence of pulmonary metastases and is now free of disease following aggressive chemotherapy and radiotherapy. (orig.)

Wilms' Tumor 1 Overexpression in Granulosa Cells Is Associated with Polycystic Ovaries in Polycystic Ovary Syndrome Patients.

Science.gov (United States)

Wang, Qun; Huang, Tao; Shu, Xin; Zhao, Shi-Gang; Liang, Yu; Muhammad, Tahir; Gao, Fei; Zhao, Han; Liu, Hong-Bin

2018-01-01

Polycystic ovary syndrome (PCOS) is a heterogeneous disorder characterized by chronic ovulatory dysfunction, hyperandrogenism, and polycystic ovaries. Wilms' tumor 1 (WT1) encoding a transcription factor involved in the differentiation of granulosa cells (GCs) regulates androgen receptor in the development of male genitalia. However, the expression pattern and possible role of WT1 in ovaries of PCOS patients are still unknown. GCs from 95 PCOS patients (PCOS group) and 62 healthy controls (control group) were isolated. The expression of WT1 in GCs was quantified using the reverse transcription-polymerase chain reaction. The correlation between WT1 expression and clinical characteristics was evaluated in PCOS patients. WT1 expression was increased in PCOS patients compared with the normal controls. The expression of WT1 was moderately correlated with testosterone (r = 0.334, p = 0.001) and luteinizing hormone (r = 0.357, p = 0.001) levels and the antral follicle counts (r = 0.337, p = 0.001). Our study provided novel insights into the relationship between hyperandrogenism and polycystic ovaries of PCOS and WT1. © 2018 S. Karger AG, Basel.

Bioanalysis of a panel of neurotransmitters and their metabolites in plasma samples obtained from pediatric patients with neuroblastoma and Wilms' tumor.

Science.gov (United States)

Konieczna, Lucyna; Roszkowska, Anna; Stachowicz-Stencel, Teresa; Synakiewicz, Anna; Bączek, Tomasz

2018-02-01

This paper details the quantitative analysis of neurotransmitters, including dopamine (DA), norepinephrine (NE), epinephrine (E), and serotonin (5-HT), along with their respective precursors and metabolites in children with solid tumors: Wilms' tumor (WT) and neuroblastoma (NB). A panel of neurotransmitters was determined with the use of dispersive liquid-liquid microextraction (DLLME) technique combined with liquid-chromatography mass spectrometry (LC-MS/MS) in plasma samples obtained from a group of pediatric subjects with solid tumors and a control group of healthy children. Next, statistical univariate analysis (t-test) and multivariate analysis (Principal Component Analysis) were performed using chromatographic data. The levels of tyrosine (Tyr) and tryptophan (Trp) (the precursors of analyzed neurotransmitters) as well as 3,4-dihydroxyphenylacetic acid (DOPAC) (a product of metabolism of DA) were significantly higher in the plasma samples obtained from pediatric patients with WT than in the samples taken from the control group. Moreover, statistically significant differences were observed between the levels of 5-HT and homovanillic acid (HVA) in the plasma samples from pediatric patients with solid tumors and the control group. However, elevated levels of these analytes did not facilitate a clear distinction between pediatric patients with WT and those with NB. Nonetheless, the application of advanced statistical tools allowed the healthy controls to be differentiated from the pediatric oncological patients. The identification and quantification of a panel of neurotransmitters as potential prognostic factors in selected childhood malignancies may provide clinically relevant information about ongoing metabolic alterations, and it could potentially serve as an adjunctive strategy in the effective diagnosis and treatment of solid tumors in children. Copyright © 2017 Elsevier B.V. All rights reserved.

Genetics of Beckwith-Wiedemann syndrome-associated tumors: common genetic pathways

NARCIS (Netherlands)

Steenman, M.; Westerveld, A.; Mannens, M.

2000-01-01

A specific subset of solid childhood tumors-Wilms' tumor, adrenocortical carcinoma, rhabdomyosarcoma, and hepatoblastoma-is characterized by its association with Beckwith-Wiedemann syndrome. Genetic abnormalities found in these tumors affect the same chromosome region (11p15), which has been

Molecular cytogenetic anomalies and phenotype alterations in a newly established cell line from Wilms tumor with diffuse anaplasia.

Science.gov (United States)

Faussillon, Marine; Murakami, Ichiro; Bichat, Magalie; Telvi, Louise; Jeanpierre, Cécile; Nezelof, Christian; Jaubert, Francis; Gogusev, Jean

2008-07-01

The novel continuous cell line WT-Pe.1 was established in vitro from Wilms tumor with histological features of diffuse anaplasia. The cultures grew as poorly differentiated epithelial-like cells with pleomorphic polygonal shapes and formation of typical monolayers. WT-Pe.1 cells were immunoreactive for cytokeratin, vimentin, laminin, villin, CD10, and CD24 proteins. Conventional cytogenetic analysis by RHG-banding revealed a hypotriploid karyotype with numerous abnormalities including ring chromosomes, double-minutes, homogeneous staining regions, radial structures, dicentrics, and several marker chromosomes. Comparative genomic hybridization analysis revealed DNA copy numbers losses on chromosome segments 1p, 3p, 6q, 9q34.1 approximately q34.3, 11q24 approximately q25, 14q12 approximately qter, 16q, 18q, and 22q11 approximately q13; gain of genomic material was localized on chromosome arms 1q, 4p, 6q, and 7p and the entire chromosome 12. With DNA from the original tumor, copy number losses were detected on chromosomes 1p, 14q, 16q, 17q, and 22q and gains were observed on 1q, 4p, 8q, 12p, 12q, and chromosome 14p. Copy number amplifications of distinct loci were found on 1q21.1 and 4p15.3, as well as an elevated copy number of cyclin D2 (CCND2) and cyclin D associated kinase (CDK4) genes on chromosome 12 (confirmed by fluorescence in situ hybridization).

Bone metastases in Wilms' tumour - report of three cases and review of literature

Energy Technology Data Exchange (ETDEWEB)

Gururangan, S. (Dept. of Hematology-Oncology, St. Jude Children' s Research Hospital, Memphis, TN (United States) Dept. of Pediatrics, Tennessee Univ., Memphis, TN (United States)); Wilimas, J.A. (Dept. of Hematology-Oncology, St. Jude Children' s Research Hospital, Memphis, TN (United States) Dept. of Pediatrics, Tennessee Univ., Memphis, TN (United States)); Fletcher, B.D. (Dept. of Pediatrics, Tennessee Univ., Memphis, TN (United States) Dept. of Diagnostic Imaging, St. Jude Children' s Research Hospital, Memphis, TN (United States) Dept. of Radiology, Tennessee Univ., Memphis, TN (United States))

1994-04-01

Bone metastases are extremely rare in patients with classical Wilms' tumor (WT). We describe the clinical and radiologic features, treatment and outcome of three patients with WT (one with favorable histology and two with anaplasia) in whom bone metastases were detected at diagnosis or relapse. Bone metastases were documented by skeletal radiographs, computed tomography and/or bone scintigraphy. The patient with favourable histology WT had no evidence of pulmonary metastases and is now free of disease following aggressive chemotherapy and radiotherapy. (orig.)

MLLT1 YEATS domain mutations in clinically distinctive Favourable Histology Wilms tumours

KAUST Repository

Perlman, Elizabeth J.; Gadd, Samantha; Arold, Stefan T.; Radhakrishnan, Anand; Gerhard, Daniela S.; Jennings, Lawrence; Huff, Vicki; Guidry Auvil, Jaime M.; Davidsen, Tanja M.; Dome, Jeffrey S.; Meerzaman, Daoud; Hsu, Chih Hao; Nguyen, Cu; Anderson, James; Ma, Yussanne; Mungall, Andrew J; Moore, Richard A.; Marra, Marco A.; Mullighan, Charles G; Ma, Jing; Wheeler, David A.; Hampton, Oliver A.; Gastier-Foster, Julie M.; Ross, Nicole; Smith, Malcolm A.

2015-01-01

Wilms tumour is an embryonal tumour of childhood that closely resembles the developing kidney. Genomic changes responsible for the development of the majority of Wilms tumours remain largely unknown. Here we identify recurrent mutations within Wilms tumours that involve the highly conserved YEATS domain of MLLT1 (ENL), a gene known to be involved in transcriptional elongation during early development. The mutant MLLT1 protein shows altered binding to acetylated histone tails. Moreover, MLLT1-mutant tumours show an increase in MYC gene expression and HOX dysregulation. Patients with MLLT1-mutant tumours present at a younger age and have a high prevalence of precursor intralobar nephrogenic rests. These data support a model whereby activating MLLT1 mutations early in renal development result in the development of Wilms tumour.

MLLT1 YEATS domain mutations in clinically distinctive Favourable Histology Wilms tumours

KAUST Repository

Perlman, Elizabeth J.

2015-12-04

Wilms tumour is an embryonal tumour of childhood that closely resembles the developing kidney. Genomic changes responsible for the development of the majority of Wilms tumours remain largely unknown. Here we identify recurrent mutations within Wilms tumours that involve the highly conserved YEATS domain of MLLT1 (ENL), a gene known to be involved in transcriptional elongation during early development. The mutant MLLT1 protein shows altered binding to acetylated histone tails. Moreover, MLLT1-mutant tumours show an increase in MYC gene expression and HOX dysregulation. Patients with MLLT1-mutant tumours present at a younger age and have a high prevalence of precursor intralobar nephrogenic rests. These data support a model whereby activating MLLT1 mutations early in renal development result in the development of Wilms tumour.

Incidence and Curability of Tumors in Childhood in Slovakia

International Nuclear Information System (INIS)

Kaiserova, E.; Subova, Z.; Bubanska, E.; Stancokova, T.; Oravkinova, I.; Plank, L.

2006-01-01

Annual incidence of cancer in Slovak Republic for children 0 - 14 years old in 1991 - 2002 was 115,2 - 143 per million and for adolescents 15 - 19 years old 156 - 196 per million, in which mild increase was observed. Most often malignancies in children have been acute lymphoblastic leukemia and tumors of central nervous system. For children 0 - 4 years old have been characteristic also embryonal tumors (neuroblastoma, Wilms' tumor, retinoblastoma). In older than 10 years incidence of Hodgkins' lymphoma, osteosarcoma and carcinomas has been increasing. All children and adolescents 0 - 18 years have been treated exclusively in the last 10 years in three pediatric oncological centers. The survival significantly improved in last 10 years. Overall 4-years survival has been 76 % in years 2000 - 2003 in comparison with 5-years survival 63 % in 1990 - 1994. More than 75 % survival was achieved in acute lymphoblastic leukemia, Hodgkins' and non-Hodgkins' lymphomas, retinoblastoma, Wilms' tumor, germinal cell tumors and soft-tissue sarcomas. The worst survival was observed in acute non-lymphoblastic leukemia (56 %) and liver tumors (41 %). (author)

Loss of heterozygosity at 11p13 and 11p15 in Wilms tumor: a study of 22 cases from India.

Science.gov (United States)

Sigamani, Elanthenral; Wari, Mohammad Nahidul; Iyer, Venkateswaran K; Agarwala, Sandeep; Sharma, Arundhati; Bakhshi, Sameer; Dinda, Amit

2013-03-01

11p13 and 11p15 loss of heterozygosity (LOH) in Wilms tumor (WT), the commonest molecular pathogenetic event in WT, shows variation in different parts of the world. The present study looked for the presence of 11p13 and 11p15 LOH as well as nephrogenic rests in WT occurring in India. Twenty-two cases of WT were subjected to thorough pathological examination for presence of nephrogenic rests. Fresh frozen tissue was evaluated for LOH at 11p13 and 11p15, using PCR for microsatellite markers. Among twenty-two consecutive cases of WT, 20 were unilateral and 2 were bilateral. 6/22 showed LOH at 11p13 (27.7 %) and 1/22 showed LOH at 11p15 (4.54 %). 2/22 cases showed presence of nephrogenic rests. One of the cases with LOH at 11p13 had intralobar nephrogenic rest in the adjacent kidney. One specimen had perilobar nephrogenic rest in the adjacent kidney but did not show LOH for either 11p13 or 11p15 in the tumor. LOH at 11p13 is seen in 27.27 % of WT in India, which is similar to reports in the English language literature. LOH at 11p15 was seen in 4.54 % of WT, which is lower than that reported from Western subjects.

Radiological changes of bones and soft tissues after irradiation therapy in patients with Wilms' tumor and neuroblastoma

Energy Technology Data Exchange (ETDEWEB)

Hirose, Hiroaki; Okabe, Ikuo

1989-04-01

Late effects of tele cobalt 60 therapy on bones and soft tissues were studied radiologically in 24 patients with neuroblastoma and Wilms' tumor. The degree of changes in spinal bodies was influenced by the dose of irradiation as well as the age of patients at the time of irradiation. In patients who had 15 to 19 Gy of irradiation at the ages under one year old, a moderate to severe degree of changes was observed. Many patients showed atrophies of iliac bone, ribs, and erector spinae and psoas muscles on the side of the irradiation. In patients who were equal to or over 12 y.o. at the time of the examination, the degree of atrophy of erector spinae muscles on the side of the irradiation was greater than that of the patients who were less than 12 y.o.. Scoliosis was observed in 71% of patients and it had a tendency to aggravate at puberty. Because there was a significant correlation between the degree of scoliosis and the severity of the atrophic erector spinae muscle, the latter was thought to contribute much to the development of the former. At present, all patients are living with no limitation of their daily activities and no one needs medical care. (author).

Wilms tumour: prognostic factors, staging, therapy and late effects

International Nuclear Information System (INIS)

Kaste, Sue C.; Dome, Jeffrey S.; Babyn, Paul S.; Graf, Norbert M.; Grundy, Paul; Godzinski, Jan; Levitt, Gill A.; Jenkinson, Helen

2008-01-01

Wilms tumour is the most common malignant renal tumour in children. Dramatic improvements in survival have occurred as the result of advances in anaesthetic and surgical management, irradiation and chemotherapy. Current therapies are based on trials and studies primarily conducted by large multi-institutional cooperatives including the Societe Internationale d'Oncologie Pediatrique (SIOP) and the Children's Oncology Group (COG). The primary goals are to treat patients according to well-defined risk groups in order to achieve the highest cure rates, to decrease the frequency and intensity of acute and late toxicity and to minimize the cost of therapy. The SIOP trials and studies largely focus on the issue of preoperative therapy, whereas the COG trials and studies start with primary surgery. This paper reviews prognostic factors and staging systems for Wilms tumour and its current treatment with surgery and chemotherapy. Surgery remains a crucial part of treatment for nephroblastoma, providing local primary tumour control and adequate staging and possibly controlling the metastatic spread and central vascular extension of the disease. Partial nephrectomy, when technically feasible, seems reasonable not only in those with bilateral disease but also in those with unilateral disease where the patient has urological disorders or syndromes predisposing to malignancy. Partial nephrectomy, however, is frequently not sufficient for an anaplastic variant of tumour. The late effects for Wilms tumour and its treatment are also reviewed. The treatment of Wilms tumour has been a success story, and currently in excess of 80% of children diagnosed with Wilms tumour can look forward to long-term survival, with less than 20% experiencing serious morbidity at 20 years from diagnosis. The late complications are a consequence of the type and intensity of treatment required, which in turn reflects the nature and extent of the original tumour. Continual international trial development

Wilms tumour: prognostic factors, staging, therapy and late effects

Energy Technology Data Exchange (ETDEWEB)

Kaste, Sue C. [St. Jude Children' s Research Hospital, Department of Radiological Sciences, Memphis, TN (United States); Dome, Jeffrey S. [St. Jude Children' s Research Hospital, Department of Oncology, Memphis, TN (United States); Babyn, Paul S. [Hospital for Sick Children, Department of Radiology, Toronto (Canada); Graf, Norbert M. [University Hospital of the Saarland, Clinic for Pediatric Oncology and Hematology, Homburg (Germany); Grundy, Paul [University of Alberta, Division of Pediatric Hematology, Oncology and Palliative Care, and Northern Alberta Children' s Cancer Program, Edmonton (Canada); Godzinski, Jan [Mother and Child Institute, Department of Oncological Surgery for Children and Adolescents, Warsaw (Poland); Levitt, Gill A. [Great Ormond Street Hospital for Sick Children NHS Trust, Paediatric Oncology, London (United Kingdom); Jenkinson, Helen [Birmingham Children' s Hospital NHS Trust, Oncology Department, Birmingham (United Kingdom)

2008-01-15

Wilms tumour is the most common malignant renal tumour in children. Dramatic improvements in survival have occurred as the result of advances in anaesthetic and surgical management, irradiation and chemotherapy. Current therapies are based on trials and studies primarily conducted by large multi-institutional cooperatives including the Societe Internationale d'Oncologie Pediatrique (SIOP) and the Children's Oncology Group (COG). The primary goals are to treat patients according to well-defined risk groups in order to achieve the highest cure rates, to decrease the frequency and intensity of acute and late toxicity and to minimize the cost of therapy. The SIOP trials and studies largely focus on the issue of preoperative therapy, whereas the COG trials and studies start with primary surgery. This paper reviews prognostic factors and staging systems for Wilms tumour and its current treatment with surgery and chemotherapy. Surgery remains a crucial part of treatment for nephroblastoma, providing local primary tumour control and adequate staging and possibly controlling the metastatic spread and central vascular extension of the disease. Partial nephrectomy, when technically feasible, seems reasonable not only in those with bilateral disease but also in those with unilateral disease where the patient has urological disorders or syndromes predisposing to malignancy. Partial nephrectomy, however, is frequently not sufficient for an anaplastic variant of tumour. The late effects for Wilms tumour and its treatment are also reviewed. The treatment of Wilms tumour has been a success story, and currently in excess of 80% of children diagnosed with Wilms tumour can look forward to long-term survival, with less than 20% experiencing serious morbidity at 20 years from diagnosis. The late complications are a consequence of the type and intensity of treatment required, which in turn reflects the nature and extent of the original tumour. Continual international trial

Expression of multidrug resistance-related protein (MRP-1), lung resistance-related protein (LRP) and topoisomerase-II (TOPO-II) in Wilms' tumor: immunohistochemical study using TMA methodology.

Science.gov (United States)

Fridman, Eduard; Skarda, Jozef; Pinthus, Jonatan H; Ramon, Jonathan; Mor, Yoran

2008-06-01

MRP-1, LRP and TOPO-II are all associated with protection of the cells from the adverse effects of various chemotherapeutics. The aim of this study was to measure the expression of these proteins in Wilms' tumor (WT). TMA block was constructed from 14 samples of WT's and from xenografts derived from them. Sections of the TMA were used for immunostaining against MRP-1, LRP and TOPO-IIa. All normal kidneys expressed MRP-1 but were either weakly or negatively stained for LRP and TOPO-IIa. In WT samples, MRP-1 was universally expressed, exclusively in the tubular component, while there was no expression of LRP and TOPO-IIa showed heterogeneous distribution. The xenografts varied in their MRP-1 and TOPO-IIa expression and exhibited weak/negative staining of LRP. This study shows that although all the proteins evaluated, had different expression patterns in the tumor samples, the most prominent changes in expression were found for MRP-1. The exact clinical implications of these changes in expression and their relevance to the resistance of these tumors to chemotherapy requires further investigation. The finding of different expression profiles for the multidrug resistance proteins in the original WT's and their xenografts suggests that the results of animal cancer models may be difficult to interpret.

New definitions of focal and diffuse anaplasia in Wilms tumor: the International Society of Paediatric Oncology (SIOP) experience.

Science.gov (United States)

Vujanić, G M; Harms, D; Sandstedt, B; Weirich, A; de Kraker, J; Delemarre, J F

1999-05-01

Unlike the original definitions of focal (FA) and diffuse anaplasia (DA) in Wilms tumor (WT), recently redefined FA and DA proved to be of prognostic significance. The aim of the study was to analyze WT from the SIOP file, the majority of which were treated with preoperative chemotherapy, in order to investigate whether chemotherapy influenced the presence of anaplasia, whether the new definitions were applicable to these tumors, and whether they were of prognostic significance. The unilateral anaplastic WT of children up to 16 years of age from the SIOP 6 and 9 nephroblastoma trials and studies were first classified according to the original definitions and analyzed. Then they were reclassified and analyzed according to the new definitions. Anaplasia was diagnosed in 86 (5.5%) of 1,554 unilateral WT. The age at diagnosis ranged from 9 to 175 months (median, 63) and more than half of children were over 5 years of age. From 15% to 85% of the tumor mass showed chemotherapy-induced changes. Blastemal anaplasia was seen in 74, stromal in 23, and epithelial in 22 cases. According to the original definitions, FA was diagnosed in 55 (64%) and DA in 31 (36%) cases. In total, 48% children were alive and well, including 53% with FA and 39% with DA (P = 0.23). When reclassified, 39 old FA cases were moved to the new DA group, resulting in 70 (81%) DA and 16 (19%) FA cases. The female-to-male ratio for FA changed from 1.9:1 to 1:1 while remained unchanged for DA. The percentage of FA stage I cases increased from 31% to 44%, while it decreased from 25% to 6% for stage III. For other stages it remained virtually unchanged. The overall 4-year actual survival was 75% for FA and 41% for DA (P = 0.03). Preoperative chemotherapy did not obliterate or produce anaplasia. The new definitions were applicable to pretreated cases and they were of prognostic significance.

Pediatric liver tumors - a pictorial review

International Nuclear Information System (INIS)

Jha, Priyanka; Tavri, Sidhartha; Patel, Chirag; Gooding, Charles; Daldrup-Link, Heike; Chawla, Soni C.

2009-01-01

Hepatic masses constitute about 5-6% of all intra-abdominal masses in children. The majority of liver tumors in children are malignant; these malignant liver tumors constitute the third most common intra-abdominal malignancy in the pediatric age group after Wilms' tumor and neuroblastoma. Only about one third of the liver tumors are benign. A differential diagnosis of liver tumors in children can be obtained based on the age of the child, clinical information (in particular AFP) and imaging characteristics. The purpose of this review is to report typical clinical and imaging characteristics of benign and malignant primary liver tumors in children. (orig.)

Molecular characterization of Wilms tumor from a resource-constrained region of sub-Saharan Africa

Science.gov (United States)

Murphy, Andrew J.; Axt, Jason R.; de Caestecker, Christian; Pierce, Janene; Correa, Hernan; Seeley, Erin H.; Caprioli, Richard M.; Newton, Mark W.; de Caestecker, Mark P.; Lovvorn, Harold N.

2012-01-01

Sub-Saharan African children have an increased incidence of Wilms tumor (WT) and experience alarmingly poor outcomes. Although these outcomes are largely due to inadequate therapy, we hypothesized that WT from this region exhibit features of biologic aggressiveness that may warrant broader implementation of high-risk therapeutic protocols. We evaluated 15 Kenyan WT (KWT) for features of aggressive disease (blastemal predominance, Ki67/cellular proliferation) and treatment resistance (anaplasia, p53 immunopositivity). To explore additional biologic features of KWT, we determined the mutational status of the CTNNB1/β-catenin and WT1 genes and performed immunostaining for markers of Wnt pathway activation (β-catenin) and nephronic progenitor cell self-renewal (WT1, CITED1, SIX2). We characterized the proteome of KWT using imaging mass spectrometry (IMS). Results were compared to histology and age-matched North American WT (NAWT) controls. For KWT patients, blastemal predominance was noted in 53.3% and anaplasia in 13%. We detected increased loss to follow up (p=0.028), disease relapse (p=0.044), mortality (p=0.001), and nuclear unrest (p=0.001) in KWT patients compared to controls. KWT and NAWT showed similar Ki67/cellular proliferation. We detected an increased proportion of epithelial nuclear β-catenin in KWT (p=0.013). All 15 KWT were found to harbor wild-type β-catenin, and 1 contained a WT1 nonsense mutation. WT1 was detected by immunostaining in 100% of KWT, CITED1 in 80%, and SIX2 in 80%. IMS revealed a molecular signature unique to KWT that was distinct from NAWT. African WTs appear to express markers of adverse clinical behavior and treatment resistance and may require alternative therapies or implementation of high-risk treatment protocols. PMID:22437966

Non-tumor enhancement at the surgical margin on CT after the removal of brain tumors

International Nuclear Information System (INIS)

Adachi, Michito; Hosoya, Takaaki; Yamaguchi, Kohichi; Yamada, Kiyotada

1992-01-01

Marginal enhancement is occasionally seen at the surgical margin on CT after the total removal of brain tumors. This enhancement disappears in due time, and therefore we call it non-tumor enhancement. It is often difficult, however, to differentiate non-tumor enhancement from tumor recurrence. In this study, we attempted to determine the characteristics of non-tumor enhancement. The subjects of the study consisted of 15 patients with astrocytoma and one with metastatic tumor in whom sequential CT scans had been performed after total removal of the tumor. Based on the observation of these sequential CT scans, the characteristics of non-tumor enhancement were presumed to be as follows: (1) In four cases, enhancement at the surgical margin persisted more than four months after surgery and then disappeared. Therefore, these cases were considered non-tumor enhancement. Prolonged duration of enhancement such as that in these cases is not necessarily due to recurrence. Marginal enhancement within 3 mm in thickness and with a well-demarcated border like that of a flax is likely to be non-tumor enhancement. (author)

Tissue expression of MLH1, PMS2, MSH2, and MSH6 proteins and prognostic value of microsatellite instability in Wilms tumor: experience of 45 cases.

Science.gov (United States)

Diniz, Gulden; Aktas, Safiye; Cubuk, Cankut; Ortac, Ragip; Vergin, Canan; Olgun, Nur

2013-05-01

Although the importance of microsatellite instability (MSI) and mismatch repair genes (MMR) is strongly established in colorectal cancer seen in the Lynch syndrome, its significance has not been fully established in Wilms tumor (WT). The aim of this study was to determine the prognostic value of MSI and MMR proteins in WT. This study included 45 pediatric cases with nephroblastoma. Protein expression was analyzed by immunohistochemistry of archival tissue sections. Real-time PCR melting analysis and fluorescence capillary electrophoresis (FCE) were performed to evaluate the MSI markers BAT25, BAT26, NR21, NR24, MONO27, penta D, and penta C in DNA extracted from tumor and normal tissues. Lower levels of MSI were observed in six cases (13.3%). There were no statistically significant correlations between MSI and some clinical prognostic factors such as stage of the tumors, and survival rates. Nineteen tumors (42.2%) showed loss of protein expression of MLH1, PMS2, MSH2, or MSH6. MMR protein defects were correlated with size (P = .021), and stage (P = .019) of the tumor, and survival rates (P < .01).Similarly MSI was also correlated with the size of the tumor (P = .046). This study showed that a small proportion of WT might be associated with the presence of MSI, as is the case with defects of DNA mismatch repair genes in the pathogenesis of WT. However, there was no concordance with the frequency of tissue expression of MMR proteins and MSI. These findings suggest that MMR genes may play an important role in the development of WT via different pathways.

Tumor size and prognosis in patients with Wilms tumor

Directory of Open Access Journals (Sweden)

Valentina Oliveira Provenzi

2015-03-01

Full Text Available OBJECTIVE: Investigate the relationship of the tumor volume after preoperative chemotherapy (TVAPQ and before preoperative chemotherapy (TVBPQ with overall survival at two and at five years, and lifetime. METHODS: Our sample consisted of consecutive patients evaluated in the period from 1989 to 2009 in an Onco-Hematology Service. Clinical, histological and volumetric data were collected from the medical records. For analysis, chi-square, Kaplan-Meier, log-rank and Cox regression tests were used. RESULTS: The sample consisted of 32 patients, 53.1% were male with a median age at diagnosis of 43 months. There was a significant association between TVAPQ>500mL and the difference between the TVBPQ and TVAPQ (p=0.015 and histologic types of risk (p=0.008. It was also verified an association between the difference between the TVBPQ and TVAPQ and the predominant stromal tumor (p=0.037. When assessing the TVAPQ of all patients, without a cutoff, there was an association of the variable with lifetime (p=0.013, i.e., for each increase of 10mL in TVAPQ there was an average increase of 2% in the risk of death. CONCLUSIONS: Although our results indicate that the TVAPQ could be considered alone as a predictor of poor prognosis regardless of the cutoff suggested in the literature, more studies are needed to replace the histology and staging by tumor size as best prognostic variable.

Bilateral disease and new trends in Wilms tumour

Energy Technology Data Exchange (ETDEWEB)

Owens, Catherine M.; Olsen, Oeystein E. [Great Ormond Street Hospital for Children NHS Trust, Department of Radiology, London (United Kingdom); Brisse, Herve J. [Institut Curie, Service de Radiodiagnostic, Paris (France); Begent, Joanna [University College Hospital, Paediatric Oncology, London (United Kingdom); Smets, Anne M. [Academic Medical Center Amsterdam, Department of Radiology, Amsterdam (Netherlands)

2008-01-15

Wilms tumour is a great therapeutic success story within paediatric oncology; its prognosis is excellent. Although mainly sporadic, occurring in otherwise well children, it occurs in a small number of genetically predisposed children. Thus regular surveillance imaging is performed in predisposed children in parts of the USA and Europe. The risks and benefits of surveillance are unclear, as the existing ad-hoc surveillance protocols are lacking in consistency of practice and equity of provision. We present guidelines for Wilms tumour surveillance based on a review of current practice and available evidence, outlined by a multidisciplinary working group in the UK. Wilms tumours are bilateral in 4-13% of affected children. Bilateral synchronous nephroblastomas are observed in 5% of affected children and are usually associated with the presence of nephrogenic rests, congenital malformations and predisposing syndromes. The major challenge in bilateral disease is to achieve a cure and at the same time to preserve sufficient functional renal tissue for normal growth and development. The association among Wilms tumour, nephrogenic rests and nephroblastomatosis makes detection and characterization of renal lesions with imaging extremely important. We discuss the relative strengths and weaknesses of the different modalities used for diagnosis and follow-up in bilateral renal disease. We also discuss newly emerging diagnostic imaging tests such as {sup 18}F-fluorodeoxyglucose positron emission tomography (FDG-PET). This technique, when fused with CT (PET-CT), allows accelerated metabolic activity to be accurately anatomically localised and so is potentially useful for staging, assessment of treatment response, and for surgical and radiotherapy planning. In addition, quantitative MRI techniques have been proved to be valuable in intracranial tumours, but no such role has been validated in abdominal disease. Diffusion-weighted imaging with calculation of ADC maps is feasible in

Bilateral disease and new trends in Wilms tumour

International Nuclear Information System (INIS)

Owens, Catherine M.; Olsen, Oeystein E.; Brisse, Herve J.; Begent, Joanna; Smets, Anne M.

2008-01-01

Wilms tumour is a great therapeutic success story within paediatric oncology; its prognosis is excellent. Although mainly sporadic, occurring in otherwise well children, it occurs in a small number of genetically predisposed children. Thus regular surveillance imaging is performed in predisposed children in parts of the USA and Europe. The risks and benefits of surveillance are unclear, as the existing ad-hoc surveillance protocols are lacking in consistency of practice and equity of provision. We present guidelines for Wilms tumour surveillance based on a review of current practice and available evidence, outlined by a multidisciplinary working group in the UK. Wilms tumours are bilateral in 4-13% of affected children. Bilateral synchronous nephroblastomas are observed in 5% of affected children and are usually associated with the presence of nephrogenic rests, congenital malformations and predisposing syndromes. The major challenge in bilateral disease is to achieve a cure and at the same time to preserve sufficient functional renal tissue for normal growth and development. The association among Wilms tumour, nephrogenic rests and nephroblastomatosis makes detection and characterization of renal lesions with imaging extremely important. We discuss the relative strengths and weaknesses of the different modalities used for diagnosis and follow-up in bilateral renal disease. We also discuss newly emerging diagnostic imaging tests such as 18 F-fluorodeoxyglucose positron emission tomography (FDG-PET). This technique, when fused with CT (PET-CT), allows accelerated metabolic activity to be accurately anatomically localised and so is potentially useful for staging, assessment of treatment response, and for surgical and radiotherapy planning. In addition, quantitative MRI techniques have been proved to be valuable in intracranial tumours, but no such role has been validated in abdominal disease. Diffusion-weighted imaging with calculation of ADC maps is feasible in

Patterns of lymph node sampling and the impact of lymph node density in favorable histology Wilms tumor: An analysis of the national cancer database.

Science.gov (United States)

Saltzman, A F; Carrasco, A; Amini, A; Aldrink, J H; Dasgupta, R; Gow, K W; Glick, R D; Ehrlich, P F; Cost, N G

2018-04-01

There is controversy about the role of lymph node (LN) sampling or dissection in the management of favorable histology (FH) Wilms tumor (WT), specifically how it performed and how it may impact survival. The objective of this study was to analyze factors affecting LN sampling patterns and the impact of LN yield and density (number of positive LNs/LNs examined) on overall survival (OS) in patients with advanced-stage favorable histology Wilms tumor (FHWT). The National Cancer Database (NCDB) was queried for patients with FHWT during 2004-2013. Demographic, clinical and OS data were abstracted for those who underwent surgical resection. Poisson regression was performed to analyze how factors influenced LN yield. Patients with positive LNs had LN density calculated and were further analyzed. A total of 2340 patients met criteria, with a median age at diagnosis of 3 years (range 0-78 years). The median number of LNs examined was three (range 0-87). Lymph node yield was affected by age, race, insurance, tumor size, laterality, advanced stage, LN positivity, and institutional volume. A total of 390 (16.6%) patients had LN-positive disease. Median LN density for these LN-positive patients was 0.38 (range 0.02-1) (Summary Figure). Estimated 5-year OS was significantly improved for those with LN density ≤0.38 vs. >0.38 (94% vs. 84.6%, P = 0.012). In this population, on multivariate analysis, age and LN density were significant predictors of OS. It is difficult to compile large numbers of cases in rare diseases like WT, and fortunately a large administrative database such as the NCDB can serve as a great resource. However, administrative data come with inherent limitations such as missing data and inability to account for a variety of factors that may influence LN yield and/or OS (specimen designation, pathologist experience, surgeon experience/volume, institutional Children's Oncology Group (COG) association, etc.). In this specific disease, the American Joint Committee

Outcome for children with metastatic solid tumors over the last four decades.

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Stephanie M Perkins

Full Text Available Outcomes for pediatric solid tumors have significantly improved over the last 30 years. However, much of this improvement is due to improved outcome for patients with localized disease. Here we evaluate overall survival (OS for pediatric patients with metastatic disease over the last 40 years.The United States Surveillance, Epidemiology, and End Results (SEER database was used to conduct this study. Patients diagnosed between 0 and 18 years of age with metastatic Ewings sarcoma, neuroblastoma, osteosarcoma, rhabdomyosarcoma or Wilms tumor were included in the analysis.3,009 patients diagnosed between 1973-2010 met inclusion criteria for analysis. OS at 10 years for patients diagnosed between 1973-1979, 1980-1989, 1990-1999 and 2000-2010 was 28.3%, 37.2%, 44.7% and 49.3%, respectively (p<0.001. For patients diagnosed between 2000-2010, 10-year OS for patients with Ewing sarcoma, neuroblastoma, osteosarcoma, rhabdomyosarcoma and Wilms tumor was 30.6%, 54.4%, 29.3%, 27.5%, and 76.6%, respectively, as compared to 13.8%, 25.1%, 13.6%, 17.9% and 57.1%, respectively, for patients diagnosed between 1973-1979. OS for neuroblastoma significantly increased with each decade. For patients with osteosarcoma and Ewing sarcoma, there was no improvement in OS over the last two decades. There was no improvement in outcome for patients with rhabdomyosarcoma or Wilms tumor over the last 30 years.OS for pediatric patients with metastatic solid tumors has significantly improved since the 1970s. However, outcome has changed little for some malignancies in the last 20-30 years. These data underscore the importance of continued collaboration and studies to improve outcome for these patients.

Multicentre study of Wilm's tumours treated by different therapeutic ...

African Journals Online (AJOL)

National Wilm's Tumour Study (NWTS) group and the. International ... improvement in the survival of children with cancer in ... at diagnosis, sex, incidence, presenting symptoms, pre- ... localized in the left kidney in 22 (55%) patients, the right.

Multiple mechanisms of MYCN dysregulation in Wilms tumour

NARCIS (Netherlands)

Williams, Richard D.; Chagtai, Tasnim; Alcaide-German, Marisa; Apps, John; Wegert, Jenny; Popov, Sergey; Vujanic, Gordan; van Tinteren, Harm; van den Heuvel-Eibrink, Marry M.; Kool, Marcel; de Kraker, Jan; Gisselsson, David; Graf, Norbert; Gessler, Manfred; Pritchard-Jones, Kathy

2015-01-01

Genomic gain of the proto-oncogene transcription factor gene MYCN is associated with poor prognosis in several childhood cancers. Here we present a comprehensive copy number analysis of MYCN in Wilms tumour (WT), demonstrating that gain of this gene is associated with anaplasia and with poorer

Cytomorphology and immunohistochemistry of extrarenal rhabdoid tumor: A case report with review of literature

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Manjula Jain

2011-01-01

Full Text Available Extrarenal rhabdoid tumor (ERRT is a rare, aggressive tumor with extremely poor prognosis. We report a case of ERRT with intraspinal extension in a 1.5-year-old child diagnosed by fine needle aspiration cytology (FNAC and immunohistochemistry. The child presented with a right lumbar region lump of two months duration. Ultrasound guided FNAC was performed and cell block was prepared. Smears were highly cellular and showed a dispersed population of large round cells having abundant pale eosinophillic cytoplasm, centrally to eccentrically placed nucleus with large prominent nucleoli. Immunohistochemistry was carried out on cell block which was positive for epithelial membrane antigen EMA and Vimentin. It was negative for leucocyte common antigen [LCA], wilms tumor 1, WT1, desmin and neuron specific enolaseNSE, thus ruling out other tumors like lymphoma, Wilms tumor, rhabdomyosarcoma, and neuroblastoma. A final diagnosis of ERRT was given. ERRT is an extremely rare tumor of retroperitoneal area; it should be included in the differential diagnosis of malignant round cell tumor in children. Cell block in this case is mandatory for putting up the panel of immunohistochemistry which can clinch the diagnosis of rhabdoid tumor and treatment can be started as early as possible.

Frequency of WT1 and 11p15 constitutional aberrations and phenotypic correlation in childhood Wilms tumour patients

NARCIS (Netherlands)

Segers, H.; Kersseboom, R.; Alders, M.; Pieters, R.; Wagner, A.; van den Heuvel-Eibrink, M. M.

2012-01-01

Introduction: In 9-17% of Wilms tumour patients a predisposing syndrome is present, in particular WT1-associated syndromes and overgrowth syndromes. Constitutional WT1 mutations or epigenetic changes on chromosome 11p15 have also been described in Wilms tumour patients without phenotypic

[Maxillofacial and dental abnormalities in some multiple abnormality syndromes. "Cri du chat" syndrome, Wilms' tumor-aniridia syndrome; Sotos syndrome; Goldenhar syndrome].

Science.gov (United States)

Berio, A; Trucchi, R; Meliota, M

1992-05-01

The paper describes the maxillo-facial and dental anomalies observed in some chromosome and non-chromosome poly-malformative syndromes ("Cri du chat" syndrome; Wilms' tumour; Sotos' syndrome; Goldenhar's syndrome). The Authors emphasise the possibility of diagnosing these multiple deformity syndromes from maxillo-facial alterations in early infancy; anomalous tooth position and structure cal also be successfully treated immediately after the first appearance of teeth. This is a particularly promising field of pediatrics and preventive pediatric medicine.

Intraoperative Spinal Navigation for the Removal of Intradural Tumors: Technical Notes.

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Stefini, Roberto; Peron, Stefano; Mandelli, Jaime; Bianchini, Elena; Roccucci, Paolo

2017-08-05

In recent years, spinal surgery has incorporated the many advantages of navigation techniques to facilitate the placement of pedicle screws during osteosynthesis, mainly for degenerative diseases. However, spinal intradural tumors are not clearly visible by intraoperative fluoroscopy or computed tomography scans, thereby making navigation necessary. To evaluate the use of spinal navigation for the removal of intradural and spinal cord tumors using spinal magnetic resonance imaging (MRI) merged with intraoperative 3-dimensional (3-D) fluoro images. After fixing the patient reference frame on the spinous process, the 3-D fluoro images were obtained in the surgical room. Using this image as the reference, the preoperative volumetric MRI images and intraoperative 3-D fluoro images were merged using automated software or manually. From January to July 2016, we performed 10 navigated procedures for intradural spinal tumors by merging MRI and 3-D fluoro images. Nine patients had an intradural extramedullary tumor, 6 had neurinomas, and 3 had meningiomas; 1 patient had an intramedullary spinal cord metastasis. The surgically demonstrated benefits of spinal navigation for the removal of intradural tumors include the decreased risk of surgery at the wrong spinal level, a minimal length of skin incision and muscle strip, and a reduction in bone removal extension. Furthermore, this technique offers the advantage of opening the dura as much as is necessary and, in the case of intrinsic spinal cord tumors, it allows the tumor to be centered. Otherwise, this would not be visible, thus enabling the precise level and the posterior midline sulcus to be determined when performing a mielotomy. Copyright © 2017 by the Congress of Neurological Surgeons

Multiple mechanisms of MYCN dysregulation in Wilms tumour

Science.gov (United States)

Williams, Richard D.; Chagtai, Tasnim; Alcaide-German, Marisa; Apps, John; Wegert, Jenny; Popov, Sergey; Vujanic, Gordan; van Tinteren, Harm; van den Heuvel-Eibrink, Marry M.; Kool, Marcel; de Kraker, Jan; Gisselsson, David; Graf, Norbert; Gessler, Manfred; Pritchard-Jones, Kathy

2015-01-01

Genomic gain of the proto-oncogene transcription factor gene MYCN is associated with poor prognosis in several childhood cancers. Here we present a comprehensive copy number analysis of MYCN in Wilms tumour (WT), demonstrating that gain of this gene is associated with anaplasia and with poorer relapse-free and overall survival, independent of histology. Using whole exome and gene-specific sequencing, together with methylation and expression profiling, we show that MYCN is targeted by other mechanisms, including a recurrent somatic mutation, P44L, and specific DNA hypomethylation events associated with MYCN overexpression in tumours with high risk histologies. We describe parallel evolution of genomic copy number gain and point mutation of MYCN in the contralateral tumours of a remarkable bilateral case in which independent contralateral mutations of TP53 also evolve over time. We report a second bilateral case in which MYCN gain is a germline aberration. Our results suggest a significant role for MYCN dysregulation in the molecular biology of Wilms tumour. We conclude that MYCN gain is prognostically significant, and suggest that the novel P44L somatic variant is likely to be an activating mutation. PMID:25749049

Use of Wilms Tumor 1 Gene Expression as a Reliable Marker for Prognosis and Minimal Residual Disease Monitoring in Acute Myeloid Leukemia With Normal Karyotype Patients.

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Marjanovic, Irena; Karan-Djurasevic, Teodora; Ugrin, Milena; Virijevic, Marijana; Vidovic, Ana; Tomin, Dragica; Suvajdzic Vukovic, Nada; Pavlovic, Sonja; Tosic, Natasa

2017-05-01

Acute myeloid leukemia with normal karyotype (AML-NK) represents the largest group of AML patients classified with an intermediate prognosis. A constant need exists to introduce new molecular markers for more precise risk stratification and for minimal residual disease (MRD) monitoring. Quantitative assessment of Wilms tumor 1 (WT1) gene transcripts was performed using real-time polymerase chain reaction. The bone marrow samples were collected at the diagnosis from 104 AML-NK patients and from 34 of these patients during follow-up or disease relapse. We found that overexpression of the WT1 gene (WT1 high status), present in 25.5% of patients, was an independent unfavorable factor for achieving complete remission. WT1 high status was also associated with resistance to therapy and shorter disease-free survival and overall survival. Assessment of the log reduction value of WT1 expression, measured in paired diagnosis/complete remission samples, revealed that patients with a log reduction of < 2 had a tendency toward shorter disease-free survival and overall survival and a greater incidence of disease relapse. Combining WT1 gene expression status with NPM1 and FLT3-ITD mutational status, we found that the tumor behavior of intermediate patients (FLT3-ITD - /NPM1 - double negative) with WT1 high status is almost the same as the tumor behavior of the adverse risk group. WT1 expression status represents a good molecular marker of prognosis, response to treatment, and MRD monitoring. Above all, the usage of the WT1 expression level as an additional marker for more precise risk stratification of AML-NK patients could lead to more adapted, personalized treatment protocols. Copyright © 2017 Elsevier Inc. All rights reserved.

Radiotherapy of the most frequent solid tumors in childhood

International Nuclear Information System (INIS)

Pfeiffer, J.; Kamprad, F.

1980-01-01

During the past decade the prognosis of malignant tumors in childhood could be clearly improved, realized by combining surgery, radiation therapy and chemotherapy. Recommendations for the use of radiotherapy for the most frequent solid tumors in childhood are represented basing on the experience of the study groups 'Pediatric Hematology and Oncology' of the Society for Pediatrics of the GDR and 'Tumors in Childhood' of the Section of Children's Surgery of the GDR. Besides general problems which have to be taken into consideration in the treatment of infantile tumors the radiotherapeutical measures for Wilms' tumors, neuroblastomas, cerebral tumors, embryonal sarcomas of the soft parts and bone tumors are discussed. The necessary close cooperation of the attending branches is pointed out and both the regional centralization of patients' care and a superregional cooperation are required. (author)

DNA methylation profile distinguishes clear cell sarcoma of the kidney from other pediatric renal tumors.

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Hitomi Ueno

Full Text Available A number of specific, distinct neoplastic entities occur in the pediatric kidney, including Wilms' tumor, clear cell sarcoma of the kidney (CCSK, congenital mesoblastic nephroma (CMN, rhabdoid tumor of the kidney (RTK, and the Ewing's sarcoma family of tumors (ESFT. By employing DNA methylation profiling using Illumina Infinium HumanMethylation27, we analyzed the epigenetic characteristics of the sarcomas including CCSK, RTK, and ESFT in comparison with those of the non-neoplastic kidney (NK, and these tumors exhibited distinct DNA methylation profiles in a tumor-type-specific manner. CCSK is the most frequently hypermethylated, but least frequently hypomethylated, at CpG sites among these sarcomas, and exhibited 490 hypermethylated and 46 hypomethylated CpG sites in compared with NK. We further validated the results by MassARRAY, and revealed that a combination of four genes was sufficient for the DNA methylation profile-based differentiation of these tumors by clustering analysis. Furthermore, THBS1 CpG sites were found to be specifically hypermethylated in CCSK and, thus, the DNA methylation status of these THBS1 sites alone was sufficient for the distinction of CCSK from other pediatric renal tumors, including Wilms' tumor and CMN. Moreover, combined bisulfite restriction analysis could be applied for the detection of hypermethylation of a THBS1 CpG site. Besides the biological significance in the pathogenesis, the DNA methylation profile should be useful for the differential diagnosis of pediatric renal tumors.

Anteriorly placed tumors to the conus: removal by interradicular window.

Science.gov (United States)

Ciappetta, P; Taurone, S; Spoletini, M; Artico, M

2017-01-01

Tumors anteriorly situated to the medullary conus are rarely encountered and represent a true surgical challenge. We examined the literature on this topic, concluding that there are no previous reports on alternative surgical techniques different to the traditional one. We report two cases of intradural extramedullary tumor operated on by a technique performed through a window opened between the spinal roots, which allows an easy, effective and useful resection. We describe a new operative technique which ensures a complete removal of these tumors and discuss clinical implications in the light of the available literature on this topic.

Association between renal cystic lesions and bilateral Wilms' tumours

Energy Technology Data Exchange (ETDEWEB)

Simanovsky, Natalia; Hiller, Nurith [Hadassah-Hebrew University Medical Center at Mt Scopus, Department of Medical Imaging, POB 12000, Jerusalem (Israel); Revel-Vilk, Shoshana; Weintraub, Michael [Hadassah-Hebrew University Medical Center, Department of Pediatric Hematology/ Oncology, Jerusalem (Israel)

2016-06-15

Evaluate a potential association between Wilms' tumour (WT) and renal cystic lesions. Digital records and imaging files of consecutive patients diagnosed with WT between 2004 and 2014 were retrospectively reviewed under an Institutional Review Board waiver of informed consent. The locations of renal cysts seen on US, CT, and/or MRI were recorded and compared with the locations of newly developed WT. A total of 48 patients (mean age 3 years 9 months) presented with newly diagnosed WT in the study period. Mean follow-up was 4.5 (range 1-10) years. WT was unilateral in 40 children, bilateral in 8. Renal cysts were identified in only one of the forty patients (2.5 %) with unilateral disease - in the contralateral kidney. In contrast, renal cysts were found in seven of eight patients with bilateral WT (87.5 %), in two of whom, new tumours developed in the same location where cysts had been seen on previous imaging studies. Renal cystic lesions in patients with Wilms' tumour should be regarded as potential tumour precursors, and followed with frequent imaging. (orig.)

Immunohistochemical expression of p53 proteins in Wilms' tumour: a possible association with the histological prognostic parameter of anaplasia.

Science.gov (United States)

Cheah, P L; Looi, L M; Chan, L L

1996-01-01

Wilms' tumour (nephroblastoma) has been associated with chromosomal abnormalities at the 11p13, 11p15 and 16q regions. A study into the possibility of mutations occurring within p53, the ubiquitous adult tumour suppressor gene, in Wilms' tumour was carried out. Thirty-eight cases were studied. Of these 36 were categorised into the favourable histology group and two into the unfavourable histology group based on the National Wilms' Tumour Study criteria. Archival formalin-fixed, paraffin-embedded tissue sections from each case were stained with a polyclonal (AB565:Chemicon) and a monoclonal (DO7:Dako) antibody raised against p53 protein using a peroxidase-labelled streptavidin biotin kit (Dako). 'Cure' (disease-free survival of 60 months or longer) was documented in 39% of cases with favourable histology tumours. Eleven percent in this group succumbed to the disease. Both cases with unfavourable histology died. Four out of 36 (11%) tumours with favourable histology demonstrated weak to moderate staining with both AB565 and DO7 in more than 75% of tumour cells. In contrast, p53 protein expression in unfavourable histology tumours was significantly increased compared with the favourable histology group (P = 0.021) with both cases demonstrating immunopositivity in > 75% of tumour cells when stained with AB565 and DO7. The intensity of staining ranged from moderate to strong in both cases. It appears from this preliminary study that the immunohistochemical expression of p53 protein in Wilms' tumour, presumably a result of mutation in the p53 tumour suppressor gene, correlates with histological classification, histological categorisation being one of the useful features in the prognostic assessment of Wilms' tumours.

Clinically Relevant Subsets Identified by Gene Expression Patterns Support a Revised Ontogenic Model of Wilms Tumor: A Children's Oncology Group Study

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Samantha Gadd

2012-08-01

Full Text Available Wilms tumors (WT have provided broad insights into the interface between development and tumorigenesis. Further understanding is confounded by their genetic, histologic, and clinical heterogeneity, the basis of which remains largely unknown. We evaluated 224 WT for global gene expression patterns; WT1, CTNNB1, and WTX mutation; and 11p15 copy number and methylation patterns. Five subsets were identified showing distinct differences in their pathologic and clinical features: these findings were validated in 100 additional WT. The gene expression pattern of each subset was compared with published gene expression profiles during normal renal development. A novel subset of epithelial WT in infants lacked WT1, CTNNB1, and WTX mutations and nephrogenic rests and displayed a gene expression pattern of the postinduction nephron, and none recurred. Three subsets were characterized by a low expression of WT1 and intralobar nephrogenic rests. These differed in their frequency of WT1 and CTNNB1 mutations, in their age, in their relapse rate, and in their expression similarities with the intermediate mesoderm versus the metanephric mesenchyme. The largest subset was characterized by biallelic methylation of the imprint control region 1, a gene expression profile of the metanephric mesenchyme, and both interlunar and perilobar nephrogenic rests. These data provide a biologic explanation for the clinical and pathologic heterogeneity seen within WT and enable the future development of subset-specific therapeutic strategies. Further, these data support a revision of the current model of WT ontogeny, which allows for an interplay between the type of initiating event and the developmental stage in which it occurs.

A monoclonal antibody stains blastemal but not tubular components of Wilms' tumour

NARCIS (Netherlands)

Sarawar, S. R.; Schlingemann, R. O.; Kelsey, A.; Fleming, S.; Kumar, S.

1988-01-01

The monoclonal antibody PAL-E is specific for endothelial cells in a wide variety of normal and tumour tissue. In normal kidney, PAL-E reacts exclusively with the endothelium of non-glomerular blood vessels. In Wilms' tumour, binding of PAL-E was not restricted to the endothelium; staining of

Retroperitoneal Malignant Peripheral Nerve Sheath Tumor Replacing an Absent Kidney in a Child

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Samin Alavi

2013-01-01

Full Text Available Malignant peripheral nerve sheath tumors (MPNSTs are nonrhabdomyosarcoma soft tissue sarcomas with rare occurrence in children specially in the retroperitoneum. We describe a young child who presented with an abdominal mass. Both ultrasound and computed tomography revealed a large right-sided abdominal mass in the anatomic place of right kidney, while no kidney or ureter was observed at that side. He underwent surgical resection of the tumor with a primary impression of Wilms tumor. To the authors’ knowledge, this is the first case of retroperitoneal malignant peripheral nerve sheath tumor and absent kidney. This case suggests the very rare probability of association of MPNSTs in children with genitourinary tract anomalies such as renal agenesis.

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

[Hydrocephalus Associated with Small Clinoidal Meningioma that Resolved after Tumor Removal:A Case Report].

Science.gov (United States)

Fujiwara, Hidemoto; Aiba, Toyotaka; Watanabe, Toru; Hiraishi, Tetsuya; Fujii, Yukihiko

2016-12-01

Small meningiomas causing hydrocephalus without obstruction of the ventricular system are rare. Herein, we report a case of small clinoidal meningioma with communicating hydrocephalus, which resolved after tumor removal. A 70-year-old woman presented with a 1-month history of memory disturbance followed by gait disturbance. MR images revealed a right clinoidal meningioma, 2 cm in diameter, and dilatation of the ventricles suggesting communicating hydrocephalus. The cerebrospinal fluid(CSF)pressure was 130 mmH2O, as determined via a lumbar puncture. High concentrations of protein(65mg/dL)were detected in the lumbar CSF. The tumor was completely removed via a frontotemporal craniotomy. Higher protein concentrations(94mg/dL)were detected in the CSF obtained intraoperatively from the sylvian cistern. The histopathological diagnosis was meningothelial meningioma. The patient's symptoms improved markedly after surgery. Postoperative MR images revealed resolution of the hydrocephalus. The lumbar CSF protein concentration returned to normal(43mg/dL). Neither tumor recurrence nor progression of hydrocephalus has been observed for 4 years. Communicating hydrocephalus, associated with a small meningioma at the supratentorial region, has not been described. Previous studies have shown that patients with meningioma may develop communicating hydrocephalus after tumor removal or stereotactic radiosurgery. Thus, it is interesting that the small supratentorial meningioma in our case developed communicating hydrocephalus without any therapeutic intervention. Considering the CSF protein concentration, we speculate that the hydrocephalus was the result of CSF malabsorption associated with high CSF protein concentration and CSF pathway obstruction at the suprasellar cistern caused by the tumor.

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.

[Vitamin-resistant rickets cured by removal of a bone tumor. Review of the literature].

Science.gov (United States)

François, S; Lefort, G; Poli-Merol, M L; Gaillard, D; Roussel, B; Sulmont, V; Daoud, S

1997-01-01

Rickets secondary to bone or soft tissue tumors are rare in children. Majority of the reported cases occurred in adults older than thirty. This entity can be cured after tumor removal. The authors present a case in a ten year boy and literature review. A ten year boy complained of diffuse bone and muscle weakness for two years. A diagnosis of arthritis was made but the patient continued to complain. Serum calcium level was normal (2.33 mmol/l), phosphorus was very low (0.43 mmol/l), serum alkaline phosphatase was high, parathyroid hormone and vitamin D level were normal. Urinalysis showed abnormal phosphate excretion. The absence of malabsorption, no family history of rickets or hypophosphatermy presence of a marked excess of urinary phosphate, very low serum phosphate and normal serum calcium, vitamin D and parathyroid hormone levels led us to consider a diagnosis of tumor induced osteomalacia. Radiographs showed a large round radiolucent lesion in the left superior pubic ramus and generalized demineralisation. We performed a complete tumor resection and the space was filled with bone graft. On histopathologic examination it was a benign mesenchymal tumor. Rapid reversal of biochemical anomalies, radiographs anomalies and clinical manifestation were observed after complete tumor resection. The authors have described the tumor, the osteomalacia and the pathogenesis of tumor rickets. Histologically the most common causative tumors were vascular tumors, mesenchymal tumors and non ossifying tumors. The tumor were of bone or soft tissue origin. Clinical symptoms were muscular weakness, bone and muscle pain. Biochemically there is a very low phosphate level, a normal serum calcium level as well as a normal vitamin D and PTH level. There is a significant high level of urinal phosphate. The mechanism proposed to explain oncogenic osteomalacia includes tumor secretion of phosphaturic substance other than PTH and calcitonin. Another hypothesis is a substance interfering

TP53 alterations in Wilms tumour represent progression events with strong intratumour heterogeneity that are closely linked but not limited to anaplasia

OpenAIRE

Wegert, Jenny; Vokuh, Christian; Ziegler, Barbara; Ernestus, Karen; Leuschner, Ivo; Furtwängler, Rhoikos; Graf, Norbert; Gessler, Manfred

2018-01-01

TP53 mutations have been associated with anaplasia in Wilms tumour, which conveys a high risk for relapse and fatal outcome. Nevertheless, TP53 alterations have been reported in no more than 60% of anaplastic tumours, and recent data have suggested their presence in tumours that do not fulfil the criteria for anaplasia, questioning the clinical utility of TP53 analysis. Therefore, we characterized the TP53 status in 84 fatal cases of Wilms tumour, irrespective of histological subtype. We iden...

TP53 alterations in Wilms tumour represent progression events with strong intratumour heterogeneity that are closely linked but not limited to anaplasia

OpenAIRE

Wegert, Jenny; Vokuhl, Christian; Ziegler, Barbara; Ernestus, Karen; Leuschner, Ivo; Furtwängler, Rhoikos; Graf, Norbert; Gessler, Manfred

2017-01-01

Abstract TP53 mutations have been associated with anaplasia in Wilms tumour, which conveys a high risk for relapse and fatal outcome. Nevertheless, TP53 alterations have been reported in no more than 60% of anaplastic tumours, and recent data have suggested their presence in tumours that do not fulfil the criteria for anaplasia, questioning the clinical utility of TP53 analysis. Therefore, we characterized the TP53 status in 84 fatal cases of Wilms tumour, irrespective of histological subtype...

The first investigation of Wilms' tumour atomic structure-nitrogen and carbon isotopic composition as a novel biomarker for the most individual approach in cancer disease

Science.gov (United States)

Taran, Katarzyna; Frączek, Tomasz; Sikora-Szubert, Anita; Sitkiewicz, Anna; Młynarski, Wojciech; Kobos, Józef; Paneth, Piotr

2016-01-01

The paper describes a novel approach to investigating Wilms' tumour (nephroblastoma) biology at the atomic level. Isotope Ratio Mass Spectrometry (IRMS) was used to directly assess the isotope ratios of nitrogen and carbon in 84 Wilms' tumour tissue samples from 28 cases representing the histological spectrum of nephroblastoma. Marked differences in nitrogen and carbon isotope ratios were found between nephroblastoma histological types and along the course of cancer disease, with a breakout in isotope ratio of the examined elements in tumour tissue found between stages 2 and 3. Different isotopic compositions with regard to nitrogen and carbon content were observed in blastemal Wilms' tumour, with and without focal anaplasia, and in poorly- and well-differentiated epithelial nephroblastoma. This first assessment of nitrogen and carbon isotope ratio reveals the previously unknown part of Wilms' tumour biology and represents a potential novel biomarker, allowing for a highly individual approach to treating cancer. Furthermore, this method of estimating isotopic composition appears to be the most sensitive tool yet for cancer tissue evaluation, and a valuable complement to established cancer study methods with prospective clinical impact. PMID:27732932

Interactive stereotactic surgical system for the removal of intracranial tumors utilizing the CO2 laser and CT-derived database

International Nuclear Information System (INIS)

Kall, B.A.; Kelly, P.J.; Goerss, S.J.

1985-01-01

The transposition of data derived from computed tomography into three-dimensional stereotactic space has been found to be useful for the removal of intracranial tumors by a stereotactically directed CO 2 laser. Serial cross sections of a CT-derived tumor volume sliced orthogonal to any surgical trajectory are displayed in the operating room. Tumor slices are interactively vaporized with a stereotactically directed carbon dioxide laser. In this manner, the entire mass detected by CT scanning may be removed within a three-dimensionally controlled environment

Palbociclib in Treating Patients With Relapsed or Refractory Rb Positive Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Activating Alterations in Cell Cycle Genes (A Pediatric MATCH Treatment Trial)

Science.gov (United States)

2018-05-15

Advanced Malignant Solid Neoplasm; RB1 Positive; Recurrent Childhood Ependymoma; Recurrent Ewing Sarcoma; Recurrent Glioma; Recurrent Hepatoblastoma; Recurrent Kidney Wilms Tumor; Recurrent Langerhans Cell Histiocytosis; Recurrent Malignant Germ Cell Tumor; Recurrent Malignant Glioma; Recurrent Medulloblastoma; Recurrent Neuroblastoma; Recurrent Non-Hodgkin Lymphoma; Recurrent Osteosarcoma; Recurrent Peripheral Primitive Neuroectodermal Tumor; Recurrent Rhabdoid Tumor; Recurrent Rhabdomyosarcoma; Recurrent Soft Tissue Sarcoma; Refractory Ependymoma; Refractory Ewing Sarcoma; Refractory Glioma; Refractory Hepatoblastoma; Refractory Langerhans Cell Histiocytosis; Refractory Malignant Germ Cell Tumor; Refractory Malignant Glioma; Refractory Medulloblastoma; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Osteosarcoma; Refractory Peripheral Primitive Neuroectodermal Tumor; Refractory Rhabdoid Tumor; Refractory Rhabdomyosarcoma; Refractory Soft Tissue Sarcoma

Abdominal masses in infants and children: Analysis of 92 cases with special reference to radiological findings and differential diagnosis

Energy Technology Data Exchange (ETDEWEB)

Choi, Kyung Hee; Suh, Jung Ho; Choi, Byung Sook [Yonsei University College of Medicine, Seoul (Korea, Republic of)

1974-10-15

The abdominal tumors in children are different from those of adults in respect to age brackets, basic tumor types, sites of origin and clinical manifestations. In children carcinoma are exceedingly uncommon. Most of the tumors are embryonal in origin and teratoma make up the bulk of the remaining neoplasms. Abdominal tumors are usually silent until they have assumed tremendous size. Early detection and aggressive philosophy of management became important as the trend to cure with radiation and chemotherapy increased. In a child with an abdominal mass x-ray exams are useful and often diagnostic. The 92 cases of abdominal masses in infants and children under the age of 16, who had been treated at Severance Hospital for the duration of 14 years from 1960 to 1973 were reviewed in respect to their basic type of tumors, site of origin, age incidence as well as radiographic findings. The results may be briefly summarized as follows; 1. The commonest tumor was ovarian cyst being 25 cases which were largely encountered over the age of 12. The next common tumors were Wilms' tumor, 17 cases, hepatoma, 9 cases, neuroblastoma and hydronephrosis, 8 cases each, teratoma 6 cases, etc. 2. The retroperitoneal space including kidneys were the most commonly involved site (43.5%), of which Wilms' tumor was the commonest. 3. About 2/3 of tumors developed under the age of 6 and the commonest lesions was Wilms' tumor and the next being neuroblastoma, teratoma, ovarian cyst and so forth. 4.In all tumors except ovarian tumors and choledochal cyst, male was more frequently affected. 5. In plain abdominal 75% of neuroblastoma crossed the mid line while in Wilms' tumor only 2 cases (14%) showed midline cross. Calcific density was present in all 6 cases of teratoma (100%) as dense and discrete ossification, in 3 cases of neuroblastoma (38%) characteristically in diffuse stippled appearance and in one case of Wilms' tumor (6%), in which calcification was nonspecific. 9. Intravenous pyelogram was

Fatty degeneration in a Wilms' tumour after chemotherapy

International Nuclear Information System (INIS)

Jeanes, A.C.; Beese, R.C.; McHugh, K.; Ramsay, A.D.

2002-01-01

We report a case of extensive fatty change in a Wilms' tumour after chemotherapy demonstrated on CT associated with an increase in tumour volume, in a 10-month-old girl with Beckwith-Wiedemann syndrome. Changes in tumour characteristics after chemotherapy on imaging usually reflect necrosis, haemorrhage and calcification. Assessment of response to therapy is dependent on a documented reduction in tumour volume. In this case, CT showed an increase in tumour size with development of an extensive fatty component following treatment. Subsequent histological examination on the nephrectomy specimen confirmed an extensive fatty component with no evidence of residual blastema. The development of such an extensive fatty component is very unusual. In this case such fatty change was an indicator of tumour sensitivity and response to treatment. (orig.)

Efficacy of ONC201 in Desmoplastic Small Round Cell Tumor.

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Hayes-Jordan, Andrea A; Ma, Xiao; Menegaz, Brian A; Lamhamedi-Cherradi, Salah-Eddine; Kingsley, Charles V; Benson, Jalen A; Camacho, Pamela E; Ludwig, Joseph A; Lockworth, Cynthia R; Garcia, Gloria E; Craig, Suzanne L

2018-05-01

Desmoplastic Small Round Cell Tumor (DSRCT) is a rare sarcoma tumor of adolescence and young adulthood, which harbors a recurrent chromosomal translocation between the Ewing's sarcoma gene (EWSR1) and the Wilms' tumor suppressor gene (WT1). Patients usually develop multiple abdominal tumors with liver and lymph node metastasis developing later. Survival is poor using a multimodal therapy that includes chemotherapy, radiation and surgical resection, new therapies are needed for better management of DSRCT. Triggering cell apoptosis is the scientific rationale of many cancer therapies. Here, we characterized for the first time the expression of pro-apoptotic receptors, tumor necrosis-related apoptosis-inducing ligand receptors (TRAILR1-4) within an established human DSRCT cell line and clinical samples. The molecular induction of TRAIL-mediated apoptosis using agonistic small molecule, ONC201 in vitro cell-based proliferation assay and in vivo novel orthotopic xenograft animal models of DSRCT, was able to inhibit cell proliferation that was associated with caspase activation, and tumor growth, indicating that a cell-based delivery of an apoptosis-inducing factor could be relevant therapeutic agent to control DSRCT. Copyright © 2018. Published by Elsevier Inc.

Skeletal changes in growing spine following radiotherapy of tumors

International Nuclear Information System (INIS)

Spissak, L.; Horniakova, M.

1987-01-01

An analysis is given of a group of 20 children after nephrectomy and radiotherapy of Wilms' tumor and of one child following ovariectomy and radiotherapy of a dysgermanoma more than 5 years after the termination of the therapy. Morphological and structural changes were evaluated in the vertebrae as well as axial alterations of the spine. Interrelationships were found between the radiation dose, the patient's age and the degree of the skeletal changes in the spine. The most pronounced morphological, structural and axial changes occurred in children below 4 years treated with radiation doses above 20.0 Gy. (author). 1 tab., 5 figs., 3 refs

Intra-Abdominal Desmoplastic Small Round Cell Tumor with Elevated Serum CA 125: A Case Report

Directory of Open Access Journals (Sweden)

Sheau-Fang Yang

2003-10-01

Full Text Available Desmoplastic small round cell tumor (DSRCT is a rare and highly aggressive tumor usually involving the peritoneum. It occurs more commonly in young males and is characterized by distinctive clinical, histologic, and immunophenotypic features. The histogenesis of DSRCT remains unknown. Coexpression of epithelial, mesenchymal, and neural antigens in the same cell provides evidence that DSRCT may arise from a primitive pluripotent stem cell with divergent differentiation. Recently, according to cytogenetic studies, some authors have proposed that the divergent differentiation of DSRCT may be the result of the fusion of Ewing's sarcoma gene and Wilms' tumor suppressor gene. Clinically, an elevated serum CA 125 concentration is found in some patients with DSRCT. We present the case of a 29-year-old man with diffuse intra-abdominal DSRCT and elevated serum CA 125 concentration and briefly review the relevant literature.

Impact of removed tumor volume and location on patient outcome in glioblastoma.

Science.gov (United States)

Awad, Al-Wala; Karsy, Michael; Sanai, Nader; Spetzler, Robert; Zhang, Yue; Xu, Yizhe; Mahan, Mark A

2017-10-01

locations). OS significantly improved with EOR in univariate analysis, averaging 22.3, 19.7, and 13.2 months for >90, 80-90, and 70-80% resection, respectively. Survival was 22.8, 19.0, and 12.7 months for 0, 0-5, and 5-10 ml postoperative residual, respectively. A hazard model showed that larger preoperative tumor volume [hazard ratio (HR) 1.05, 95% CI 1.02-1.07], greater age (HR 1.02, 95% CI 1.01-1.03), multifocality (HR 1.44, 95% CI 1.01-2.04), and deep nuclei/basal ganglia (HR 2.05, CI 1.27-3.3) were the most predictive of poor survival after adjusting for KPS and tumor location. There was a negligible but significant interaction between EOR and preoperative tumor volume (HR 0.9995, 95% CI 0.9993-0.9998), but EOR alone did not correlate with OS after adjusting for other factors. The interaction between EOR and preoperative tumor volume represented tumor volume removed during surgery. In conclusion, EOR alone was not an important predictor of outcome during GBM treatment once preoperative tumor volume, age, and deep nuclei/basal ganglia location were factored. Instead, the interaction between EOR and preoperative volume, representing reduced disease burden, was an important predictor of reducing OS. Removal of tumor from eloquent cortex did not impact postoperative KPS. These results suggest aggressive surgical treatment to reduce postoperative residual while maintaining postoperative KPS may aid patient survival outcomes for a given tumor size and location.

Unusual renal angiomyolipoma in tuberous sclerosis

International Nuclear Information System (INIS)

Schwartz, A.M.

1980-01-01

A patient with tuberous sclerosis and a normal intravenous urogram 5 years previously presented with a large and palpable upper pole renal mass. Since patients with tuberous sclerosis have small bilateral hamartomas, a Wilms' tumor was suspected. In retrospect, inhomogeneous nephrograms should have alerted the radiologist to the multiplicity of other small lesions. Also, a partially lucent rim should have substantiated that the lesion was not a Wilms' tumor. (orig.) [de

Stages of Wilms Tumor

Science.gov (United States)

... through the urethra and leaves the body. Enlarge Anatomy of the female urinary system showing the kidneys, adrenal glands, ureters, bladder, and urethra. Urine is made in the renal tubules and collects in the renal pelvis of ...

Proficiency performance benchmarks for removal of simulated brain tumors using a virtual reality simulator NeuroTouch.

Science.gov (United States)

AlZhrani, Gmaan; Alotaibi, Fahad; Azarnoush, Hamed; Winkler-Schwartz, Alexander; Sabbagh, Abdulrahman; Bajunaid, Khalid; Lajoie, Susanne P; Del Maestro, Rolando F

2015-01-01

Assessment of neurosurgical technical skills involved in the resection of cerebral tumors in operative environments is complex. Educators emphasize the need to develop and use objective and meaningful assessment tools that are reliable and valid for assessing trainees' progress in acquiring surgical skills. The purpose of this study was to develop proficiency performance benchmarks for a newly proposed set of objective measures (metrics) of neurosurgical technical skills performance during simulated brain tumor resection using a new virtual reality simulator (NeuroTouch). Each participant performed the resection of 18 simulated brain tumors of different complexity using the NeuroTouch platform. Surgical performance was computed using Tier 1 and Tier 2 metrics derived from NeuroTouch simulator data consisting of (1) safety metrics, including (a) volume of surrounding simulated normal brain tissue removed, (b) sum of forces utilized, and (c) maximum force applied during tumor resection; (2) quality of operation metric, which involved the percentage of tumor removed; and (3) efficiency metrics, including (a) instrument total tip path lengths and (b) frequency of pedal activation. All studies were conducted in the Neurosurgical Simulation Research Centre, Montreal Neurological Institute and Hospital, McGill University, Montreal, Canada. A total of 33 participants were recruited, including 17 experts (board-certified neurosurgeons) and 16 novices (7 senior and 9 junior neurosurgery residents). The results demonstrated that "expert" neurosurgeons resected less surrounding simulated normal brain tissue and less tumor tissue than residents. These data are consistent with the concept that "experts" focused more on safety of the surgical procedure compared with novices. By analyzing experts' neurosurgical technical skills performance on these different metrics, we were able to establish benchmarks for goal proficiency performance training of neurosurgery residents. This

Bilateral nephroblastoma - case report

International Nuclear Information System (INIS)

Luczynska, E.; Aniol, J.

2008-01-01

Wilms tumor is the most common renal tumor in children Synchronous bilateral Wilms tumor (BWT) accounts for 5% of all patients registered to the National Wilms Tumor Study Group (NWSTG). A 28-year-old female patient was presented to Oncology Institute with right kidney tumor. Her left kidney was resected due to Wilms tumor in the sixth month of her life. Abdominal ultrasound was performed and demonstrated a massive right kidney tumor. Then the abdominal CT was undertaken with the use of 16-slice CT scanner, revealing rotation displacement of the right kidney, with signs of compensative overgrowth and displaced upwards. In the lower and peripheral part of the kidney a nodular mass 7 X 10 X 9 cm in size was visible. The tumor was well-demarcated, showing heterogeneous contrast enhancement, extending from the inferio-lateral renal pole.The tumor was adjacent to iliopsoas muscle and abdominal cavity walls; no infiltration of those structures was noted. Described lesion surrounded inferio-medial part of the kidney, approaching kidney pelvis without infiltrating them. Supero-anterior and medial part of the kidney showed normal structure with correct contrasted urine excretion. Lymph nodes enlargement within periaortal area was not detected. Surgical procedure was performed resulting in excision of the tumor with kidney preservation. Histopathology examination revealed nephroblastoma. The tumor was surrounded by a thin, fibrous capsule. Surrounding parenchyma and far tissue were not involved. The CT examination performed 4 months after nephron sparing surgery revealed: right kidney of 137 X 51 mm in size, normally located, with correct structure and function. Multislice abdominal CT have clearly visualized the tumor of the single kidney, showing precise localization of the mass with relation to the kidney pelvis and vessels, allowing for nephron sparing surgical excision. (authors)

Study of Genistein in Pediatric Oncology Patients (UVA-Gen001)

Science.gov (United States)

2017-06-20

Lymphoma; Childhood Lymphoma; Solid Tumor; Childhood Solid Tumor; Neuroblastoma; Ewing Sarcoma; Hodgkin Lymphoma; Non-Hodgkin Lymphoma; Rhabdomyosarcoma; Soft Tissue Sarcoma; Medulloblastoma; Germ Cell Tumor; Wilms Tumor; Brain Neoplasms; Medulloblastoma, Childhood; Neuroectodermal Tumors, Primitive

The developmental programme for genesis of the entire kidney is recapitulated in Wilms tumour

Science.gov (United States)

Anaka, Matthew R.; Morison, Ian M.; Reeve, Anthony E.

2017-01-01

Wilms tumour (WT) is an embryonal tumour that recapitulates kidney development. The normal kidney is formed from two distinct embryological origins: the metanephric mesenchyme (MM) and the ureteric bud (UB). It is generally accepted that WT arises from precursor cells in the MM; however whether UB-equivalent structures participate in tumorigenesis is uncertain. To address the question of the involvement of UB, we assessed 55 Wilms tumours for the molecular features of MM and UB using gene expression profiling, immunohistochemsitry and immunofluorescence. Expression profiling primarily based on the Genitourinary Molecular Anatomy Project data identified molecular signatures of the UB and collecting duct as well as those of the proximal and distal tubules in the triphasic histology group. We performed immunolabeling for fetal kidneys and WTs. We focused on a central epithelial blastema pattern which is the characteristic of triphasic histology characterized by UB-like epithelial structures surrounded by MM and MM-derived epithelial structures, evoking the induction/aggregation phase of the developing kidney. The UB-like epithelial structures and surrounding MM and epithelial structures resembling early glomerular epithelium, proximal and distal tubules showed similar expression patterns to those of the developing kidney. These observations indicate WTs can arise from a precursor cell capable of generating the entire kidney, such as the cells of the intermediate mesoderm from which both the MM and UB are derived. Moreover, this provides an explanation for the variable histological features of mesenchymal to epithelial differentiation seen in WT. PMID:29040332

Molecular-Guided Therapy for Childhood Cancer

Science.gov (United States)

2017-07-07

Neuroblastoma; Medulloblastoma; Glioma; Ependymoma; Choroid Plexus Neoplasms; Craniopharyngioma; Dysembryoplastic Neuroepithelial Tumor; Meningioma; Primitive Neuroectodermal Tumors (PNETs); Germ Cell Tumors; Rhabdomyosarcoma; Non-rhabdomyosarcoma; Ewings Sarcoma; Osteosarcoma; Wilms Tumor; Renal Cell Carcinoma; Malignant Rhabdoid Tumor; Clear Cell Sarcoma; Liver Tumors

Maternal Residential Proximity to Major Roadways and Pediatric Embryonal Tumors in Offspring

Directory of Open Access Journals (Sweden)

Shwetha V. Kumar

2018-03-01

Full Text Available The environmental determinants of pediatric embryonal tumors remain unclear. Because of the growing concern over the impact of exposures to traffic-related air pollution on pediatric cancer, we conducted a population-based study evaluating the impact of maternal residential proximity to major roadways on the risk of pediatric embryonal tumors in offspring. We identified children diagnosed with neuroblastoma, Wilms tumor, retinoblastoma, or hepatoblastoma at <5 years of age from the Texas Cancer Registry and selected unaffected controls from birth certificates. Two residential proximity measures were used: (1 distance to the nearest major roadway, and (2 within 500 m of a major roadway. Logistic regression was used to estimate the adjusted odds ratio (aOR and 95% confidence interval (CI for each proximity measure on pediatric embryonal tumors. The odds of an embryonal tumor were increased in children born to mothers living within 500 m of a major roadway (aOR = 1.24, 95% CI: 1.00, 1.54. This was consistent for most tumor subtypes, with the strongest associations observed for unilateral retinoblastoma (aOR = 2.57, 95% CI: 1.28, 5.15, for every kilometer closer the mother lived to the nearest major roadway. These findings contribute to the growing evidence that traffic-related air pollution may increase risk for certain pediatric tumors.

Pattern of malignant solid tumors and lymphomas in children in the east delta of Egypt: A five-year study.

Science.gov (United States)

Hesham, Mervat; Atfy, Mervat; Hassan, Tamer; Abdo, Mohamed; Morsy, Saed; El Malky, Mohamed; Latif, Dalia Abdel

2014-11-01

Worldwide, the incidence and mortality rates of childhood cancers differ. The study of incidence patterns and survival rates in childhood malignancies is important in aiding in the planning of treatment centers and in obtaining further information with regard to the etiology. Few studies have investigated the survival in cases of childhood solid tumors in Egypt. The aim of the current study was to evaluate the patterns, frequency and outcome of solid tumors and lymphomas in children admitted to and followed up at the Pediatric Oncology Department of Zagazig University Hospital (Zagazig, Egypt) over a duration of 5 years (January 2004 to December 2008). A retrospective study was conducted, which included 155 children with solid tumors and lymphomas. The medical records were reviewed and the relevant data collected, in particular, those concerning demographic, clinical, histopathological, laboratory and imaging data as well as the treatment plans and outcomes. The mean age of patients was 5.6±3.04 years at diagnosis. The patients comprised 94 males and 61 females. Non-Hodgkin lymphoma (NHL) was the most common tumor type, followed by neuroblastoma (31.0 and 29.0%, respectively). When patients were stratified in terms of age (<5, ≥5 but <10, and ≥10 years), the <5-years-of-age group exhibited the greatest number of patients. Fever, pallor and pain were the most frequent initial clinical presentations among the patients and stage II was the most common stage (39.1%) followed by stage IV, III and I (35.0, 20.3 and 5.6% respectively). The overall 5-year survival rate in the study group was 66.7%. The survival rate was significantly higher in patients with Wilm's tumor and Hodgkin lymphoma, followed by NHL (92.0, 88.0 and 72.0%, respectively; P<0.001), while the mortality rate was significantly higher in patients with neuroblastoma (P<0.001). In conclusion, NHL and neuroblastoma were the most common tumors; the survival rates were higher in patients with Wilm's tumor

Submicroscopic deletions at the WAGR locus, revealed by nonradioactive in situ hybridization.

OpenAIRE

Fantes, J A; Bickmore, W A; Fletcher, J M; Ballesta, F; Hanson, I M; van Heyningen, V

1992-01-01

Fluorescence in situ hybridization (FISH) with biotin-labeled probes mapping to 11p13 has been used for the molecular analysis of deletions of the WAGR (Wilms tumor, aniridia, genitourinary abnormalities, and mental retardation) locus. We have detected a submicroscopic 11p13 deletion in a child with inherited aniridia who subsequently presented with Wilms tumor in a horseshoe kidney, only revealed at surgery. The mother, who has aniridia, was also found to carry a deletion including both the ...

Frequent Chromosome Aberrations Revealed by Molecular Cytogenetic Studies in Patients with Aniridia

OpenAIRE

Crolla, John A.; van Heyningen, Veronica

2002-01-01

Seventy-seven patients with aniridia, referred for cytogenetic analysis predominantly to assess Wilms tumor risk, were studied by fluorescence in situ hybridization (FISH), through use of a panel of cosmids encompassing the aniridia-associated PAX6 gene, the Wilms tumor predisposition gene WT1, and flanking markers, in distal chromosome 11p13. Thirty patients were found to be chromosomally abnormal. Cytogenetically visible interstitial deletions involving 11p13 were found in 13 patients, 11 o...

Endoscopic transnasal approach for removing pituitary tumors

Directory of Open Access Journals (Sweden)

Mirian Cabral Moreira de Castro

2014-05-01

Full Text Available To describe a series of 129 consecutive patients submitted to the resection of pituitary tumors using the endoscopic transsphenoidal approach in a public medical center. Method: Retrospective analysis based on the records of patients submitted to the resection of a pituitary tumor through the endoscopic transsphenoidal approach between 2004 and 2009. Results: One hundred and twenty-nine records were analyzed. The tumor was non-secreting in 96 (74.42% and secreting in 33 patients (22.58%. Out of the secretory tumors, the most prevalent was the growth hormone producer (7.65%, followed by the prolactinoma, (6.98%. Eleven patients developed cerebral spinal fluid (CSF fistulas, and four of them developed meningitis. One patient died due to intracerebral hemorrhage in the postoperative period. Conclusion: The endoscopic transsphenoidal approach to sellar tumors proved to be safe when the majority of the tumors were non-secreting. The most frequent complication was CSF. This technique can be done even in a public hospital with financial limits, since the health professionals are integrated.

Evaluation of malignant solid tumor in childhood with FDG-PET

International Nuclear Information System (INIS)

Ishida, Amane; Goto, Hiroaki; Kuroki, Fumiko

2006-01-01

Usefulness of FDG-PET (18F-deoxyglucose PET) was examined in evaluation of diagnosis and therapeutic efficacy of childhood malignant solid tumors. Subjects were 32 patients (16 males) of the median age of 7 y (1 - 27 y), involving those with neuroblastoma (9 cases), hepatoblastoma (4), chronic granulomatous disorder (4) and others (each ≤2). They underwent 75 FDG-PET examinations for diagnosis before and during treatment in authors' hospital in the period from May 2001 to December 2003. Standard uptake value (SUV), 1 x 1 cm region of interest (ROI) of abnormally high distribution area of radioactivity in the lesion/FDG dose/kg body wt., was used for evaluation: SUV>1.5 was defined positive. In neuroblastoma, FDG was found to be highly distributed and kinetics of SUV, to be useful for evaluation of therapeutic efficacy and early metastasis detection. In some cases of hepatoblastoma, the therapeutic effectiveness and recurrence were not satisfactorily evaluative. The distribution of FDG was not satisfactory in Wilms' tumor relative to other tumors. The PET was thought to be useful, despite their small case number examined, for those evaluations of Ewing's tumor, dysgerminoma and Langerhans cell histiocytosis. Thus FDG-PET was found useful for detection, evaluation of therapeutic efficacy and early metastasis detection of pediatric malignant solid tumors. (T.I.)

Proceedings of the 3. Muenster symposium on late effects after tumor therapy in childhood and adolescence. Abstracts

International Nuclear Information System (INIS)

Willich, Normann; Boelling, Tobias

2009-01-01

The volume on the 3rd Muenster Symposion on late effects after tumor therapy in childhood and adolescence contains 7 contributions: Evaluation of side effects after radiotherapy in childhood and adolescence; from retrospective case reports to a perspective, multicentric and transnational approach; late effects surveillance system after childhood cancer in Germany, Austria and parts of Switzerland - update 2009; second malignant neoplasm after childhood cancer in Germany - results from the long-term follow-up of the German childhood cancer registry; secondary neoplasm after Wilm's tumor in Germany; second cancer after total-body irradiation (TBI) in childhood; late toxicity in children undergoing hematopoietic stem cell transplantation with TBI-containing conditioning regimens for hematological malignancies; radiation toxicity following busulfan/melphalan high-dose chemotherapy in the EURO-EWING-99-trials: review of GPOH data

Proceedings of the 3. Muenster symposium on late effects after tumor therapy in childhood and adolescence. Abstracts

Energy Technology Data Exchange (ETDEWEB)

Willich, Normann; Boelling, Tobias (eds.) [Univ. Hospital Muenster (Germany). Dept. of Radiotherapy

2009-08-15

The volume on the 3rd Muenster Symposion on late effects after tumor therapy in childhood and adolescence contains 7 contributions: Evaluation of side effects after radiotherapy in childhood and adolescence; from retrospective case reports to a perspective, multicentric and transnational approach; late effects surveillance system after childhood cancer in Germany, Austria and parts of Switzerland - update 2009; second malignant neoplasm after childhood cancer in Germany - results from the long-term follow-up of the German childhood cancer registry; secondary neoplasm after Wilm's tumor in Germany; second cancer after total-body irradiation (TBI) in childhood; late toxicity in children undergoing hematopoietic stem cell transplantation with TBI-containing conditioning regimens for hematological malignancies; radiation toxicity following busulfan/melphalan high-dose chemotherapy in the EURO-EWING-99-trials: review of GPOH data.

Strategy of diagnosis and treatment for pediatric solid tumor patients using FDG-PET

International Nuclear Information System (INIS)

Hosono, Ako; Watanabe, Atsuko; Tsuji, Naoko; Kawamoto, Hiroshi; Makimoto, Atsushi; Tateishi, Ukihide; Terauthi, Takashi

2006-01-01

Usefulness of FDG-PET (18F-deoxyglucose PET) was investigated in diagnosis and therapeutic planning of childhood and adolescence malignant solid tumors. Evidence was based on 46 patients (25 males) of ages 5-30 y, involving those with rhabdomyosarcoma (17 cases), Ewing's sarcoma (13), osteosarcoma (5), neuroblastoma (4), Wilms' tumor (2), germinoma (2), and each 1 case of ganglioblastoma, retinoblastoma and hepatoblastoma. In total, they underwent 104 FDG-PET examinations for diagnosis before and during treatment in authors' hospital in the period from January 2005 to February 2006. Evaluations were done with the standard uptake value (SUV, 1 x 1 cm ROI of abnormally high distribution area of radioactivity in the lesion/FDG dose/kg body wt.), by recurrence, by early detection of exacerbation and by follow up of residual tumors, of which typical image findings were herein presented. From the aspects of the present purposes, it was concluded that FDG-PET had advantages of high resolution, short imaging time, quantitative diagnosis (SUV) as well as the tumor detection, and had defects of difficulty of detection of tumors of <1 cm size, of distribution to normal or benign tissues and of difficulty of central nervous system (CNS) imaging. (T.I.)

Reduction of postoperative chemotherapy in children with stage I intermediate-risk and anaplastic Wilms' tumour (SIOP 93-01 trial): a randomised controlled trial

NARCIS (Netherlands)

de Kraker, J.; Graf, N.; van Tinteren, H.; Pein, F.; Sandstedt, B.; Godzinski, J.; Tournade, M. F.

2004-01-01

Background Present treatment for Wilms' tumour is very successful. Now, efforts are aimed at reducing toxicity and burden of treatment by shortening schedules without loss of effectiveness. The objective of this randomised trial was to assess whether postoperative chemotherapy for patients with

Ovarian lymphoma

International Nuclear Information System (INIS)

Bonet Fonseca, Ivan; Diaz Anaya, Amnia; Francis, Tabu

2012-01-01

50 % of pediatric oncologic pathology corresponds to mass or solid tumors, reaching about 20 % of total abdomen. The tumors that most frequently occur in the abdomen are nephroblastoma or Wilms tumor, Burkitts lymphoma, neuroblastoma, and ovarian germ cell tumors

Skeletal sequelae of radiation therapy for malignant childhood tumors

International Nuclear Information System (INIS)

Butler, M.S.; Robertson, W.W. Jr.; Rate, W.; D'Angio, G.J.; Drummond, D.S.

1990-01-01

One hundred forty-three patients who received radiation therapy for childhood tumors, and survived to the age of skeletal maturity, were studied by retrospective review of oncology records and roentgenograms. Diagnoses for the patients were the following: Hodgkin's lymphoma (44), Wilms's tumor (30), acute lymphocytic leukemia (26), non-Hodgkin's lymphoma (18), Ewing's sarcoma (nine), rhabdomyosarcoma (six), neuroblastoma (six), and others (four). Age at the follow-up examination averaged 18 years (range, 14-28 years). Average length of follow-up study was 9.9 years (range, two to 18 years). Asymmetry of the chest and ribs was seen in 51 (36%) of these children. Fifty (35%) had scoliosis; 14 had kyphosis. In two children, the scoliosis was treated with a brace, while one developed significant kyphosing scoliosis after laminectomy and had spinal fusion. Twenty-three (16%) patients complained of significant pain at the radiation sites. Twelve of the patients developed leg-length inequality; eight of those were symptomatic. Three patients developed second primary tumors. Currently, the incidence of significant skeletal sequelae is lower and the manifestations are less severe than reported in the years from 1940 to 1970. The reduction in skeletal complications may be attributed to shielding of growth centers, symmetric field selection, decreased total radiation doses, and sequence changes in chemotherapy

Co-culture with podoplanin+ cells protects leukemic blast cells with leukemia-associated antigens in the tumor microenvironment.

Science.gov (United States)

Lee, Ji Yoon; Han, A-Reum; Lee, Sung-Eun; Min, Woo-Sung; Kim, Hee-Je

2016-05-01

Podoplanin+ cells are indispensable in the tumor microenvironment. Increasing evidence suggests that podoplanin may support the growth and metastasis of solid tumors; however, to the best of our knowledge no studies have determined whether or not podoplanin serves a supportive role in acute myeloid leukemia (AML). The effects of co‑culture with podoplanin+ cells on the cellular activities of the leukemic cells, such as apoptosis and cell proliferation, in addition to the expression of podoplanin in leukemic cells, were investigated. Due to the fact that genetic abnormalities are the primary cause of leukemogenesis, the overexpression of the fibromyalgia‑like tyrosine kinase‑3 gene in colony forming units was also examined following cell sorting. Podoplanin+ cells were found to play a protective role against apoptosis in leukemic cells and to promote cell proliferation. Tumor‑associated antigens, including Wilms' tumor gene 1 and survivin, were increased when leukemic cells were co‑cultured with podoplanin+ cells. In combination, the present results also suggest that podoplanin+ cells can function as stromal cells for blast cell retention in the AML tumor microenvironment.

Removal of an intra-abdominal desmoplastic small round cell tumor by repetitive debulking surgery: A case report and literature review.

Science.gov (United States)

Shimazaki, Jiro; Motohashi, Gyo; Nishida, Kiyotaka; Tabuchi, Takanobu; Ubukata, Hideyuki; Tabuchi, Takafumi

2014-05-01

In the current study, a case of recurrent desmoplastic small round cell tumor (DSRCT) is presented, which was successfully treated by repetitive debulking surgery. In May 2010, a 39-year-old male, with a history of surgical resection of intra-abdominal DSRCT, visited the Ibaraki Medical Center, Tokyo Medical University Hospital (Ami, Japan) with severe lower abdominal discomfort. Abdominal computed tomography revealed a large tumor in the pouch of Douglas with a small number of nodules in the abdominal cavity. The recurrent DSRCT was diagnosed and removed via lower anterior resection; however, complete resection was impossible due to multiple peritoneal metastases. One year later, the patient developed pain in the right groin due to the growth of metastasized tumor cells in the groin lymph nodes. The affected lymph nodes were removed utilizing an extra-peritoneal approach. At the time of writing, the patient continues to survive without any symptoms 60 months since the initial surgery. In conclusion, surgical debulking is a significant procedure for relieving patient symptoms as well as improving the survival time of patients with metastatic and recurrent DSRCT.

Cross-sectional imaging with CT and/or MRI of pediatric chest tumors

International Nuclear Information System (INIS)

Wyttenbach, R.; Vock, P.; Tschaeppeler, H.

1998-01-01

The purpose of this study was to provide an overview of the spectrum of pediatric chest masses, to present the results of cross-sectional imaging with CT and/or MRI, and to define diagnostic criteria to limit differential diagnosis. Seventy-eight children with thoracic mass lesions were retrospectively evaluated using CT (72 patients) and/or MR imaging (12 patients). All masses were evaluated for tissue characteristics (attenuation values or signal intensity, enhancement, and calcification) and were differentiated according to age, gender, location, and etiology. Twenty-eight of 38 (74 %) mediastinal masses were malignant (neuroblastoma, malignant lymphoma). Thirty of 38 (79 %) pulmonary masses were metastatic in origin, all with an already known primary tumor (osteosarcoma, Wilms tumor). With one exception, all remaining pulmonary lesions were benign. Seventeen of 21 (81 %) chest wall lesions were malignant (Ewing sarcoma, primitive neuroectodermal tumor). The majority of mediastinal and chest wall tumors in children is malignant. Lung lesions are usually benign, unless a known extrapulmonary tumor suggests pulmonary metastases. Cross-sectional imaging with CT and/or MRI allows narrowing of the differential diagnosis of pediatric chest masses substantially by defining the origin and tissue characteristics. Magnetic resonance imaging is preferred for posterior mediastinal lesions, whereas CT should be used for pulmonary lesions. For the residual locations both modalities are complementary. (orig.)

Treatment Options for Wilms Tumor

Science.gov (United States)

... factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment options ... come back) after it has been treated. Treatment Option Overview Key Points There are different types of ...

The effects of preoperative oral administration of carprofen or tramadol on postoperative analgesia in dogs undergoing cutaneous tumor removal.

Science.gov (United States)

Karrasch, Nicole M; Lerche, Phillip; Aarnes, Turi K; Gardner, Heather L; London, Cheryl A

2015-08-01

This prospective, blinded, controlled clinical study compared the effects of pre-emptive oral administration of carprofen or tramadol on pain scores and analgesic requirement in dogs undergoing cutaneous tumor removal. Thirty-six client-owned dogs presenting for cutaneous tumor removal were randomly assigned to receive carprofen, tramadol, or no treatment prior to surgery. Pain was assessed using a visual analog scale (VAS), the Modified Glasgow Composite Measure Pain Score (MGCMPS), and algometry at enrollment, prior to premedication, at extubation, then hourly for the first 4 h, and every 4 h for 24 h. Dogs scoring ≥ 7 (MGCMPS), or having a VAS measurement ≥ 40 mm were given rescue analgesia. There were no significant differences in pain VAS, MGCMPS, or algometry. There were no differences in rescue analgesia requirement, or time to rescue analgesia among groups. Carprofen, tramadol, or no pre-emptive analgesia, combined with pre-operative hydromorphone and rescue analgesia, resulted in satisfactory analgesia in the 24-hour postoperative period.

Tumores sólidos en niños: diagnóstico y terapéutica quirúrgica

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G. Gloria González, Dra.

2010-01-01

Full Text Available Los tumores malignos en pediatría representan sólo el 2% de los casos de cáncer, sin embargo, las neoplasias son en la actualidad la segunda causa de muerte en niños mayores de 1 año de edad. Cada año se diagnostican aproximadamente 130 nuevos casos de cáncer por millón de niños. La leucemia es el más frecuente de los cánceres pediátricos, seguidos por tumores de cerebro, linfomas, neuroblastomas, sarcomas, tumores de Wilms y tumor de células germinales. La probabilidad de sobrevivir a una enfermedad maligna ha mejorado desde que en 1950 se reportaron las primeras remisiones en leucemia linfocítica aguda. Actualmente, debido al desarrollo de la quimioterapia, mejora en los métodos diagnósticos y el manejo multidisciplinario de los pacientes, el porcentaje de curación es cercano a un 75%. A pesar de estos avances, es aún necesario una mejoría de los resultados en el cáncer pediátrico, que depende del diagnóstico temprano de la enfermedad.

Diagnostic accuracy of risk of malignancy index in predicting complete tumor removal at primary debulking surgery for ovarian cancer patients

DEFF Research Database (Denmark)

Fagö-Olsen, Carsten L; Håkansson, Fanny; Antonsen, Sofie L

2013-01-01

Ovarian cancer patients in whom complete tumor removal is impossible with primary debulking surgery (PDS) may benefit from neoadjuvant chemotherapy and interval debulking surgery. However, the task of performing a pre-operative evaluation of the feasibility of PDS is difficult. We aimed to invest...

Sinusoidal Obstruction Syndrome During Chemotherapy of Pediatric Cancers and its Successful Management With Defibrotide.

Science.gov (United States)

Kizilocak, Hande; Dikme, Gürcan; Özdemir, Nihal; Kuruğoğlu, Sebuh; Adaletli, İbrahim; Erkan, Tülay; Celkan, Tiraje

2017-10-01

Sinusoidal obstruction syndrome (SOS) is a life-threatening complication generally occurring after hematopoietic stem cell transplantation. SOS after standard dose chemotherapy in malignancies is rare. Between the year 1995 and 2016, 414 patients were diagnosed with acute lymphoblastic leukemia and 113 patients were diagnosed with Wilms tumor in our institution. Among these patients, 4 patients with acute lymphoblastic leukemia (0.96%) and 2 patients with Wilms tumor (1.7%) developed SOS during treatment. SOS behaves like a local disseminated intravascular coagulation. Defibrotide has proved to be effective in SOS. In this article, we report our experience with defibrotide in SOS.

Recent advances in the management of Wilms' tumor [version 1; referees: 2 approved

Directory of Open Access Journals (Sweden)

Roberto I. Lopes

2017-05-01

Full Text Available The objective of this article is to present an overview of recent trends in the management of Wilms’ tumor. With improved survival rates in the past few decades, critical long-term adverse therapy effects (such as renal insufficiency, secondary malignancies, and heart failure and prevention measures (i.e. nephron-sparing surgery and minimizing the use of radiotherapy have gained worldwide attention. Specific disease biomarkers that could help stratify high-risk from low-risk patients, and therefore fine-tune management, are in great demand. Ultimately, we aim to enhance clinical outcomes and maintain or improve current survival rates while avoiding undesirable treatment side effects and minimizing the exposure and intensity of chemotherapy and radiotherapy.

[CLINICAL APPLICATION AND EXPERIENCE IN RECONSTRUCTION OF SOFT TISSUE DEFECTS FOLLOWING MALIGNANT TUMOR REMOVAL OF LIMBS USING PERFORATOR PROPELLER FLAPS].

Science.gov (United States)

Zhu, Shan; Liu, Yuanbo; Yu, Shengji; Zang, Mengqing; Zhao, Zhenguo; Xu, Libin; Zhang, Xinxin; Chen, Bo; Ding, Qiang

2016-01-01

To explore the feasibility and technical essentials of soft tissue defect reconstruction following malignant tumor removal of limbs using perforator propeller flaps. Between July 2008 and July 2015, 19 patients with malignant limb tumor underwent defect reconstruction following tumor removal using the perforator propeller flaps. There were 13 males and 6 females with an average age of 53.4 years (range, 20-82 years). The disease duration ranged from 1 to 420 months (mean, 82 months). The tumors located at the thigh in 10 cases, at the leg in 2 cases, at the arm in 1 case, at the forearm in 1 case, around the knee in 2 cases, and around the elbow joint in 3 cases. Totally 23 flaps (from 8 cm x 3 cm to 30 cm x 13 cm in size) were used to reconstruct defects (from 4 cm x 4 cm to 24 cm x 16 cm in size). The potential source arteries included the femoral artery (n = 2), profunda femoral artery (n = 3), superficial circumflex iliac artery (n = 1), lateral circumflex femoral artery (n = 6), superior lateral genicular artery (n = 2), peroneal artery (n = 2), anterior tibial artery (n = 1), brachial artery (n = 4), and radial artery (n = 1). The remaining one was a free style perforator flap. Partial distal flap necrosis occurred in 3 cases after surgery with rotation angles of 180, 150, and 100 degrees respectively, which were reconstructed after debridement using a free-style perforator flap in 1 case and using free skin grafting in the other 2 cases. The other 20 flaps survived completely after surgery. Primary healing of incisions was obtained at the donor and recipient sites. There was no severe complication such as infection, hematoma, and total flap failure. All patients were followed up 3 months to 5 years (mean, 19 months). One patient with malignant melanoma around the elbow joint had tumor recurrence, and underwent secondary tumor resection. The appearance, texture, and color of the flaps were similar to those at the recipient site. For patients with malignant

Risk Factors for Postoperative Fibrinogen Deficiency after Surgical Removal of Intracranial Tumors.

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Naili Wei

Full Text Available Higher levels of fibrinogen, a critical element in hemostasis, are associated with increased postoperative survival rates, especially for patients with massive operative blood loss. Fibrinogen deficiency after surgical management of intracranial tumors may result in postoperative intracranial bleeding and severely worsen patient outcomes. However, no previous studies have systematically identified factors associated with postoperative fibrinogen deficiency. In this study, we retrospectively analyzed data from patients who underwent surgical removal of intracranial tumors in Beijing Tiantan Hospital date from 1/1/2013to12/31/2013. The present study found that patients with postoperative fibrinogen deficiency experienced more operative blood loss and a higher rate of postoperative intracranial hematoma, and they were given more blood transfusions, more plasma transfusions, and were administered larger doses of hemocoagulase compared with patients without postoperative fibrinogen deficiency. Likewise, patients with postoperative fibrinogen deficiency had poorer extended Glasgow Outcome Scale (GOSe, longer hospital stays, and greater hospital expenses than patients without postoperative fibrinogen deficiency. Further, we assessed a comprehensive set of risk factors associated with postoperative fibrinogen deficiency via multiple linear regression. We found that body mass index (BMI, the occurrence of postoperative intracranial hematoma, and administration of hemocoagulasewere positively associated with preoperative-to-postoperative plasma fibrinogen consumption; presenting with a malignant tumor was negatively associated with fibrinogen consumption. Contrary to what might be expected, intraoperative blood loss, the need for blood transfusion, and the need for plasma transfusion were not associated with plasma fibrinogen consumption. Considering our findings together, we concluded that postoperative fibrinogen deficiency is closely associated with

Pneumothorax as a complication of combination antiangiogenic therapy in children and young adults with refractory/recurrent solid tumors.

Science.gov (United States)

Interiano, Rodrigo B; McCarville, M Beth; Wu, Jianrong; Davidoff, Andrew M; Sandoval, John; Navid, Fariba

2015-09-01

Antiangiogenic agents show significant antitumor activity against various tumor types. In a study evaluating the combination of sorafenib, bevacizumab, and low-dose cyclophosphamide in children with solid tumors, an unexpectedly high incidence of pneumothorax was observed. We evaluated patient characteristics and risk factors for the development of pneumothorax in patients receiving this therapy. Demographics, clinical course, and radiographic data of 44 patients treated with sorafenib, bevacizumab and cyclophosphamide were reviewed. Risk factors associated with the development of pneumothorax were analyzed. Pneumothorax likely related to study therapy developed in 11 of 44 (25%) patients of whom 33 had pulmonary abnormalities. Median age of patients was 14.7 years (range, 1.08-24.5). Histologies associated with pneumothorax included rhabdoid tumor, synovial sarcoma, osteosarcoma, Ewing sarcoma, Wilms tumor, and renal cell carcinoma. Cavitation of pulmonary nodules in response to therapy was associated with pneumothorax development (Ppneumothorax was 5.7 weeks (range, 2.4-31). The development of cavitary pulmonary nodules in response to therapy is a risk factor for pneumothorax. As pneumothorax is a potentially life-threatening complication of antiangiogenic therapy in children with solid tumors, its risk needs to be evaluated when considering this therapy. Copyright © 2015 Elsevier Inc. All rights reserved.

TP53 alterations in Wilms tumour represent progression events with strong intratumour heterogeneity that are closely linked but not limited to anaplasia.

Science.gov (United States)

Wegert, Jenny; Vokuhl, Christian; Ziegler, Barbara; Ernestus, Karen; Leuschner, Ivo; Furtwängler, Rhoikos; Graf, Norbert; Gessler, Manfred

2017-10-01

TP53 mutations have been associated with anaplasia in Wilms tumour, which conveys a high risk for relapse and fatal outcome. Nevertheless, TP53 alterations have been reported in no more than 60% of anaplastic tumours, and recent data have suggested their presence in tumours that do not fulfil the criteria for anaplasia, questioning the clinical utility of TP53 analysis. Therefore, we characterized the TP53 status in 84 fatal cases of Wilms tumour, irrespective of histological subtype. We identified TP53 alterations in at least 90% of fatal cases of anaplastic Wilms tumour, and even more when diffuse anaplasia was present, indicating a very strong if not absolute coupling between anaplasia and deregulation of p53 function. Unfortunately, TP53 mutations do not provide additional predictive value in anaplastic tumours since the same mutation rate was found in a cohort of non-fatal anaplastic tumours. When classified according to tumour stage, patients with stage I diffuse anaplastic tumours still had a high chance of survival (87%), but this rate dropped to 26% for stages II-IV. Thus, volume of anaplasia or possible spread may turn out to be critical parameters. Importantly, among non-anaplastic fatal tumours, 26% had TP53 alterations, indicating that TP53 screening may identify additional cases at risk. Several of these non-anaplastic tumours fulfilled some criteria for anaplasia, for example nuclear unrest, suggesting that such partial phenotypes should be under special scrutiny to enhance detection of high-risk tumours via TP53 screening. A major drawback is that these alterations are secondary changes that occur only later in tumour development, leading to striking intratumour heterogeneity that requires multiple biopsies and analysis guided by histological criteria. In conclusion, we found a very close correlation between histological signs of anaplasia and TP53 alterations. The latter may precede development of anaplasia and thereby provide diagnostic value

Survivin selective inhibitor YM155 induce apoptosis in SK-NEP-1 Wilms tumor cells

International Nuclear Information System (INIS)

Tao, Yan-Fang; Wu, Dong; Wang, Na; Feng, Xing; Li, Yan-Hong; Ni, Jian; Wang, Jian; Pan, Jian; Lu, Jun; Du, Xiao-Juan; Sun, Li-Chao; Zhao, Xuan; Peng, Liang; Cao, Lan; Xiao, Pei-Fang; Pang, Li

2012-01-01

Survivin, a member of the family of inhibitor of apoptosis proteins, functions as a key regulator of mitosis and programmed cell death. YM155, a novel molecular targeted agent, suppresses survivin, which is overexpressed in many tumor types. The aim of this study was to determine the antitumor activity of YM155 in SK-NEP-1 cells. SK-NEP-1 cell growth in vitro and in vivo was assessed by MTT and nude mice experiments. Annexin V/propidium iodide staining followed by flow cytometric analysis was used to detect apoptosis in cell culture. Then gene expression profile of tumor cells treated with YM155 was analyzed with real-time PCR arrays. We then analyzed the expression data with MEV (Multi Experiment View) cluster software. Datasets representing genes with altered expression profile derived from cluster analyses were imported into the Ingenuity Pathway Analysis tool. YM155 treatment resulted in inhibition of cell proliferation of SK-NEP-1cells in a dose-dependent manner. Annexin V assay, cell cycle, and activation of caspase-3 demonstrates that YM155 induced apoptosis in SK-NEP-1 cells. YM155 significantly inhibited growth of SK-NEP-1 xenografts (YM155 5 mg/kg: 1.45 ± 0.77 cm 3 ; YM155 10 mg/kg: 0.95 ± 0.55 cm 3 ) compared to DMSO group (DMSO: 3.70 ± 2.4 cm 3 ) or PBS group cells (PBS: 3.78 ± 2.20 cm 3 , ANOVA P < 0.01). YM155 treatment decreased weight of tumors (YM155 5 mg/kg: 1.05 ± 0.24 g; YM155 10 mg/kg: 0.72 ± 0.17 g) compared to DMSO group (DMSO: 2.06 ± 0.38 g) or PBS group cells (PBS: 2.36 ± 0.43 g, ANOVA P < 0.01). Real-time PCR array analysis showed between Test group and control group there are 32 genes significantly up-regulated and 54 genes were significantly down-regulated after YM155 treatment. Ingenuity pathway analysis (IPA) showed cell death was the highest rated network with 65 focus molecules and the significance score of 44. The IPA analysis also groups the differentially expressed genes into biological mechanisms that are related to cell

[Microsurgical removal of olfactory groove meningiomas].

Science.gov (United States)

Liang, Ri-Sheng; Zhou, Liang-Fu; Mao, Ying; Zhang, Rong; Yang, Wei-Zhong

2011-01-01

To explore an effective method for further improving the surgical results of treatment of olfactory groove meningiomas. Sixty seven cases of olfactory groove meningiomas were treated by microneurosurgery, among which fifty seven were de novo cases, eight were recurrent tumors and the other two re-recurrent cases. Modified Derome approach was used in 12 cases, bilateral subfrontal approach in 28 cases, modified pterional approach in 21 cases and unilateral subfrontal approach in six cases. Tumors were resected microsurgically with radical removal of invaded dura, bone, and paranasal sinus mucosa. Reconstruction was performed in patients with skull base defect. Simpson grade I removal was accomplished in 59 cases, grade II in seven cases and grade IV in one case. Among 57 patients with de novo tumor, Simpson I resection was accomplished in 54 cases. Postoperative rhinorrhea and intracranial infection occurred in one case and was cured after temporal lumbar CSF drainage and antibiotic therapy. Two patients (2.9%) died within one month after operation, i.e.one aged patient of heart failure and the other of severe hypothalamus complication. Forty seven patients (72.3%) were followed up from one to ten years with an average of five years and four months. With the exception of two cases died, among the alive 45 patients, there were only three patients with tumor recurrence, which had undergone Simpson II or IV tumor resection. No recurrence was found in cases with Simpson I tumor removal. Previous blurred vision was not improved in three patients, hemiparalysis in two patients, and the other patients recovered well, resuming previous jobs or being able to take care themselves. Total tumor removal (Simpson I) should be the surgical goal for treatment of olfactory groove meningiomas, especially for de novo cases. An appropriate approach is fundamental in the effort to remove an OGM totally. Appropriate anterior skull base reconstruction with vascularized material is

No adverse effect of vincristine on handwriting in children after completion of therapy

NARCIS (Netherlands)

Hartman, A.; van den Bos, C.; van Dartel, N.; Stijnen, Th; Pieters, R.

2007-01-01

Long-term writing difficulties in children after treatment with vincristine for acute lymphoblastic leukemia, Wilms tumor, B non-Hodgkin lymphoma, and malignant mesenchymal tumors, were investigated. Handwriting of 33 survivors and 33 controls matched for age, sex, and grade, was assessed with the

Atypical choroid plexus papilloma: spontaneous resolution of diffuse leptomeningeal contrast enhancement after primary tumor removal in 2 pediatric cases.

Science.gov (United States)

Scala, Marcello; Morana, Giovanni; Milanaccio, Claudia; Pavanello, Marco; Nozza, Paolo; Garrè, Maria Luisa

2017-09-01

Atypical choroid plexus papillomas can metastasize in the form of leptomeningeal seeding. Postoperative chemotherapy is the recommended first-line treatment when gross-total removal is not achieved or in cases of disseminated disease. Here the authors report on 2 children with atypical choroid plexus papillomas and MRI findings of diffuse leptomeningeal enhancement at diagnosis, later presenting with spontaneous resolution of the leptomeningeal involvement after removal of the primary lesions. Observations in this report expand our knowledge about the natural history and biological behavior of these tumors and highlight the role of close neuroimaging surveillance in the management of atypical choroid plexus papillomas in cases with MRI evidence of diffuse leptomeningeal enhancement at presentation.

Improving Care for Children With Cancer in Low- and Middle-Income Countries--a SIOP PODC Initiative.

Science.gov (United States)

Arora, Ramandeep Singh; Challinor, Julia M; Howard, Scott C; Israels, Trijn

2016-03-01

The Paediatric Oncology in Developing Countries (PODC) committee of International Society of Paediatric Oncology (SIOP) has 10 working groups that provide a forum for individuals to engage, network, and implement improvements in the care of children with cancer in low- and middle-income countries. The development of adapted guidelines (medulloblastoma, retinoblastoma, Wilms tumor, neuroblastoma, retinoblastoma, Burkitt lymphoma, supportive care), advocacy and awareness (on hospital detention and essential drugs), education and training, and global mapping (nutritional practice, abandonment rates, and twinning collaborations) have been the initial areas of focus, and the impact of some of these activities is evident, for example, in the SIOP Africa PODC Collaborative Wilms tumor project. © 2015 Wiley Periodicals, Inc.

[Neuronavigator-guided microsurgery for resection of brain tumors].

Science.gov (United States)

Zhang, Xiang; Zhang, Jianning; Fei, Zhou; Wu, Jingwen; Fu, Luoan; Qu, Yan; Liu, Weiping; Wang, Zhanxiang; Yang, Lisun; He, Xiaosheng; Zhen, Haining; Gao, Dakuan; Cao, Weidong; Liang, Jingwen

2002-02-25

To study locating accuracy for the brain tumors and their peri-structures by the neuronavigator and elucidate the microsurgical effects. 65 patients with intracranial tumors were microsurgically treated by the application of Stealth Station and Vector Vision system. The treatment effects were summarized and the neuronavigational accuracy was discussed. After mean fiducial error (MFE) and sustained accuracy (SA) were satisfied. Total tumor removal was achieved in 63 cases (97.0%), subtotal removal in 2 cases (3.0%). The neurological functions were improved in 56 cases (86.2%), unchanged obviously in 9 cases (13.8%). No case deteriorated and died in the group. Navigation systems are reliable and accurate in making microneurosurgical plans for brain tumors. And they can provide tracing of the tumor in the operation and guide the operator's manipulation. The techniques, which help total removal of the tumors and reduce the postoperative complications, are very useful in guarantee operation effects.

Three-Dimensional Printing as an Interdisciplinary Communication Tool: Preparing for Removal of a Giant Renal Tumor and Atrium Neoplastic Mass.

Science.gov (United States)

Golab, Adam; Slojewski, Marcin; Brykczynski, Miroslaw; Lukowiak, Magdalena; Boehlke, Marek; Matias, Daniel; Smektala, Tomasz

2016-08-22

Three-dimensional (3D) printing involves preparing 3D objects from a digital model. These models can be used to plan and practice surgery. We used 3D printing to plan for a rare complicated surgery involving the removal of a renal tumor and neoplastic mass, which reached the heart atrium. A printed kidney model was an essential element of communication for physicians with different specializations.

Usefulness of [18F]FDG-PET in diagnosis of 18 tumors unapproved in health insurance. Study with multi-center survey by questionnaire

International Nuclear Information System (INIS)

Torizuka, Kanji; Ito, Kengo

2008-01-01

Usefulness of [ 18 F]fluorodeoxyglucose positron emission tomography (FDG-PET) diagnosis of the title tumors is practically realized and their approval in the health insurance might be awaited. The actual state of the diagnosis to confirm its usefulness was studied by questionnaire to facilities, where PET had been conducted for those tumors in the period July, 2005-February, 2006. Major questions concerned the purpose and finding of PET, findings by other imaging means and by tumor markers, and judgment of PET effectiveness compared with other imaging (more useful, equally or less, and its reason). In 30 facilities that gave answers, subjects were 133 cases (3-86 years old) in 18 diseases, which involved 3 cases of neuroblastoma, 13 of pheochromocytoma, 2 of carcinoid, 12 malignant pleural mesothelioma, 2 of malignant peritoneal mesothelioma, 13 of renal cell carcinoma, 2 of ureteral cancer, 4 of bladder cancer, 1 of Wilms' tumor, 24 of prostate cancer, 16 of testis tumor, 17 of mediastinal tumor, 5 of adrenal tumor, 5 of cutaneous tumor, 5 of extra-mammary Paget's disease, 7 of multiple myeloma, 1 of malignant fibrous histiocytoma and 1 of splenic hemangioma. Obtained were the judgments of highly useful in 10 diseases, fairly useful in 5, and useful in 3. Urological and cutaneous cancers above were subjected ones to their diagnosis of recurrence or metastasis postoperation, having given highly useful results, and thus FDG-PET was thought to be also highly useful in the postoperative follow-up. (R.T.)

Teresa Wilms Montt: la visceralidad como activismo

Directory of Open Access Journals (Sweden)

Cecilia Macon

2017-07-01

Full Text Available Es usual señalar que el debate sobre las emociones solo pasó a formar parte del feminismo en los últimos años. Sin embargo, es notoria la referencia en la literatura feminista de la primera ola al espacio de lo íntimo en términos de una colisión de emociones profundamente política que puede ser definida en términos de “visceralidad”. El presente trabajo se enmarca en un proyecto que tiene como objetivo final señalar el modo en que la transmisión de los afectos formó parte fundamental de la constitución del feminismo en América Latina. Este artículo se ocupa analizar la producción de la escritora chilena Teresa Wilms Montt, muy particularmente su primera obra, “Inquietudes sentimentales” (1917. No se trata meramente de argumentar la presencia de la dimensión emocional, sino de indagar en la especificidad de ese momento de su escritura donde la categoría de ‘intimidad” se torna central a la hora de establecer principios feministas. Son las características de su recorrido -donde entran en colisión las emociones más diversas - y el vínculo que establece con las luchas políticas del feminismo de corte anarquista las que abren la posibilidad de entender esta etapa, dando cuenta de la constitución de una geografía afectiva para el activismo latinoamericano. Una geografía marcada, centralmente por el desafío a “estructuras del sentir” patriarcales a través de intervenciones que sacan a la luz el papel politico de la visceralidad en tanto acción.

A Novel Technique for Endovascular Removal of Large Volume Right Atrial Tumor Thrombus

Energy Technology Data Exchange (ETDEWEB)

Nickel, Barbara, E-mail: nickel.ba@gmail.com [US Teleradiology and Quantum Medical Radiology Group (United States); McClure, Timothy, E-mail: tmcclure@gmail.com; Moriarty, John, E-mail: jmoriarty@mednet.ucla.edu [UCLA Medical Center, Department of Interventional Radiology (United States)

2015-08-15

Venous thromboembolic disease is a significant cause of morbidity and mortality, particularly in the setting of large volume pulmonary embolism. Thrombolytic therapy has been shown to be a successful treatment modality; however, its use somewhat limited due to the risk of hemorrhage and potential for distal embolization in the setting of large mobile thrombi. In patients where either thrombolysis is contraindicated or unsuccessful, and conventional therapies prove inadequate, surgical thrombectomy may be considered. We present a case of percutaneous endovascular extraction of a large mobile mass extending from the inferior vena cava into the right atrium using the Angiovac device, a venovenous bypass system designed for high-volume aspiration of undesired endovascular material. Standard endovascular methods for removal of cancer-associated thrombus, such as catheter-directed lysis, maceration, and exclusion, may prove inadequate in the setting of underlying tumor thrombus. Where conventional endovascular methods either fail or are unsuitable, endovascular thrombectomy with the Angiovac device may be a useful and safe minimally invasive alternative to open resection.

A Novel Technique for Endovascular Removal of Large Volume Right Atrial Tumor Thrombus

International Nuclear Information System (INIS)

Nickel, Barbara; McClure, Timothy; Moriarty, John

2015-01-01

Venous thromboembolic disease is a significant cause of morbidity and mortality, particularly in the setting of large volume pulmonary embolism. Thrombolytic therapy has been shown to be a successful treatment modality; however, its use somewhat limited due to the risk of hemorrhage and potential for distal embolization in the setting of large mobile thrombi. In patients where either thrombolysis is contraindicated or unsuccessful, and conventional therapies prove inadequate, surgical thrombectomy may be considered. We present a case of percutaneous endovascular extraction of a large mobile mass extending from the inferior vena cava into the right atrium using the Angiovac device, a venovenous bypass system designed for high-volume aspiration of undesired endovascular material. Standard endovascular methods for removal of cancer-associated thrombus, such as catheter-directed lysis, maceration, and exclusion, may prove inadequate in the setting of underlying tumor thrombus. Where conventional endovascular methods either fail or are unsuitable, endovascular thrombectomy with the Angiovac device may be a useful and safe minimally invasive alternative to open resection

Neurosurgical treatment of extracerebral tumors

International Nuclear Information System (INIS)

Antoniadis, G.; Kretschmer, T.; Braun, V.; Rath, S.; Richter, H.P.

1998-01-01

Extracerebral tumors represent 29-30% of the intracranial tumors. Meningiomas rank first in incidence with 13-19%, followed by neurinomas and pituitary adenomas, 6-8% each. Due to their slow growth rate, meningiomas in the initial stages do not produce symptoms. Complete recovery can be achieved by complete removal of the tumor, which in general is possible in case of tumor sites at the convexity. Meningiomas at the basis, or meningiomas spreading into the sinus sagittalis superior often cannot be totally removed, so that for treatment of remaining or recurrent tumors and anaplastic meningiomas, post-operative radiation therapy is recommended. Neurinomas of the nervus acusticus originate at the N. vestibularis and may spread from the Meatus acusticus internus into the brain stem. With the modern methods of microsurgery combined with intraoperative monitoring it is possible to preserve the Nn. facialis and cochlearis and their functions. As an alternative to surgery, radiosurgery techniques have been on trial in the last few years. Via transsphenoidal approach, intra- and suprasellar pituitary adenomas can be completely destroyed. Parasellar tumors can be resected by transcranial approach only. Remaining tumors in the Sinus cavernosus, or recurrent adenomas, should receive post-surgery radiation treatment. (orig./CB) [de

Hepatopathy-thrombocytopenia syndrome after actinomycin-D therapy: treatment with defibrotide.

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Martín-Lázaro, Juan F; Palanca, Daniel; Garcia-Iñiguez, Juan P; Madurga, Paula; Carboné, Ana

2013-02-01

We report a case of administration compassionate use defibrotide in a 13-year-old girl with Sinusoidal Obstructive Syndrome and thrombocytopenia, also known as Hepatopathy--Thrombocytopenia Syndrome (HTS) during chemotherapy for Wilms' tumor.

Size-amplified acoustofluidic separation of circulating tumor cells with removable microbeads

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Liu, Huiqin; Ao, Zheng; Cai, Bo; Shu, Xi; Chen, Keke; Rao, Lang; Luo, Changliang; Wang, Fu-Bin; Liu, Wei; Bondesson, Maria; Guo, Shishang; Guo, Feng

2018-06-01

Isolation and analysis of rare circulating tumor cells (CTCs) is of great interest in cancer diagnosis, prognosis, and treatment efficacy evaluation. Acoustofluidic cell separation becomes an attractive method due to its contactless, noninvasive, simple, and versatile features. However, the indistinctive physical difference between CTCs and normal blood cells limits the purity of CTCs using current acoustic methods. Herein, we demonstrate a size-amplified acoustic separation and release of CTCs with removable microbeads. CTCs selectively bound to size-amplifiers (40 μm-diameter anti-EpCAM/gelatin-coated SiO2 microbeads) have significant physical differences (size and mechanics) compared to normal blood cells, resulting in an amplification of acoustic radiation force approximately a hundredfold over that of bare CTCs or normal blood cells. Therefore, CTCs can be efficiently sorted out with size-amplifiers in a traveling surface acoustic wave microfluidic device and released from size-amplifiers by enzymatic degradation for further purification or downstream analysis. We demonstrate a cell separation from blood samples with a total efficiency (E total) of ∼ 77%, purity (P) of ∼ 96%, and viability (V) of ∼83% after releasing cells from size-amplifiers. Our method substantially improves the emerging application of rare cell purification for translational medicine.

Kidney Cancer

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... kind of kidney cancer called Wilms' tumor. The incidence of kidney cancer seems to be increasing. One ... doesn't go away Loss of appetite Unexplained weight loss Tiredness Fever, which usually comes and goes ( ...

Reference radiology in nephroblastoma: accuracy and relevance for preoperative chemotherapy

International Nuclear Information System (INIS)

Schenk, J.P.; Schrader, C.; Zieger, B.; Ley, S.; Troeger, J.; Furtwaengler, R.; Graf, N.; Leuschner, I.

2006-01-01

Purpose: A reference radiologic diagnosis was carried out for the purpose of quality control and in order to achieve high diagnostic accuracy in the ongoing trial and study SIOP 2001/GPOH for renal tumors during childhood. The aim of the present study is to evaluate the value of diagnostic imaging and the benefit of reference evaluation at a pediatric radiology center. Materials and Methods: In 2004 the imaging studies of 97 patients suspected of having a renal tumor were presented at the beginning of therapy. Diagnostic imaging was compared to the primary imaging results and the histological findings and was analyzed in regard to the therapeutic consequence (primary chemotherapy without prior histology). 77 MRI, 35 CT and 67 ultrasound examinations of 47 girls and 50 boys (mean age 4 years; one day to 15.87 years old) were analyzed. In addition to the histological findings, the reference pathological results were submitted in 86 cases. Results from the primary imaging corresponding to the histology and results from the reference radiology corresponding to the histology and results from the reference radiology corresponding to the histology were statistically compared in a binomial test. Results: In 76 of the reference-diagnosed Wilms' tumors, 67 were confirmed histologically. In 72 cases preoperative chemotherapy was initiated. In 5 cases neither a Wilms' tumor nor a nephroblastomatosis was found. 16 of 21 cases (76%) with reference-diagnosed non-Wilms' tumors were selected correctly. The results of the primary imaging corresponded to the histology in 71 cases, and those of the reference radiology in 82 cases. The statistical evaluation showed that the results of the reference radiology were significantly better (p=0.03971). (orig.)

Malignant tumors during the first 2 decades of life in the offspring of atomic bomb survivors

International Nuclear Information System (INIS)

Yoshimoto, Y.; Neel, J.V.; Schull, W.J.; Kato, H.; Soda, M.; Eto, R.; Mabuchi, K.

1990-01-01

The risk of cancer (incidence) prior to age 20 years has been determined for children born to atomic bomb survivors and to a suitable comparison group. Tumor ascertainment was through death certificates and the tumor registries maintained in Hiroshima and Nagasaki. The rationale for the study stemmed from the evidence that a significant proportion of such childhood tumors as retinoblastoma and Wilms tumor arise on the basis of a mutant gene inherited from one parent plus a second somatic cell mutation involving the allele of this gene. Gonadal radiation doses were calculated by the recently established DS86 system, supplemented by an ad hoc system for those children for one or both of whose parents a DS86 dose could not be computed but for whom an ad hoc dose could be developed on the basis of the available information. The total data set consisted of (1) a cohort of 31,150 live-born children one or both of whose parents received greater than 0.01 Sv of radiation at the time of the atomic bombings (average conjoint gonad exposure 0.43 Sv) and (2) two suitable comparison groups totaling 41,066 children. Altogether, 43 malignant tumors were ascertained in the children of exposed parents, and 49 malignant tumors were ascertained in the two control groups. A multiple linear regression analysis revealed no increase in malignancy in the children of exposed parents. However, examination of the data suggested that only 3.0-5.0% of the tumors of childhood that were observed in the comparison groups are associated with an inherited genetic predisposition that would be expected to exhibit an altered frequency if the parental mutation rate were increased. There is thus far no confirmation of the positive findings that Nomura found in a mouse system

Hepatic Sinusoidal Obstruction Syndrome During Chemotherapy for Childhood Medulloblastoma: Report of a Case and Review of the Literature

Science.gov (United States)

Buckland, Amy; Phillips, Marianne B.; Cole, Catherine H.; Gottardo, Nicholas G.

2014-01-01

Hepatic sinusoidal obstruction syndrome (HSOS), also known as veno-occlusive disease, is a well-recognized toxic complication after autologous and allogeneic hematopoietic stem cell transplant, during treatment of Wilms tumor and rhabdomyosarcoma associated with actinomycin-D, and during acute lymphoblastic leukemia therapy due to oral 6-thioguanine. However, its occurrence in the context of chemotherapy regimens for other childhood malignancies is rare. We report a 5-year-old girl with high-risk anaplastic medulloblastoma, who developed severe HSOS during her second cycle of maintenance chemotherapy, consisting of vincristine, cisplatin, and cyclophosphamide. She was treated with defibrotide with complete resolution of the HSOS. These findings and a review of the literature, highlight the occurrence of HSOS in children outside the established settings of hematopoietic stem cell transplantation, Wilms tumor, rhabdomyosarcoma, and acute lymphoblastic leukemia. PMID:24276042

Treatment Option Overview (Wilms Tumor and Other Childhood Kidney Tumors)

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... factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment options ... come back) after it has been treated. Treatment Option Overview Key Points There are different types of ...

General Information about Wilms Tumor and Other Childhood Kidney Tumors

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... through the urethra and leaves the body. Enlarge Anatomy of the female urinary system showing the kidneys, adrenal glands, ureters, bladder, and urethra. Urine is made in the renal tubules and collects in the renal pelvis of ...

High Concentration of Serum Soluble Fas in Patients with Head and Neck Carcinoma: A Comparative Study Before and After Surgical Removal of Tumor

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Seyed Basir Hashemi

2010-01-01

Full Text Available Background:Alternative splicing of the Fas transcript can produce a naturalsecreted isoform of this molecule. Some cancer cells can also produce soluble Fas (sFaswhich may have suppressive effects on the immune system's anti-tumor response.Elevated concentrations of sFas have been detected in the sera of patients with differentmalignancies. Materials and Methods:The concentrations of sFas in sera of patients with headand neck carcinoma (HNC, n=98 and healthy individuals (n=30 were measured bySandwich ELISAand compared to values obtained six months after surgical removalof the tumor (n=48. Data were correlated with different clinical findings of thepatients. Results:sFas concentrations in the sera of HNC patients were found to besignificantly higher in patients with different tumor stages. sFas concentration did notcorrelate with age or tumor invasiveness, however a higher concentration of sFas wasfound in the sera of patients who had higher tumor grades. Surgical removal oftumors in patients resulted in a substantial decrease in sFas concentration.Conclusion:The initial rise in sFas concentration in the sera of HNC patients andits consequent decrease could be regarded as a sign of tumor suppressive mechanisms.Additional studies are needed to fully elucidate this mechanism however these findingsmight show the prospective use of such biomarkers to determine disease prognosis andeven immunotherapeutic applications.

Malignant primary germ-cell tumor of the brain

International Nuclear Information System (INIS)

Yamamoto, Toyoshiro; Sato, Shinichi; Nakao, Satoshi; Ban, Sadahiko; Namba, Koh

1983-01-01

The unusual case of a 15 year old boy with three discrete paraventricular germ-cell tumors is reported.FThe first tumor was located just lateral to the left thalamus and included a massive cystic part around it, the second tumor in the paraventricular region above the head of the left caudate nucleus and the third tumor in the medial part of the left parietal lobe.FTotal removal of all tumors was successfully accomplished in stages at four separate operations, namely, the first tumor was removed through the left transsylvian approach, the second tumor via left superior frontal gyrus and the third tumor via left superior frontal gyrus and left superior parietal lobule.FHistological examination revealed that the first tumor was teratoma, the second was choriocarcinoma and the third was germinoma.FPrimary germ-cell tumors of the brain can be divided into 5 groups: 1) germinoma; 2) embryonal carcinoma; 3) choriocarcinoma; 4) yolk-sac tumor; or 5) teratoma.FIn this case, a combination of three different histological patterns was seen. If malignant germ-cell tumor is supected on CT, aggressive extirpation should be done, not only to determine the exact diagnosis, but also to provide the basis for subsequent adjunctive therapy. (author)

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)

Computed tomography on renal masses in dogs and cats

Energy Technology Data Exchange (ETDEWEB)

Yamazoe, Kazuaki (Gifu Univ. (Japan). Faculty of Agriculture); Ohashi, Fumihito; Kadosawa, Tsuyoshi; Nishimura, Ryohei; Sasaki, Nobuo; Takeuchi, Akira

1994-08-01

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).

Malignant Trigeminal Nerve Sheath Tumor and Anaplastic Astrocytoma Collision Tumor with High Proliferative Activity and Tumor Suppressor P53 Expression

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Maher Kurdi

2014-01-01

Full Text Available Background. The synchronous development of two primary brain tumors of distinct cell of origin in close proximity or in contact with each other is extremely rare. We present the first case of collision tumor with two histological distinct tumors. Case Presentation. A 54-year-old woman presented with progressive atypical left facial pain and numbness for 8 months. MRI of the brain showed left middle cranial fossa heterogeneous mass extending into the infratemporal fossa. At surgery, a distinct but intermingled intra- and extradural tumor was demonstrated which was completely removed through left orbitozygomatic-temporal craniotomy. Histopathological examination showed that the tumor had two distinct components: malignant nerve sheath tumor of the trigeminal nerve and temporal lobe anaplastic astrocytoma. Proliferative activity and expressed tumor protein 53 (TP53 gene mutations were demonstrated in both tumors. Conclusions. We describe the first case of malignant trigeminal nerve sheath tumor (MTNST and anaplastic astrocytoma in collision and discuss the possible hypothesis of this rare occurrence. We propose that MTNST, with TP53 mutation, have participated in the formation of anaplastic astrocytoma, or vice versa.

Orbital Tumors Excision without Bony Marginotomy under Local and General Anesthesia

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Robert A. Goldberg

2014-01-01

Full Text Available To present our experience of removing middle to deep orbital tumors using a combination of minimally invasive soft tissue approaches, sometimes under local anesthesia. Methods. In this retrospective case series, 30 patients (13 males and 17 females underwent tumor removal through eyelid crease (17 eyes, conjunctival (nine eyes, lateral canthal (two eyes, and transcaruncular (two eyes approaches. All tumors were located in the posterior half of the orbit. Six cases were removed under monitored anesthesia care with local block, and 24 were under general anesthesia. Results. The median (range age and follow-up duration were 48.5 (31–87 years old and 24.5 (4–375 weeks, respectively. Visual acuity and ocular motility showed improvement or no significant change in all but one patient at the latest followup. Confirmed pathologies revealed cavernous hemangioma (15 cases, pleomorphic adenoma (5 cases, solitary fibrous tumor (4 cases, neurofibroma (2 cases, schwannoma (2 cases, and orbital varix (1 case. None of the patients experienced recurrence. Conclusions. Creating a bony marginotomy increases intraoperative exposure of the deep orbit but adds substantial time and morbidity. Benign orbital tumors can often be removed safely through small soft-tissue incisions, without bone removal and under local anesthesia.

Intraoperative MRI to control the extent of brain tumor surgery

International Nuclear Information System (INIS)

Knauth, M.; Sartor, K.; Wirtz, C.R.; Tronnier, V.M.; Staubert, A.; Kunze, S.

1998-01-01

Intraoperative MRI definitely showed residual tumor in 6 of the 18 patients and resulted in ambiguous findings in 3 patients. In 7 patients surgery was continued. Early postoperative MRI showed residual tumor in 3 patients and resulted in uncertain findings in 2 patients. The rate of patients in whom complete removal of enhancing tumor could be achieved was 50% at the time of the intraoperative MR examination and 72% at the time of the early postoperative MR control. The difference in proportion of patients with 'complete tumor removal' between the groups who had been operated on using neuronavigation (NN) and intraoperative MRI (ioMRI) and those who had been operated on using only modern neurosurgical techniques except NN and ioMRI was statistically highly significant (Fisher exact test; P=0.008). Four different types of surgically induced contrast enhancement were observed. These phenomena carry different confounding potentials with residual tumor. Conclusion: Our preliminary experience with intraoperative MRI in patients with enhancing intraaxial tumors is encouraging. Combined use of neuronavigation and intraoperative MRI was able to increase the proportion of patients in whom complete removal of the enhancing parts of the tumor was achieved. Surgically induced enhancement requires careful analysis of the intraoperative MRI in order not to confuse it with residual tumor. (orig.) [de

Tumor thrombus formation in two dogs with insulinomas.

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Hambrook, Lydia E; Kudnig, Simon T

2012-10-15

A 9-year-old sexually intact male Staffordshire Bull Terrier and a 9-year-old neutered male Boxer were evaluated for intermittent neurologic signs including muscle tremors, ataxia, episodic collapse, disorientation, and seizures. Both dogs had low blood glucose and high serum insulin concentrations. Results of abdominal ultrasonography were unremarkable for both dogs. Exploratory laparotomy revealed a mass that extended from the body of the pancreas into the pancreaticoduodenal vein in each dog. Marginal resection of pancreatic masses was performed, and tumor thrombi were removed via venotomy in both dogs. Histologic evaluation indicated the masses were pancreatic islet cell tumors with tumor thrombi. Clinical signs resolved following surgical resection of tumors and tumor thrombi, and the dogs were euglycemic during the follow-up period (17 and 45 months after surgery). Although gross tumor thrombus formation has been identified in humans with insulinomas, tumor thrombi have not been previously reported for dogs with insulinomas. Surgical removal of tumor thrombi via venotomy seemed to be well tolerated by the dogs. Tumor thrombus formation did not seem to adversely affect prognosis for the 2 dogs of this report.

Chlamydia trachomatis induces an upregulation of molecular biomarkers podoplanin, Wilms' tumour gene 1, osteopontin and inflammatory cytokines in human mesothelial cells.

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De Filippis, Anna; Buommino, Elisabetta; Domenico, Marina Di; Feola, Antonia; Brunetti-Pierri, Raffaella; Rizzo, Antonietta

2017-05-01

Chlamydia trachomatis is the most prevalent infection of the genital tract in women worldwide. C. trachomatis has a tendency to cause persistent infection and induce a state of chronic inflammation, which has been reported to play a role in carcinogenesis. We report that persistent C. trachomatis infection increases the expression of inflammatory tumour cytokines and upregulates molecular biomarkers such as podoplanin, Wilms' tumour gene 1 and osteopontin in primary cultures of mesothelial cells (Mes1) and human mesothelioma cells (NCI). Infection experiments showed that Mes1 and NCI supported the growth of C. trachomatisin vitro, and at an m.o.i. of 4, the inclusion-forming units/cell showed many intracellular inclusion bodies after 3 days of infection. However, after 7 days of incubation, increased proliferative and invasive activity was also observed in Mes1 cells, which was more evident after 14 days of incubation. ELISA analysis revealed an increase in vascular endothelial growth factor, IL-6, IL-8, and TNF-α release in Mes1 cells infected for a longer period (14 days). Finally, real-time PCR analysis revealed a strong induction of podoplanin, Wilms' tumour gene 1 and osteopontin gene expression in infected Mes1 cells. The aim of the present study was to investigate the inflammatory response elicited by C. trachomatis persistent infection and the role played by inflammation in cell proliferation, secretion of proinflammatory cytokines and molecular biomarkers of cancer. The results of this study suggest that increased molecular biomarkers of cancer by persistent inflammation from C. trachomatis infection might support cellular transformation, thus increasing the risk of cancer.

Organ-Preserving Surgical Treatment of a Horseshoe Kidney Occupied by a Large Renal Cell Carcinoma with Extensive Venous Invasion: A Case Report.

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Linxweiler, Johannes; Shayesteh-Kheslat, Roushanak; Fries, Peter; Schneider, Günther; Janssen, Martin; Ohlmann, Carsten H; Stöckle, Michael; Siemer, Stefan; Saar, Matthias

2018-01-01

The horseshoe kidney is one of the most common congenital disorders affecting the urogenital system. Following a fusion of the lower kidney poles, which in turn lead to the formation of an isthmus, this anatomical variation is accompanied by other characteristic properties like an incomplete ascension, ventral rotation of the pelvices as well as atypical vascular supply. Even though renal carcinoids and Wilms tumors are more common in horseshoe kidneys, the incidence of renal cell carcinomas seems to be unaffected. Here we report the case of a locally advanced renal cell carcinoma with extensive venous invasion occurring in a horseshoe kidney and its complex surgical management. The whole primary tumor as well as a majority of venous tumor thrombi could be removed by a combination of 2/3 nephrectomy and cavotomy with thrombectomy. During 1 year of follow-up, the patient neither suffered from a tumor relapse, nor did he require renal replacement therapy. Thus, we conclude that even in cases of RCC where advanced disease is associated with complex anatomical situations, organ-preserving surgical treatment should be pursued to achieve excellent functional and oncological results. © 2016 S. Karger AG, Basel.

Preoperative trans-arterial embolization for spinal tumor: embolization techniques and results

International Nuclear Information System (INIS)

Shi Haibin; Xu Daizhe

2000-01-01

Objective: To analyze the technique and to evaluate the safety and value of preoperative trans-arterial embolization of hypervascular spinal tumors. Methods: Eighteen patients with hypervascular spinal tumors underwent trans-arterial embolization before surgery. They arose intradural in six patients and extradural in 12. Thirty-one arteries were embolized with polyvinyl alcohol (PVA) particles (150-500 μm), of which 18 with additional pieces of gelatin sponge for proximal pedicular embolization. The criteria for judging the effectiveness of embolization were the completeness of tumor removal and estimated blood loss during surgery. Results: Tumor embolization with total occlusion was obtained in eight patients, second to total in seven, subtotal in one, and partial in two. There were no symptomatic complications associated with embolization. Tumors were totally removed in 17 patients and nearly totally removed in one. The average estimated blood loss during surgery was 1100 ml (range, 200-6000 ml) for all 18 patients, and 1540 ml in patients with extradural tumors. Conclusions: Preoperative embolization of hypervascular spinal tumors is safe and effective. It can make complete resection of a tumor possible and can make an unresectable tumor resectable. Superselection or flow control is necessary to achieve effective devascularization and avoid complications

Effects on pulmonary function of whole lung irradiation for Wilm's tumour in children

International Nuclear Information System (INIS)

Benoist, M.R.; Lemerle, J.; Jean, R.; Rufin, P.; Scheinmann, P.; Paupe, J.

1982-01-01

The effect of whole lung irradiation on lung function was investigated in 48 children treated for Wilm's tumour with pulmonary metastases. Lung function tests were performed before irradiation and were repeated annually for as long as possible, the length of follow-up varying from two to 17 years. A reduction in both lung volume and in dynamic compliance was clearly observed. In some patients these changes occurred in the early post-irradiation months, but in most the decrease observed progressed over longer periods of time. Static pressure volume curves, bloodgases, and carbon monoxide transfer were normal. These findings make it unlikely that post-irradiation pulmonary fibrosis was involved. Another explanation for the decreased lung volume and dynamic compliance might be failure of alveolar multiplication. Muscular injury is unlikely as the patients were able to produce normal transthoracic pressures. A failure of chest wall growth is also possible and would explain the progressive restrictive impairment but not the early lung function changes. It is suggested that the early effects detected in some patients were the result of lung injury and that later effects resulted from impaired chest wall growth. (author)

Renal function after unilateral nephrectomy for Wilms' tumour: the influence of radiation therapy

International Nuclear Information System (INIS)

Graaf, S.S.N. de; Gent, H. van; Reitsma-Bierens, W.Ch.C.; Luyk, W.H.J. van; Postma, A.; Dolsma, W.V.

1996-01-01

The effect of therapy on renal function after unilateral nephrectomy for Wilms' tumour was studied. In the second year following unilateral nephrectomy, glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) were estimated simultaneously by measuring 125 I-iothalamate clearance and 131 I-hippurate clearance. Of 41 evaluable patients, 29 received chemotherapy as sole treatment modality following nephrectomy (group 1); 12 patients additionally received radiation therapy to a field that included the remaining kidney (group 2). Results were expressed as standard deviation scores (z-scores). In group 1, mean z-score for GFR was -0.27 (94.6% of normal) and in group 2 mean z-score was -1.51 (72.7% of normal for two kidneys) (P = 0.022, Mann-Whitney U-test). Mean z-score for ERPF was -0.09 (97.0%) in group 1 and -1.53 (73.8%) in group 2 (P 0.039). It was concluded that the combination of chemotherapy and radiation therapy, in contrast to chemotherapy alone, negatively affects the ability of the remaining kidney to adjust its function after the loss of its counterpart. (author)

Carotid Body Tumors: A Review of 25 Years Experience in Diagnosis and Management of 56 Tumors

Science.gov (United States)

Sayed, Ahmed; Elwan, Hussein; Fouad, Fouad M.S.; Kamal Eldin, Hussein; Khairy, Hussein; Elhindawy, Khaled; Taha, Ahmed; Hefnawy, Engie

2014-01-01

Objective: To report our experience in the management of CBTs and review the literature. Materials and Methods: 56 CBTs were operated upon over a period of 25 years. Surgical intervention was planned according to the Shamblin classification. Thirty-nine of the tumors were in males (69.64%), and 17 of the tumors were in females (30.36%). The average age was 42 (ages ranging between 32 and 47). Twenty-two tumors were diagnosed and treated with Shamblin type I, twenty-eight with type II and eight tumors with type III. All patients were unilateral except two had bilateral carotid tumors. Thirty-five lumps were de novo (group A), while 21 lumps were treated after a prior trial of removal (group B). Results: The incidence of carotid reconstruction was lower among group A (1/35) compared to group B (9/21). Complications were less in group A than group B (23% vs. 30%). There were 4 cases with suspected malignancy and no recurrences during the follow-up period. Conclusion: There is an increased incidence of major vascular reconstruction in cases not properly investigated or diagnosed and in cases with prior attempts of removal in Primary Hospitals. Proper diagnosis of suspicious lumps is mandatory. Resection of CBTs by surgeons with experience in vascular reconstruction is recommended. Vascular reconstructions have to be performed safely without serious complications. Also today, prior attempts of removal are not so common, with preoperative evaluation using the latest diagnostic tools. Cranial nerves injury (especially the Hypoglossal Nv) continues to be the most common complication. PMID:25298832

One-stage surgery for removal of intravascular leiomyomatosis extending to right ventricle.

Science.gov (United States)

Chiang, Ching-Shu; Chen, Po-Lin; Kuo, Tzu-Ting; Chen, I-Ming; Wu, Nai-Yuan; Chang, Hsiao-Huang

2018-03-01

Intravascular leiomyomatosis (IVL) is a rare nonmalignant tumor that can be fatal if untreated. A 49-year-old nulliparous Asian woman who underwent hysterectomy and left salpingo-oophorectomy for multiple uterine leiomyomas 18 months prior presented complaining of intermittent palpitation and chest tightness for approximately 1 month. Echocardiography revealed a large mobile tumor mass extending from the inferior vena cava (IVC) to the right atrium that partially obstructed IVC flow and tricuspid inflow. Thoracicabdominopelvic computed tomography revealed a left adnexal tumor (4.8 × 2.5 cm) causing intravascular obstruction extending from the left internal iliac vein to the IVC, right atrium, and right ventricle. IVL with right heart involvement INTERVENTIONS:: Under cardiopulmonary bypass, a one-stage surgery combining sternotomy and laparotomy was performed. The tumor was approached and extracted via sternotomy, and tumor detachment and removal of residual tumors was accomplished via laparotomy. A firm, smooth, and regularly shape tumor 15.5 × 5.5 × 2.5 in size was completely removed and histopathologically confirmed as IVL. The patient tolerated the surgical procedure well and no postoperative complication was noted. We describe a one-stage surgical approach to completely remove an IVL extending to the right ventricle.

Peculiarities of the Perioperative Care of Elderly Patients Undergoing Endoscopic Transsphenoidal Removal of Tumors in the Chiasmatic-Sellar Area

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Alexey B. Kurnosov

2017-01-01

Full Text Available Nowadays, in the age of rapid introduction of digital and fiber-optic technologies in neurosurgery, a technique for removal of tumors in the chiasmatic-sellar area via an endoscopic transsphenoidal endonasal approach is actively developed; this technique is considered less invasive and is well tolerated by patients, thus permitting to operate patients with somatic complications, as well as the elderly. Taking into account these facts, as well as world statistic data indicating a continuous trend of population aging in developed countries, including Russia, optimization of the perioperative care of elderly patients with a tumor of the chiasmatic-sellar area becomes the problem of highest priority. In order to solve it, this review discusses the basic peculiarities of the perioperative management of elderly patients (characteristics of their somatic status and possible variants of the pre-operational state with a pathology in the chiasmatic-sellar region; it also presents the modern and most acceptable alternative solutions of this difficult problem (introduction of modern methods of anesthesia, management of the postoperative cognitive dysfunction, postoperative pain syndrome, and postoperative nausea and vomiting. 

Perlman syndrome : Four additional cases and review

NARCIS (Netherlands)

Henneveld, HT; van Lingen, RA; Hamel, BCJ; Stolte-Dijkstra, [No Value; van Essen, AJ

1999-01-01

Perlman syndrome was first described in 1973 and comprises nephromegaly with renal dysplasia and Wilms tumor, macrosomia, cryptorchidism, and multiple facial anomalies. Polyhydramnios and hypoglycaemia are often found. Twelve children have been described from six different families. Five came from

Desmoid tumor within lesser sac

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Čolović Radoje

2006-01-01

Full Text Available Desmoid tumors or fibromatoses comprise a number of benign fibrous proliferative lesions that have local infiltrative growth and tendency to recur after incomplete excision. They never metastasize. The authors present a 31-year old woman who, due to epigastric pain and palpable mass detected on presentation, underwent the excision of firm tumorous mass, 210x140x115mm in diameter, from the lesser sac. Compressing the splenic vein, the tumor caused left-sided portal hypertension which subsided after the mass was removed. The recovery was uneventful. The histological examination verified typical desmoid tumor. Twelve years after surgery, the patient remained symptom-free with no signs of recurrence.

Endocrine Tumors Causing Arterial Hypertension: Pathophysiological Mechanisms and Clinical Implications.

Science.gov (United States)

Buonacera, Agata; Stancanelli, Benedetta; Malatino, Lorenzo

2017-09-01

Some tumors are a relatively rare and amendable cause of hypertension, often associated with a higher cardiovascular morbidity and mortality, as compared with that of both general population and patients with essential hypertension. This worse prognosis is not entirely related to blood pressure increase, because the release of substances from the tumor can directly influence blood pressure behavior. Diagnostic approach is challenging and needs a deep knowledge of the different neuro-hormonal and genetic mechanisms determining blood pressure increase. Surgical tumor removal can, but not always, cause blood pressure normalization, depending on how early was tumor detection, since a long-standing history of hypertension is often associated with a much weaker effect on blood pressure. Moreover, target organ damage can be affected by the substances themselves released by the tumors as well as by tumor removal. In this review we consider the phenotype and genetic features of patients with tumor-induced hypertension and focus on their diagnostic work-up.

Malignant primary germ-cell tumor of the brain: case report

Energy Technology Data Exchange (ETDEWEB)

Yamamoto, Toyoshiro; Sato, Shinichi; Nakao, Satoshi; Ban, Sadahiko; Namba, Koh (Kobe Municipal Central Hospital (Japan))

1983-04-01

The unusual case of a 15 year old boy with three discrete paraventricular germ-cell tumors is reported. The first tumor was located just lateral to the left thalamus and included a massive cystic part around it, the second tumor in the paraventricular region above the head of the left caudate nucleus and the third tumor in the medial part of the left parietal lobe. Total removal of all tumors was successfully accomplished in stages at four separate operations, namely, the first tumor was removed through the left transsylvian approach, the second tumor via left superior frontal gyrus and the third tumor via left superior frontal gyrus and left superior parietal lobule. Histological examination revealed that the first tumor was teratoma, the second was choriocarcinoma and the third was germinoma. Primary germ-cell tumors of the brain can be divided into 5 groups: 1) germinoma; 2) embryonal carcinoma; 3) choriocarcinoma; 4) yolk-sac tumor; or 5) teratoma. In this case, a combination of three different histological patterns was seen. If malignant germ-cell tumor is supected on CT, aggressive extirpation should be done, not only to determine the exact diagnosis, but also to provide the basis for subsequent adjunctive therapy.

Mutation of the PAX6 gene in a sporadic patient with atypical aniridia

Energy Technology Data Exchange (ETDEWEB)

Zhu, D.; Li, Y.; Traboulsi, E.I. [Wilmer Eye Institute, Baltimore, MD (United States)] [and others

1994-09-01

A 28 year-old man presented with poor vision since childhood and gradual further decline of several years duration. His visual acuity measures 20/200 OD with -11.50 + 0.50 x 150 and 20/100 OS with -12.25 + 0.25 x 35. He had a fine nystagmus. His visual fields were full. There was a circumferential pannus with areas of corneal stromal opacification. The iris was hypoplastic with atypical colobomatous defects. The lenses had scattered cortical opacities. The intraocular pressures were normal. The optic nerves had cup disk ratios of 0.6 OU. The family history was negative for similar defects. A diagnosis of aniridia was made and blood was drawn for analysis of the PAX6 gene. PCR amplification of exon 5 showed heterozygous fragments with one allele being larger than normal. Direct DNA sequencing of the individual heterozygous allele showed a 41 base pair insertion at nucleotide 483 in exon 5 of the paired domain. This frameshift mutation changed codon 71 to a stop codon. The diagnosis of aniridia was confirmed in this atypical patient, who will need to be monitored for his high risk of glaucoma. The risk of developing Wilms` tumor in patients with mutations within the aniridia gene is presumably negligible since the neighboring Wilms` tumor gene is unaffected. The identification of intragenic mutations of the PAX6 gene in patients with sporadic aniridia modifies the management of such patients because of recognition of the increased risk of glaucoma and by reducing the necessity for frequent monitoring for the presence of Wilms` tumor.

Kidney (Renal Cell) Cancer—Patient Version

Science.gov (United States)

Kidney cancer can develop in adults and children. The main types of kidney cancer are renal cell cancer, transitional cell cancer, and Wilms tumor. Certain inherited conditions increase the risk of kidney cancer. Start here to find information on kidney cancer treatment, research, and statistics.

Abnormal contrast enhancement after the removal of meningioma

Energy Technology Data Exchange (ETDEWEB)

Soutsu, M; Hashimoto, T; Yamaguchi, Y; Sekino, H; Nakamura, N [Jikei Univ., Tokyo (Japan). School of Medicine

1980-12-01

Since the introduction of Computerized Tomography (CT) in August, 1976, we have had experience with 20 patients with meningioma. In 10 patients out of the 20, the tumors were removed totally and examined by contrast-enhanced CT pre- and postoperatively. In spite of the total removal of the tumor, however, 5 patients showed an unexpected abnormal enhancement at the operated site after the infusion of a contrast material. These changes were relatively intense, so it was suspected that there were residual tumors. Comparing these 5 cases with the remaining 5 that had no postoperative enhancement, the former cases were shown by operative and angiographical findings to have more adhesion and connecting vessels between tumor and cortex. Since such a postoperative abnormal enhancement is seen along the corticotomy or retracted area after operations for and aneurysm or another lesion, damaged brain tissue was though to be the probable reason for this enhancement. Moreover, the circulatory change caused by cutting the connecting vessels between tumor and cortex was considered to be another reason for the enhancement. This mechanism was also considered to be one reason for the post-meningioma syndrome by El-Banhawy. These postoperative enhancements decreased in density and became obscure within 4 or 8 weeks. Any reappearance of such changes after that period should be examined, carefully to determine whether or not it marks a recurrent tumor.

Clinical impact of anatomo-functional evaluation of brain function during brain tumor surgery

International Nuclear Information System (INIS)

Mikuni, Nobuhiro; Kikuchi, Takayuki; Matsumoto, Atsushi; Yokoyama, Yohei; Takahashi, Jun; Hashimoto, Nobuo

2009-01-01

To attempt to improve surgical outcome of brain surgery, clinical significance of anatomo-functional evaluation of brain function during resection of brain tumors was assessed. Seventy four patients with glioma located near eloquent areas underwent surgery while awake. Intraoperative tractography-integrated functional neuronavigation and cortical/subcortical electrical stimulation were correlated with clinical symptoms during and after resection of tumors. Cortical functional areas were safely removed with negative electric stimulation and eloquent cortices could be removed in some circumstances. Subcortical functional mapping was difficult except for motor function. Studying cortical functional compensation allows more extensive removal of brain tumors located in the eloquent areas. (author)

Surgery of resectable nonfunctioning neuroendocrine pancreatic tumors.

Science.gov (United States)

Dralle, Henning; Krohn, Sabine L; Karges, Wolfram; Boehm, Bernhard O; Brauckhoff, Michael; Gimm, Oliver

2004-12-01

Nonfunctioning neuroendocrine pancreatic tumors (NFNEPTs) comprise about one-third of pancreatic endocrine tumors. Based on immunohistochemistry, nonfunctioning tumors are difficult to distinguish from functioning ones; therefore the final diagnosis is basically the result of a synopsis of pathology and clinical data. Owing to their incapacity to produce hormone-dependent symptoms, NFNEPTs are detected incidentally or because of uncharacteristic symptoms resulting from local or distant growth. About two-thirds of NFNEPTs are located in the pancreatic head, so jaundice may be a late symptom of this tumor. Modern diagnostic procedures are best applied by a stepwise approach: first endoscopic ultrasonography and computed tomography/magnetic resonance imaging followed by somatostatin receptor scintigraphy or positron emission tomography (or both). Due to significant false-positive and false-negative findings, for decision-making the latter should be confirmed by a second imaging modality. Regarding indications for surgery and the surgical approach to the pancreas, three pancreatic manifestations of NFNEPTs can be distinguished: (1) solitary benign non-multiple endocrine neoplasia type 1 (non-MEN-1); (2) multiple benign MEN-1; and (3) malignant NFNEPTs. Reviewing the literature and including our experience with 18 NFNEPTs (8 benign, 10 malignant) reported here, the following conclusions can be drawn: (1) Solitary benign non-MEN-1 NFNEPTs can be removed by enucleation or by pancreas-, spleen-, and duodenum-preserving techniques in most cases. The choice of surgical technique depends on the location and site of the tumor and its anatomic relation to the pancreatic duct. (2) With multiple benign MEN-1 NFNEPTs, because of the characteristics of the underlying disease a preferred, more conservative concept (removal of only macrolesions) competes with a more radical procedure (left pancreatic resection with enucleation of head macrolesions). Further studies are necessary to

A flexible endoscope-assisted interhemispheric transcallosal approach through the contralateral ventricle for the removal of a third ventricle craniopharyngioma: A technical report.

Science.gov (United States)

Yano, Shigetoshi; Hide, Takuichiro; Shinojima, Naoki; Ueda, Yutaka; Kuratsu, Jun-Ichi

2015-01-01

Intraventricular craniopharyngiomas are difficult to remove. We combined an interhemispheric transcallosal approach with a flexible endoscope (videoscope) for successful tumor removal. A 52-year-old male complained of general fatigue and memory disturbance. Magnetic resonance imaging revealed a well-enhanced third ventricle mass with dilatation of lateral ventricles. During removal with the interhemispheric transcallosal approach, a videoscope that was inserted into the left lateral ventricle revealed the interface of the tumor and the ventricular wall. The tumor was pushed to the right using forceps and removed totally through the right foramen of Monro without any fornix injury. This procedure is a safe option for removing third ventricular tumors especially in the case with hydrocephalus.

Phosphaturic mesenchymal tumor of the brain without tumor-induced osteomalacia in an 8-year-old girl: case report.

Science.gov (United States)

Ellis, Mark B; Gridley, Daniel; Lal, Suresh; Nair, Geetha R; Feiz-Erfan, Iman

2016-05-01

Phosphaturic mesenchymal tumor (mixed connective tissue variant) (PMT-MCT) are tumors that may cause tumor-induced osteomalacia and rarely appear intracranially. The authors describe the case of an 8-year-old girl who was found to have PMT-MCT with involvement of the cerebellar hemisphere and a small tumor pedicle breaching the dura mater and involving the skull. This was removed surgically in gross-total fashion without further complication. Histologically the tumor was confirmed to be a PMT-MCT. There was no evidence of tumor-induced osteomalacia. At the 42-month follow-up, the patient is doing well, has no abnormalities, and is free of recurrence. PMT-MCTs are rare tumors that may involve the brain parenchyma. A gross-total resection may be effective to cure these lesions.

Neurophysiological intraoperative monitoring during an optic nerve schwannoma removal.

Science.gov (United States)

San-Juan, Daniel; Escanio Cortés, Manuel; Tena-Suck, Martha; Orozco Garduño, Adolfo Josué; López Pizano, Jesús Alejandro; Villanueva Domínguez, Jonathan; Fernández Gónzalez-Aragón, Maricarmen; Gómez-Amador, Juan Luis

2017-10-01

This paper reports the case of a patient with optic nerve schwannoma and the first use of neurophysiological intraoperative monitoring of visual evoked potentials during the removal of such tumor with no postoperative visual damage. Schwannomas are benign neoplasms of the peripheral nervous system arising from the neural crest-derived Schwann cells, these tumors are rarely located in the optic nerve and the treatment consists on surgical removal leading to high risk of damage to the visual pathway. Case report of a thirty-year-old woman with an optic nerve schwannoma. The patient underwent surgery for tumor removal on the left optic nerve through a left orbitozygomatic approach with intraoperative monitoring of left II and III cranial nerves. We used Nicolet Endeavour CR IOM (Carefusion, Middleton WI, USA) to performed visual evoked potentials stimulating binocularly with LED flash goggles with the patient´s eyes closed and direct epidural optic nerve stimulation delivering rostral to the tumor a rectangular current pulse. At follow up examinations 7 months later, the left eye visual acuity was 20/60; Ishihara score was 8/8 in both eyes; the right eye photomotor reflex was normal and left eye was mydriatic and arreflectic; optokinetic reflex and ocular conjugate movements were normal. In this case, the epidural direct electrical stimulation of optic nerve provided stable waveforms during optic nerve schwannoma resection without visual loss.

Aniridia--Wilms′ tumour association--a case with 11p 13-14.1 deletion and ventricular septal defect.

OpenAIRE

Rao S; Athale U; Kadam P; Gladstone B; Nair C; Pai S; Kurkure P; Advani S

1992-01-01

A two year old female child with bilateral wilms tumor (WT) along with multiple congenital anomalies like bilateral aniridia with congenital cataracts and nystagmus, microcephaly, mental retardation and ventricular septal defect has been described. The karyotype analysis revealed 46 xx, del 11p 13-14.1. Association of ventricular septal defect with the classical features of ′Aniridia-Wilms′ tumor association′ is an unusual feature in this case.

[Extracorporeal circulation and hypothermy surgery in tumors with vena cava extension: 20 years experience at the University Clinic of Navarra].

Science.gov (United States)

Rioja Zuazu, J; Rodríguez-Rubio Cortadillas, F; Zudaire Bergera, J J; Saiz Sansi, A; Rosell Costa, D; Robles García, J E; Rábago, G; Berián Polo, J M

2008-04-01

We present our 20 years experience treating patients with vena cava extension in whom an extracorporeal circulation, hypothermia, cardio circulatory arrest (ECC-H-CCA) in order to perform, together with a tumoral resection, a thrombus resection. From 1985 to 2005 a total of 28 retroperitoneal tumor were treated: 25 renal cancers, a Wilms tumor, a paratesticular rabdomiosarcoma, and a pheocromocitoma. All of them had an extension by means of thrombus above the suprahepatics veins. All of them were treated by means of ECC-H-CCA for thrombus extraction. A descriptive study of the serie is performed as well as a Kaplan Meyer survival study. Surgical complications were present within 10 patients (35%), with a surgical mortality of two patients (7%): one intra-operatively because a massive embolism of the lungs and the other because of a lung embolism on the 4th post-operative day. Global actuarial survival was 29.1+/-10% at three years and 17.5+/-8% at five years. Analyzing only who do not have metastatic lesions, nor lymph nodes at diagnosis their three year survival was 50.9+/-16.3% and 32.2+/-16% at five years. Mean while those who have any metastatic lesion at diagnosis their three and five years survival was 20.8+/-12% and 10.4+/-9% respectively. The employ of surgical techniques with ECC-H-CCA with in oncological pathology associated with vena cava thrombus is justified and its employment does not worsen the survival; it is indicated because its results, allowing a complete tumoral resection in a safe and reproducible fashion.

Multi-site and multi-depth in vivo cancer localization enhancement after auto-fluorescence removal

International Nuclear Information System (INIS)

Montcuquet, A.S.; Herve, L.; Navarro, F.; Dinten, J.M.; Mars, J.I.

2011-01-01

Fluorescence imaging in diffusive media locates tumors tagged by injected fluorescent markers in NIR wavelengths. For deep embedded markers, natural auto-fluorescence of tissues comes to be a limiting factor to tumor detection and accurate FDOT reconstructions. A spectroscopic approach coupled with Non-negative Matrix Factorization source separation method is explored to discriminate fluorescence sources according to their fluorescence spectra and remove unwanted auto-fluorescence. We successfully removed auto-fluorescence from acquisitions on living mice with a single subcutaneous tumor or two capillary tubes inserted at different depths. (authors)

Extended Endoscopic Endonasal Approach for Craniopharyngioma Removal.

Science.gov (United States)

Messerer, Mahmoud; Maduri, Rodolfo; Daniel, Roy Thomas

2018-02-01

Objective  Endoscopic transsphenoidal extended endoscopic approach (EEA) represents a valid alternative to microsurgery for craniopharyngiomas removal, especially for retrochiasmatic lesions without large parasellar extension. The present video illustrates the salient surgical steps of the EEA for craniopahryngioma removal. Patient  A 52-year-old man presented with a bitemporal hemianopia and a bilateral decreased visual acuity. MRI showed a Kassam type III cystic craniopharyngioma with a solid component ( Fig. 1 , panels A and B). Surgical Procedure  The head is rotated 10 degrees toward the surgeons. The nasal step is started through the left nostril with a middle turbinectomy. A nasoseptal flap is harvested and positioned in the left choana. The binostril approach allows a large sphenoidotomy to expose the key anatomic landmarks. The craniotomy boundaries are the planum sphenoidale superiorly, the median opticocarotid recesses, the internal carotid artery laterally and the clival recess inferiorly. After dural opening and superior intercavernous sinus coagulation, the tumor is entirely removed ( Fig. 2 , panels A and B). Skull base reconstruction is ensured by fascia lata grafting and nasoseptal flap positioning. Results  Postoperative MRI showed the complete tumor resection ( Fig. 1 , panels C and D). At 3 months postoperatively, the bitemporal hemianopia regressed and the visual acuity improved. A novel left homonymous hemianopia developed secondary to optic tract manipulation. Conclusions  The extended EEA is a valid surgical approach for craniopharyngioma resection. A comprehensive knowledge of the sellar and parasellar anatomy is mandatory for safe tumor removal with decreased morbidity and satisfactory oncologic results. The link to the video can be found at: https://youtu.be/NrCPPnVK2qA .

Stages of Gastrointestinal Carcinoid Tumors

Science.gov (United States)

... used: Endoscopic resection : Surgery to remove a small tumor that is on the inside lining of the GI tract. An endoscope is inserted through the mouth and passed through the esophagus to the stomach and sometimes, the duodenum . An ...

Management of CNS tumors

International Nuclear Information System (INIS)

Griem, M.L.

1987-01-01

The treatment of tumors of the CNS has undergone a number of changes based on the impact of CT. The use of intraoperative US for the establishment of tumor location and tumor histology is demonstrated. MR imaging also is beginning to make an impact on the diagnosis and treatment of tumors of the CNS. Examples of MR images are shown. The authors then discuss the important aspects of tumor histology as it affects management and newer concepts in surgery, radiation, and chemotherapy on tumor treatment. The role of intraoperative placement of radioactive sources, the utilization of heavy particle radiation therapy, and the potential role of other experimental radiation therapy techniques are discussed. The role of hyperfractionated radiation and of neutrons and x-ray in a mixed-beam treatment are discussed in perspective with standard radiation therapy. Current chemotherapy techniques, including intraarterial chemotherapy, are discussed. The complications of radiation therapy alone and in combination with chemotherapy in the management of primary brain tumors, brain metastases, and leukemia are reviewed. A summary of the current management of pituitary tumors, including secreting pituitary adenomas and chromophobe adenomas, are discussed. The treatment with heavy particle radiation, transsphenoidal microsurgical removal, and combined radiotherapeutic and surgical management are considered. Tumor metastasis management of lesions of the brain and spinal cord are considered

The distal region of 11p13 and associated genetic diseases

NARCIS (Netherlands)

Mannens, M.; Hoovers, J. M.; Bleeker-Wagemakers, E. M.; Redeker, E.; Bliek, J.; Overbeeke-Melkert, M.; Saunders, G.; Williams, B.; van Heyningen, V.; Junien, C.

1991-01-01

The distal region of human chromosome band 11p13 is believed to contain a cluster of genes involved in the development of the eye, kidney, urogenital tract, and possibly the nervous system. Genetic abnormalities of this region can lead to Wilms tumor, aniridia, urogenital abnormalities, and mental

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 ...

Kidney (Renal Cell) Cancer—Health Professional Version

Science.gov (United States)

Kidney cancer has three main types. Renal cell cancer, or renal cell adenocarcinoma, forms in the tubules of the kidney. Transitional cell carcinoma forms in the renal pelvis and ureter. Wilms tumors are common in children. Find evidence-based information on kidney cancer treatment, research, genetics, and statistics.

Cerenkov and radioluminescence imaging of brain tumor specimens during neurosurgery

Science.gov (United States)

Spinelli, Antonello Enrico; Schiariti, Marco P.; Grana, Chiara M.; Ferrari, Mahila; Cremonesi, Marta; Boschi, Federico

2016-05-01

We presented the first example of Cerenkov luminescence imaging (CLI) and radioluminescence imaging (RLI) of human tumor specimens. A patient with a brain meningioma localized in the left parietal region was injected with 166 MBq of Y90-DOTATOC the day before neurosurgery. The specimens of the tumor removed during surgery were imaged using both CLI and RLI using an optical imager prototype developed in our laboratory. The system is based on a cooled electron multiplied charge coupled device coupled with an f/0.95 17-mm C-mount lens. We showed for the first time the possibility of obtaining CLI and RLI images of fresh human brain tumor specimens removed during neurosurgery.

Alfa-fetoprotein secreting ovarian sex cord-stromal tumor

Directory of Open Access Journals (Sweden)

Kusum D Jashnani

2013-01-01

Full Text Available Ovarian sex cord-stromal tumors are relatively infrequent neoplasms that account for approximately 8% of all primary ovarian tumors. They are a heterogeneous group of neoplasms composed of cells derived from gonadal sex cords (granulosa and Sertoli cells, specialized gonadal stroma (theca and Leydig cells, and fibroblasts. They may show androgenic or estrogenic manifestations. We report such a tumor associated with markedly raised serum alpha-fetoprotein (AFP levels in a young female presenting with a mass and defeminising symptoms. Serum AFP levels returned to normal on removal of tumor.

Imaging of childhood inflammatory myofibroblastic tumor

Energy Technology Data Exchange (ETDEWEB)

Oguz, Berna; Ozcan, Hatice Nursun; Omay, Burak; Ozgen, Burce; Haliloglu, Mithat [Division of Pediatric Radiology, Hacettepe University Faculty of Medicine, Department of Radiology, Altindag / Sihhiye, Ankara (Turkey)

2015-10-15

Inflammatory myofibroblastic tumor is a rare benign neoplasm and most commonly involves the lung but occurs in extrapulmonary locations. To present imaging findings in inflammatory myofibroblastic tumors in children based on a single-centre experience. We retrospectively reviewed CT and MRI findings of children diagnosed with inflammatory myofibroblastic tumor in a single institution. We identified 15 children (range: 1-17 years) with inflammatory myofibroblastic tumor. The tumor was localized to the lung (n = 5), mediastinum (n = 3), trachea (n = 1), bronchus (n = 1), abdomen (n = 2) and orbit (n = 3). All the extraorbital tumors were solid masses with homogeneous or heterogeneous enhancement. Four lung tumors and one posterior mediastinal tumor contained calcification. Local recurrence following surgical removal occurred in two children with invasion of the esophagus and of the left atrium in one. Localized masses were seen in all children with orbital tumour. Two of these had episcleritis and perineuritis; one had episcleritis, tendonitis, perineuritis, myositis and dacryoadenitis. The locations and imaging features of inflammatory myofibroblastic tumors are variable. (orig.)

Does childhood cancer affect parental divorce rates? A population-based study.

Science.gov (United States)

Syse, Astri; Loge, Jon H; Lyngstad, Torkild H

2010-02-10

PURPOSE Cancer in children may profoundly affect parents' personal relationships in terms of psychological stress and an increased care burden. This could hypothetically elevate divorce rates. Few studies on divorce occurrence exist, so the effect of childhood cancers on parental divorce rates was explored. PATIENTS AND METHODS Data on the entire Norwegian married population, age 17 to 69 years, with children age 0 to 20 years in 1974 to 2001 (N = 977,928 couples) were retrieved from the Cancer Registry, the Central Population Register, the Directorate of Taxes, and population censuses. Divorce rates for 4,590 couples who were parenting a child with cancer were compared with those of otherwise similar couples by discrete-time hazard regression models. Results Cancer in a child was not associated with an increased risk of parental divorce overall. An increased divorce rate was observed with Wilms tumor (odds ratio [OR], 1.52) but not with any of the other common childhood cancers. The child's age at diagnosis, time elapsed from diagnosis, and death from cancer did not influence divorce rates significantly. Increased divorce rates were observed for couples in whom the mothers had an education greater than high school level (OR, 1.16); the risk was particularly high shortly after diagnosis, for CNS cancers and Wilms tumors, for couples with children 0 to 9 years of age at diagnosis, and after a child's death. CONCLUSION This large, registry-based study shows that cancer in children is not associated with an increased parental divorce rate, except with Wilms tumors. Couples in whom the wife is highly educated appear to face increased divorce rates after a child's cancer, and this may warrant additional study.

A Novel WT1 Gene Mutation in a Three-Generation Family with Progressive Isolated Focal Segmental Glomerulosclerosis

Science.gov (United States)

Caridi, Gianluca; Malaventura, Cristina; Dagnino, Monica; Leonardi, Emanuela; Artifoni, Lina; Ghiggeri, Gian Marco; Tosatto, Silvio C.E.; Murer, Luisa

2010-01-01

Background and objectives: Wilms tumor-suppressor gene-1 (WT1) plays a key role in kidney development and function. WT1 mutations usually occur in exons 8 and 9 and are associated with Denys-Drash, or in intron 9 and are associated with Frasier syndrome. However, overlapping clinical and molecular features have been reported. Few familial cases have been described, with intrafamilial variability. Sporadic cases of WT1 mutations in isolated diffuse mesangial sclerosis or focal segmental glomerulosclerosis have also been reported. Design, setting, participants, & measurements: Molecular analysis of WT1 exons 8 and 9 was carried out in five members on three generations of a family with late-onset isolated proteinuria. The effect of the detected amino acid substitution on WT1 protein's structure was studied by bioinformatics tools. Results: Three family members reached end-stage renal disease in full adulthood. None had genital abnormalities or Wilms tumor. Histologic analysis in two subjects revealed focal segmental glomerulosclerosis. The novel sequence variant c.1208G>A in WT1 exon 9 was identified in all of the affected members of the family. Conclusions: The lack of Wilms tumor or other related phenotypes suggests the expansion of WT1 gene analysis in patients with focal segmental glomerulosclerosis, regardless of age or presence of typical Denys-Drash or Frasier syndrome clinical features. Structural analysis of the mutated protein revealed that the mutation hampers zinc finger-DNA interactions, impairing target gene transcription. This finding opens up new issues about WT1 function in the maintenance of the complex gene network that regulates normal podocyte function. PMID:20150449

Clinical observation of video-assisted removal of sarcoma of the nasopharynx after simultaneous laryngectomy

Directory of Open Access Journals (Sweden)

A. P. Polyakov

2015-01-01

Full Text Available Sarcomas in the head and neck are rare, and account for approximately 5-15% of all sarcomas in adults, is less than 1% of all neoplasms of the head and neck. Due to anatomical and physiological features of the structure of the nasopharynx, surgical treatment of tumors of this localization is associated with a number of technical difficulties, the high risk of non-radical removal of the tumor, it was therefore necessary for adequate surgical access. The article describes video-assisted access for removal of tumors of the nasopharynx with good visualization of the tumor process simultaneously after laryngectomy with the formation of pharyngotomy, providing the opportunity to monitor the postoperative area of the nasopharynx and early detection of continued tumor growth in patients with soft tissue sarcoma of the nasopharynx and combined lesions of the larynx.

Severe gonadotoxic insult manifests early in young girls treated for Ewing sarcoma

DEFF Research Database (Denmark)

Mörse, Helena; Elfving, Maria; Turkiewicz, Aleksandra

2016-01-01

We prospectively investigated anti-Müllerian hormone (AMH) as a measure of ovarian insult in young females during and after treatment for Wilms tumor (WT), osteosarcoma (OS), and Ewing sarcoma (ES).Twenty-one female childhood cancer patients, with a mean age of 7.9 years (range 0.6-17), entered...

Frequency of malignant tumors during the first two decades of life in the offspring (F1) of atomic bomb survivors

International Nuclear Information System (INIS)

Yoshimoto, Yasuhiko; Neel, J.V.; Kato, Hiroo; Mabuchi, Kiyohiko; Schull, W.J.; Soda, Midori; Eto, Ryozo.

1990-05-01

The incidence of cancer prior to age 20 has been determined in children born to atomic bomb survivors and to a suitable comparison group. Tumor ascertainment was through death certificates and the tumor registries maintained in Hiroshima and Nagasaki. The rationale for the study stemmed from the evidence that a significant proportion of childhood tumors such as retinoblastoma and Wilms' tumor arise on the basis of a mutant gene inherited from one parent plus a second somatic cell mutation involving the allele of this gene. Gonadal radiation doses were calculated using the recently established DS86 system, supplemented by an ad hoc system for those children whose parents' (one or both) DS86 dose could not be computed but for whom a dose could be developed on the basis of the available information. The total data set consisted of: 1) a cohort of 31,150 liveborn children, one or both of whose parents received ≥ 0.01 Sv of radiation at the time of the A-bombings (an average conjoint gonad exposure of 0.435 Sv), and 2) two suitable comparison groups, totaling 41,066 children. A total of 92 cancer cases at age less than 20 years was confirmed; 49 and 43 cases, respectively, in the 0 Sv and ≥ 0.01 Sv groups. A multiple linear regression analysis revealed no increase in malignancy in the children of exposed parents. However, examination of the data suggested that only 3.0 % to 5.0 % of the tumors of childhood observed in the comparison groups are associated with an inherited genetic predisposition that would be expected to exhibit an altered frequency if the parental mutation rate were increased. These is thus far no confirmation of the positive findings of Nomura in a mouse system. (author)

A knock-in mouse line conditionally expressing the tumor suppressor WTX/AMER1.

Science.gov (United States)

Boutet, Agnès; Comai, Glenda; Charlet, Aurélie; Jian Motamedi, Fariba; Dhib, Haroun; Bandiera, Roberto; Schedl, Andreas

2017-11-01

WTX/AMER1 is an important developmental regulator, mutations in which have been identified in a proportion of patients suffering from the renal neoplasm Wilms' tumor and in the bone malformation syndrome Osteopathia Striata with Cranial Sclerosis (OSCS). Its cellular functions appear complex and the protein can be found at the membrane, within the cytoplasm and the nucleus. To understand its developmental and cellular function an allelic series for Wtx in the mouse is crucial. Whereas mice carrying a conditional knock out allele for Wtx have been previously reported, a gain-of-function mouse model that would allow studying the molecular, cellular and developmental role of Wtx is still missing. Here we describe the generation of a novel mouse strain that permits the conditional activation of WTX expression. Wtx fused to GFP was introduced downstream a stop cassette flanked by loxP sites into the Rosa26 locus by gene targeting. Ectopic WTX expression is reported after crosses with several Cre transgenic mice in different embryonic tissues. Further, functionality of the fusion protein was demonstrated in the context of a Wtx null allele. © 2017 Wiley Periodicals, Inc.

A SOLITARY FIBROUS ORBITAL TUMOR IN A PATIENT WITH NEUROFIBROMATOSIS AND AN UTERINE CARCINOMA

Directory of Open Access Journals (Sweden)

E. E. Grishina

2016-01-01

Full Text Available We present a rare combination of a solitary fibrous orbital tumor and uterine cancer in a  female patient with type I  neurofibromatosis. This 77-year old patient developed a  left painless exophthalmos within 2 years and decreased visual acuity of the left eye. At the age of 20  she was diagnosed with type I neurofibromatosis. Half a year ago she underwent hysteron-oophorectomy due to uterine adenocarcinoma. The visual acuity of her left eye was decreased to 0.3, with an increase of intraocular pressure to 30 mm Hg. She had a 13-mm left-sided exophthalmos with misplacement of the eye downwards and laterally at 40°. Reposition of the left eye was severely impaired, with limitation of the eye movements to all directions. Ophthalmoscopy showed optic disc discoloration and blunting of its inner border. The patient underwent trans-conjunctival orbitotomy, with removal of three encapsulated tumor nodules. Histological and immunochemical studies of the removed tissue identified solitary fibrous tumor of the left orbit with an undetermined malignant potential. In the post-operative period, visual acuity of the left eye was 0.2, with no exophthalmos and right position of the eye. There was a non-significant limitation of the left eye movement to the left and to the right. X-ray computed tomography confirmed radical tumor excision. Conclusion: Solitary fibrous tumor is a  rare orbital neoplasm. Nevertheless, it should be included into the differential diagnosis list of spin-cell orbital tumors. It is necessary to aim at tumor removal through the least traumatic orbital access. Relapsing course of the tumor is the rationale for a  long-term follow-up of patients after removal of solitary fibrous orbital tumor.

Granular cell tumor of the esophagus. Report of three cases.

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Cohle, S D; McKechnie, J C; Truong, L; Jurco, S

1981-06-01

Granular cell tumors, (formerly called myoblastomas) involving the esophagus were encountered in three patients. In all three the tumors were asymptomatic and in two they were multiple. The first published endoscopic photographs of such a tumor are presented. The successful total removal of this neoplasm using the endoscope is described. The pathologic, radiologic and therapeutic aspects of previously reported cases of granular cell tumor of the esophagus are reviewed and compared with the three reported herein.

Tandem duplication of 11p12-p13 in a child with borderline development delay and eye abnormalities: dose effect of the PAX6 gene product?

NARCIS (Netherlands)

Aalfs, C. M.; Fantes, J. A.; Wenniger-Prick, L. J.; Sluijter, S.; Hennekam, R. C.; van Heyningen, V.; Hoovers, J. M.

1997-01-01

We report on a girl with a duplication of chromosome band 11p12-->13, which includes the Wilms tumor gene (WT1) and the aniridia gene (PAX6). The girl had borderline developmental delay, mild facial anomalies, and eye abnormalities. Eye findings were also present in most of the 11 other published

The Standardized Histogram Shift of T2 Magnetic Resonance Image (MRI) Signal Intensities of Nephroblastoma Does Not Predict Histopathological Diagnostic Information

OpenAIRE

M?ller, Sabine; David, Ruslan; Marias, Kostas; Graf, Norbert

2015-01-01

The objective of this study is to assess standardized histograms of signal intensities of T2-weighted magnetic resonance image (MRI) modality before and after preoperative chemotherapy for nephroblastoma (Wilms? tumor). All analyzed patients are enrolled in the International Society of Paediatric Oncology (SIOP) 2001/GPOH trial.1 The question to be answered is whether the comparison of the histograms can add new knowledge by comparing them with the histology of the tumor after preoperative ch...

General Information about Gastrointestinal Carcinoid Tumors

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... used: Endoscopic resection : Surgery to remove a small tumor that is on the inside lining of the GI tract. An endoscope is inserted through the mouth and passed through the esophagus to the stomach and sometimes, the duodenum . An ...

Treatment Option Overview (Gastrointestinal Carcinoid Tumors)

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... used: Endoscopic resection : Surgery to remove a small tumor that is on the inside lining of the GI tract. An endoscope is inserted through the mouth and passed through the esophagus to the stomach and sometimes, the duodenum . An ...

Effect of surgical resection combined with transcatheter arterial chemoembolization on postoperative serum tumor marker levels and stem cell characteristics during tumor recurrence

Directory of Open Access Journals (Sweden)

Sen Yang

2017-05-01

Full Text Available Objective: To study the effect of surgical resection combined with transcatheter arterial chemoembolization (TACE on postoperative serum tumor marker levels and stem cell characteristics during tumor recurrence. Methods: A total of 98 patients with liver cancer who received radical resection in our hospital between May 2013 and July 2015 were reviewed and divided into TACE group and control group according to whether they received TACE within two months after surgical resection. Serum levels of tumor markers were detected 4 weeks after operation; the tumor recurrence was followed up within 3 years after operation, and the expression of stem cell marker molecules and cell proliferation molecules in recurrent lesions were detected. Results: 4 weeks after radical hepatectomy, serum AFP, AFP-L3, GP73 and GPC3 levels in TACE group were significantly lower than those in control group; Nanog, CD133, EpCAM, PICK1, CyclinD1, C-myc and Survivin expression in surgically removed lesions of TACE group were not different from those of control group while Nanog, CD133, EpCAM, PICK1, CyclinD1, C-myc and Survivin expression in recurrent lesions were significantly lower than those of control group. Conclusion: Surgical resection combined with TACE can more effectively remove liver cancer lesions, reduce the tumor marker levels and inhibit the tumor stem cell characteristics and cell proliferation activity in recurrent lesions.

Pseudoanaplastic tumors of bone

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Bahk, Won-Jong [Uijongbu St. Mary Hospital, The Catholic University of Korea, Department of Orthopaedic Surgery, Gyunggido, 480-821 (Korea); Mirra, Joseph M. [Orthopaedic Hospital, Orthopedic Oncology, Los Angeles, California (United States)

2004-11-01

To discuss the concept of pseudoanaplastic tumors of bone, which pathologically show hyperchromatism and marked pleomorphism with quite enlarged, pleomorphic nuclei, but with no to extremely rare, typical mitoses, and to propose guidelines for their diagnosis. From a database of 4,262 bone tumors covering from 1971 to 2001, 15 cases of pseudoanaplastic bone tumors (0.35% of total) were retrieved for clinical, radiographic and pathologic review. Postoperative follow-up after surgical treatment was at least 3 years and a maximum of 7 years. There were eight male and seven female patients. Their ages ranged from 10 to 64 years with average of 29.7 years. Pathologic diagnoses of pseudoanaplastic variants of benign bone tumors included: osteoblastoma (4 cases), giant cell tumor (4 cases), chondromyxoid fibroma (3 cases), fibrous dysplasia (2 cases), fibrous cortical defect (1 case) and aneurysmal bone cyst (1 case). Radiography of all cases showed features of a benign bone lesion. Six cases, one case each of osteoblastoma, fibrous dysplasia, aneurysmal bone cyst, chondromyxoid fibroma, giant cell tumor and osteoblastoma, were initially misdiagnosed as osteosarcoma. The remaining cases were referred for a second opinion to rule out sarcoma. Despite the presence of significant cytologic aberrations, none of our cases showed malignant behavior following simple curettage or removal of bony lesions. Our observation justifies the concept of pseudoanaplasia in some benign bone tumors as in benign soft tissue tumors, especially in their late evolutionary stage when bizarre cytologic alterations strongly mimic a sarcoma. (orig.)

Occult left atrial ball-like thrombus in a patient referred for surgical removal of suspected cerebellum tumor

International Nuclear Information System (INIS)

Możeńska, Olga; Kalińska, Irena; Brodowski, Karol; Walecki, Jerzy; Kosior, Dariusz A.

2014-01-01

Atrial fibrillation and related cardio-embolic cerebrovascular accidents are two well-defined major healthcare problems worldwide. It has been approximated that 2.2 million people in America and 4.5 million in European Union have paroxysmal or persistent atrial fibrillation. And atrial fibrillation itself is an independent long-term risk factor of stroke. We present a case of patient referred to our center for surgical removal of suspected cerebellum tumor, a case that had a rather unexpected ending. A 58-year-old male patient with a history of atrial fibrillation, congestive heart failure (NYHA II/III), stable coronary artery disease, diabetes type 2 and hyperlipidemia presented with vertigo, headaches, mainly during physical activity and increased tiredness. Performed computer tomography revealed two lesions in the cerebellum and in the left lateral chamber. The diagnosis of a proliferative disease of the cerebellum was established and patient was referred to the Neurosurgical Department. Fortunately, before the operation the echocardiography was performed, which revealed two lesions in left atrium. The decision of the Heart Team was to refer the patient for an open-heart surgery, in which two thrombi were removed. Neurosurgeons decided to withdraw from further surgery and proceed with head MRI and conservative treatment, deciding that the lesion in the cerebellum was most likely an ischemic area. Looking at the brain lesion should always be done from the whole patient’s perspective. And using mutlimodality imaging may lead to appropriate diagnosis, correct course of therapeutic action and unexpected ending of a rather non-extraordinary case

Lateral skull base approaches in the management of benign parapharyngeal space tumors.

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Prasad, Sampath Chandra; Piccirillo, Enrico; Chovanec, Martin; La Melia, Claudio; De Donato, Giuseppe; Sanna, Mario

2015-06-01

To evaluate the role of lateral skull base approaches in the management of benign parapharyngeal space tumors and to propose an algorithm for their surgical approach. Retrospective study of patients with benign parapharyngeal space tumors. The clinical features, radiology and preoperative management of skull base neurovasculature, the surgical approaches and overall results were recorded. 46 patients presented with 48 tumors. 12 were prestyloid and 36 poststyloid. 19 (39.6%) tumors were paragangliomas, 15 (31.25%) were schwannomas and 11 (23%) were pleomorphic adenomas. Preoperative embolization was performed in 19, stenting of the internal carotid artery in 4 and permanent balloon occlusion in 2 patients. 19 tumors were approached by the transcervical, 13 by transcervical-transparotid, 5 by transcervical-transmastoid, 6, 1 and 2 tumors by the infratemporal fossa approach types A, B and D, respectively. Total radical tumor removal was achieved in 46 (96%) of the cases. Lateral skull base approaches have an advantage over other approaches in the management of benign tumors of the parapharyngeal space due to the fact that they provide excellent exposure with less morbidity. The use of microscope combined with bipolar cautery reduces morbidity. Stenting of internal carotid artery gives a chance for complete tumor removal with arterial preservation. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Can selective pharmaco-angiography of renal tumor replace the examination under surgery?

International Nuclear Information System (INIS)

Will, C.H.; Bach, D.; Koop, H.; Impekoven, P.

1996-01-01

Can primary nephrectomy be performed without preliminary sample excision of the tumor if pharmaco-angiography of the kidney has demonstrated the typical tumor vascularization? To clarify this question in 32 patients with 'displacing mass' of the kidney, verified in sonography and computer-tomography, or hematuria of unknown origin, we prospectively performed an additional pharmaco-angiography of the respective kidney. In 18 patients with tumor vascularization in the pharmaco-angiography, intraoperatively we found 15 malignant renal cell carcinomas, 1 patient with transitional cell carcinoma of the renal pelvis, 1 leiomyosarcoma, and 1 high-differentiated tumor of only 2 cm in diameter with unclear dignity, which was treated by enucleation. In cases of an intrarenal lesion of more than 3 cm in diameter and additional tumor vascularization seen in selective pharmaco-angiography, the kidney undoubtedly can be removed by primary nephrectomy without a preliminary sample excision to confirm the diagnosis. For tumors with a diameter of less than 3 cm and additional tumor-vascularization, the option should be enucleation. If there is a 'tumor' without typical malignant vascularization, the exploration by sample excision should be performed. Depending on the histological result the tumor should be removed by enucleation or nephrectomy. (orig.) [de

Human Langerhans cells use an IL-15R-α/IL-15/pSTAT5-dependent mechanism to break T-cell tolerance against the self-differentiation tumor antigen WT1.

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Romano, Emanuela; Cotari, Jesse W; Barreira da Silva, Rosa; Betts, Brian C; Chung, David J; Avogadri, Francesca; Fink, Mitsu J; St Angelo, Erin T; Mehrara, Babak; Heller, Glenn; Münz, Christian; Altan-Bonnet, Gregoire; Young, James W

2012-05-31

Human CD34(+) progenitor-derived Langerhans-type dendritic cells (LCs) are more potent stimulators of T-cell immunity against tumor and viral antigens in vitro than are monocyte-derived DCs (moDCs). The exact mechanisms have remained elusive until now, however. LCs synthesize the highest amounts of IL-15R-α mRNA and protein, which binds IL-15 for presentation to responder lymphocytes, thereby signaling the phosphorylation of signal transducer and activator of transcription 5 (pSTAT5). LCs electroporated with Wilms tumor 1 (WT1) mRNA achieve sufficiently sustained presentation of antigenic peptides, which together with IL-15R-α/IL-15, break tolerance against WT1 by stimulating robust autologous, WT1-specific cytolytic T-lymphocytes (CTLs). These CTLs develop from healthy persons after only 7 days' stimulation without exogenous cytokines and lyse MHC-restricted tumor targets, which include primary WT1(+) leukemic blasts. In contrast, moDCs require exogenous rhuIL-15 to phosphorylate STAT5 and attain stimulatory capacity comparable to LCs. LCs therefore provide a more potent costimulatory cytokine milieu for T-cell activation than do moDCs, thus accounting for their superior stimulation of MHC-restricted Ag-specific CTLs without need for exogenous cytokines. These data support the use of mRNA-electroporated LCs, or moDCs supplemented with exogenous rhuIL-15, as vaccines for cancer immunotherapy to break tolerance against self-differentiation antigens shared by tumors.

Paraneoplastic neurological disorders in children with benign ovarian tumors.

Science.gov (United States)

Hsu, Mei-Hsin; Huang, Chao-Ching; Hung, Pi-Lien; Huang, Hsiu-Mei; Huang, Li-Tung; Huang, Chao-Cheng; Sheen, Jiunn-Ming; Huang, Song-Chei; Chang, Ying-Chao

2014-03-01

Paraneoplastic neurological diseases (PND) are rare, but potentially treatable disorders. Paraneoplastic encephalitis is rapidly emerging as an important but likely under-recognized condition in children. The aim of this study was to assess the prevalence and spectrum of PND in children with benign ovary tumor and the long-term outcome. We retrospectively reviewed the charts of all female patients below 18years of age diagnosed with a benign ovarian tumor proven by pathology between January 1993 and December 2010. All the clinical symptoms developed within 5years of tumor diagnosis and the related investigations were recorded. There were total 133 children and adolescents with benign ovarian tumors, mostly mature teratoma. Six patients (4.5%) had neuropsychiatric manifestations and all but one were beyond age 10years. The most common neuropsychiatric presentations were depression or low mood (84%), headache (50%), mutism (50%), hypoventilation (50%), seizures (30%), hallucination (30%), vomiting and hypersalivation (30%). Three patients (2.2%) had serious PND including acute disseminated encephalomyelitis in 1 and anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis in 2. Although all of three improved after tumor removal, one without immunotherapy had neurological sequelae and prolonged ICU stay. The prevalence of PND in benign ovary tumor is not so uncommon in children. It is important to survey ovary tumors in female adolescents with subacute presentation of multiple-level involvement of neuraxis where no clear alternate diagnosis is possible. Treatment of serious PND associated with ovary tumors should include immunotherapy in addition to tumor removal. Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Historical Trends in the Use of Radiation Therapy for Pediatric Cancers: 1973-2008

International Nuclear Information System (INIS)

Jairam, Vikram; Roberts, Kenneth B.; Yu, James B.

2013-01-01

Purpose: This study was undertaken to assess historical trends in the use of radiation therapy (RT) for pediatric cancers over the past 4 decades. Methods: The National Cancer Institute's Surveillance, Epidemiology, and End Results database of the 9 original tumor registries (SEER-9) was queried to identify patients aged 0 to 19 years with acute lymphoblastic leukemia, acute myeloid leukemia, bone and joint cancer, cancer of the brain and nervous system, Hodgkin lymphoma, neuroblastoma, non-Hodgkin lymphoma, soft tissue cancer, Wilms tumor, or retinoblastoma from 1973 to 2008. Patients were grouped into 4-year time epochs. The number and percentage of patients who received RT as part of their initial treatment were calculated per epoch by each diagnosis group from 1973 to 2008. Results: RT use for acute lymphoblastic leukemia, non-Hodgkin lymphoma, and retinoblastoma declined sharply from 57%, 57%, and 30% in 1973 to 1976 to 11%, 15%, and 2%, respectively, in 2005 to 2008. Similarly, smaller declines in RT use were also seen in brain cancer (70%-39%), bone cancer (41%-21%), Wilms tumor (75%-53%), and neuroblastoma (60%-25%). RT use curves for Wilms tumor and neuroblastoma were nonlinear with nadirs in 1993 to 1996 at 39% and 19%, respectively. There were minimal changes in RT use for Hodgkin lymphoma, soft tissue cancer, or acute myeloid leukemia, roughly stable at 72%, 40%, and 11%, respectively. Almost all patients treated with RT were given external beam RT exclusively. However, from 1985 to 2008, treatments involving brachytherapy, radioisotopes, or combination therapy increased in frequency, comprising 1.8%, 4.6%, and 11.9% of RT treatments in brain cancer, soft tissue cancer, and retinoblastoma, respectively. Conclusions: The use of RT is declining over time in 7 of 10 pediatric cancer categories. A limitation of this study is a potential under-ascertainment of RT use in the SEER-9 database including the delayed use of RT

Historical Trends in the Use of Radiation Therapy for Pediatric Cancers: 1973-2008

Energy Technology Data Exchange (ETDEWEB)

Jairam, Vikram [Yale School of Medicine, Department of Therapeutic Radiology, New Haven, Connecticut (United States); Roberts, Kenneth B. [Yale School of Medicine, Department of Therapeutic Radiology, New Haven, Connecticut (United States); Yale Cancer Center, New Haven, Connecticut (United States); Cancer Outcomes, Public Policy, and Effectiveness Research (COPPER) Center at Yale, New Haven, Connecticut (United States); Yu, James B., E-mail: james.b.yu@yale.edu [Yale School of Medicine, Department of Therapeutic Radiology, New Haven, Connecticut (United States); Yale Cancer Center, New Haven, Connecticut (United States); Cancer Outcomes, Public Policy, and Effectiveness Research (COPPER) Center at Yale, New Haven, Connecticut (United States)

2013-03-01

Purpose: This study was undertaken to assess historical trends in the use of radiation therapy (RT) for pediatric cancers over the past 4 decades. Methods: The National Cancer Institute's Surveillance, Epidemiology, and End Results database of the 9 original tumor registries (SEER-9) was queried to identify patients aged 0 to 19 years with acute lymphoblastic leukemia, acute myeloid leukemia, bone and joint cancer, cancer of the brain and nervous system, Hodgkin lymphoma, neuroblastoma, non-Hodgkin lymphoma, soft tissue cancer, Wilms tumor, or retinoblastoma from 1973 to 2008. Patients were grouped into 4-year time epochs. The number and percentage of patients who received RT as part of their initial treatment were calculated per epoch by each diagnosis group from 1973 to 2008. Results: RT use for acute lymphoblastic leukemia, non-Hodgkin lymphoma, and retinoblastoma declined sharply from 57%, 57%, and 30% in 1973 to 1976 to 11%, 15%, and 2%, respectively, in 2005 to 2008. Similarly, smaller declines in RT use were also seen in brain cancer (70%-39%), bone cancer (41%-21%), Wilms tumor (75%-53%), and neuroblastoma (60%-25%). RT use curves for Wilms tumor and neuroblastoma were nonlinear with nadirs in 1993 to 1996 at 39% and 19%, respectively. There were minimal changes in RT use for Hodgkin lymphoma, soft tissue cancer, or acute myeloid leukemia, roughly stable at 72%, 40%, and 11%, respectively. Almost all patients treated with RT were given external beam RT exclusively. However, from 1985 to 2008, treatments involving brachytherapy, radioisotopes, or combination therapy increased in frequency, comprising 1.8%, 4.6%, and 11.9% of RT treatments in brain cancer, soft tissue cancer, and retinoblastoma, respectively. Conclusions: The use of RT is declining over time in 7 of 10 pediatric cancer categories. A limitation of this study is a potential under-ascertainment of RT use in the SEER-9 database including the delayed use of RT.

Historical trends in the use of radiation therapy for pediatric cancers: 1973-2008.

Science.gov (United States)

Jairam, Vikram; Roberts, Kenneth B; Yu, James B

2013-03-01

This study was undertaken to assess historical trends in the use of radiation therapy (RT) for pediatric cancers over the past 4 decades. The National Cancer Institute's Surveillance, Epidemiology, and End Results database of the 9 original tumor registries (SEER-9) was queried to identify patients aged 0 to 19 years with acute lymphoblastic leukemia, acute myeloid leukemia, bone and joint cancer, cancer of the brain and nervous system, Hodgkin lymphoma, neuroblastoma, non-Hodgkin lymphoma, soft tissue cancer, Wilms tumor, or retinoblastoma from 1973 to 2008. Patients were grouped into 4-year time epochs. The number and percentage of patients who received RT as part of their initial treatment were calculated per epoch by each diagnosis group from 1973 to 2008. RT use for acute lymphoblastic leukemia, non-Hodgkin lymphoma, and retinoblastoma declined sharply from 57%, 57%, and 30% in 1973 to 1976 to 11%, 15%, and 2%, respectively, in 2005 to 2008. Similarly, smaller declines in RT use were also seen in brain cancer (70%-39%), bone cancer (41%-21%), Wilms tumor (75%-53%), and neuroblastoma (60%-25%). RT use curves for Wilms tumor and neuroblastoma were nonlinear with nadirs in 1993 to 1996 at 39% and 19%, respectively. There were minimal changes in RT use for Hodgkin lymphoma, soft tissue cancer, or acute myeloid leukemia, roughly stable at 72%, 40%, and 11%, respectively. Almost all patients treated with RT were given external beam RT exclusively. However, from 1985 to 2008, treatments involving brachytherapy, radioisotopes, or combination therapy increased in frequency, comprising 1.8%, 4.6%, and 11.9% of RT treatments in brain cancer, soft tissue cancer, and retinoblastoma, respectively. The use of RT is declining over time in 7 of 10 pediatric cancer categories. A limitation of this study is a potential under-ascertainment of RT use in the SEER-9 database including the delayed use of RT. Copyright © 2013 Elsevier Inc. All rights reserved.

Mechanical disruption of tumors by iron particles and magnetic field application results in increased anti-tumor immune responses.

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Myriam N Bouchlaka

Full Text Available The primary tumor represents a potential source of antigens for priming immune responses for disseminated disease. Current means of debulking tumors involves the use of cytoreductive conditioning that impairs immune cells or removal by surgery. We hypothesized that activation of the immune system could occur through the localized release of tumor antigens and induction of tumor death due to physical disruption of tumor architecture and destruction of the primary tumor in situ. This was accomplished by intratumor injection of magneto-rheological fluid (MRF consisting of iron microparticles, in Balb/c mice bearing orthotopic 4T1 breast cancer, followed by local application of a magnetic field resulting in immediate coalescence of the particles, tumor cell death, slower growth of primary tumors as well as decreased tumor progression in distant sites and metastatic spread. This treatment was associated with increased activation of DCs in the draining lymph nodes and recruitment of both DCs and CD8(+T cells to the tumor. The particles remained within the tumor and no toxicities were observed. The immune induction observed was significantly greater compared to cryoablation. Further anti-tumor effects were observed when MRF/magnet therapy was combined with systemic low dose immunotherapy. Thus, mechanical disruption of the primary tumor with MRF/magnetic field application represents a novel means to induce systemic immune activation in cancer.

Endoscopic third ventriculostomy and posterior fossa tumors.

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Di Rocco, Federico; Jucá, Carlos Eduardo; Zerah, Michel; Sainte-Rose, Christian

2013-02-01

The management of hydrocephalus associated with a posterior fossa tumor is debated. Some authors emphasize the advantages of an immediate tumor removal that may normalize the cerebrospinal fluid (CSF) dynamics. However, in clinical practice, the mere excision of the lesion has been demonstrated to be accompanied by a persisting hydrocephalus in about one third of the cases. Preoperative endoscopic third ventriculostomy (ETV) offers several advantages. It may control the intracranial pressure (ICP), avoid the necessity of an emergency procedure, allow appropriate scheduling of the operation for tumor removal, and eliminate the risks related to the presence of an external drainage. The procedure also reduces the incidence of postoperative hydrocephalus. A final advantage, more difficult to weight, but obvious to the neurosurgeon, is the possibility to remove the lesion with a relaxed brain and normal ICP. In the postoperative phase, ETV can be used in case of persisting hydrocephalus, both in patients who underwent only the excision of the tumor and in those whose preoperative ETV failed as a consequence of intraventricular bleeding with secondary closure of the stoma (redoETV). The main advantage of postoperative ETV is that the procedure is carried out only in case of persisting hydrocephalus; its use is consequently more selective than preoperative ETV. The disadvantage consists in the common use of an external CSF drainage in the first few postoperative days, which is necessary to control the pressure and for ruling out those cases that reach a spontaneous cure of the hydrocephalus. The authors review the criteria for patient selection and the results of ETV performed in case of hydrocephalus secondary to a posterior fossa tumor. Preoperative ETV constitutes an effective procedure for controlling the hydrocephalus associated with posterior fossa tumors. It might lower the rate of persistent postoperative hydrocephalus and result in a short hospital stay. Low

A Clinical and Genetic Review of Aniridia

OpenAIRE

Reza Jafari; Ahmad Ahmadzadeh Amiri

2015-01-01

Aniridia is a congenital pan-ocular, bilateral disorder. The term aniridia is a misleading misnomer, since at least a rudimentary iris is always present. Varied forms range from almost total absence to only mild hypoplasia of the iris. It is inherent in a number of syndromes, including Wilms tumor Aniridia-Genital anomalies-retardation (WAGR). Aniridia has been shown to be associated with mutations in the PAX6 gene, located on chromosome 11p13, telomeric to the Wilms’ tumor predisposition gen...

[Anti-FGF23 antibody therapy for patients with tumor-induced osteomalacia].

Science.gov (United States)

Kinoshita, Yuka; Fukumoto, Seiji

2014-08-01

Tumor-induced osteomalacia (TIO) is a disease caused by fibroblast growth factor 23 (FGF23) secreted from the causative tumor. This disease is cured by complete surgical removal of the tumor. However, there are several difficult cases in which the responsible tumors cannot be found, are incompletely removed, or relapse after the surgery. Anti-FGF23 antibody is being studied as a novel therapy for FGF23-related hypophosphatemic diseases. The efficacy of anti-FGF23 antibodies were confirmed using a murine model of X-linked hypophosphatemic rickets (XLHR) , which is the most common heritable form of FGF23-related hypophosphatemic disease. In addition, results of phase I study of single injection of humanized anti-FGF23 antibody for adult patients with XLHR were recently published and the safety and effectiveness of this antibody was shown. This antibody therapy may be useful for patients with TIO with similar pathogenesis to that of XLHR.

CARCINOID TUMOR OF THE DUODENUM: a rare tumor at an unusual site. Case series from a single institution

Directory of Open Access Journals (Sweden)

Jaques WAISBERG

2013-03-01

Full Text Available Context Duodenal carcinoids are extremely rare, and their characteristics and biological behavior have not been fully elucidated. Objective To analyze the clinicopathological characteristics of patients with resected duodenal carcinoids. Methods Twenty patients (12 females and 8 males were investigated. Their average age was 66.4 ± 5.8 years old (43 to 88 years old. The data corresponding to the clinical picture, diagnosis, treatment, and prognosis of patients with duodenal carcinoid tumors subjected to resection over a period of 18 years (1993-2011 were analyzed. Results The most common symptoms were dyspepsia (50% and epigastric pain (45% followed by weight loss (10% and vomiting (5%. Carcinoid syndrome was not observed in any patient. The lesion was located on the first part of the duodenum in 15 (75% patients, the second part in 4 (20% patients, and the third part in 1 (5% patient. The diagnosis of a carcinoid tumor was established through an endoscopic excision biopsy in 19 (95% patients and an histopathological examination of the surgical specimen in 1 (5% patient. The average tumor size was 1.1 cm ± 0.4 cm (0.3 cm to 6.0 cm. Nineteen (95% patients were initially treated by endoscopic resection of the duodenal lesion. One patient (5%, whose tumor was on the third part of the duodenum underwent a duodenectomy of the third and fourth duodenal parts and duodenojejunal anastomosis. The duodenal carcinoid resection margin was involved in four (20% patients. Four (20% patients were subjected to a partial gastrectomy to fully remove the lesion. The tumor was restricted to the submucosal layer in 16 (80% cases, and it penetrated into the muscular layer in 4 (20% cases. All patients exhibited positive chromogranin A, neuron-specific enolase, and/or synaptophysin immunostaining. The average duration of the follow-up period was 39.6 months (3 to 96 months. Twelve (60% of the 20 cases in this series are alive without any evidence of active

Echocardiographic and non-gated computed tomographic findings of intrapericardial tumor and mediastinal tumor adjacent to the heart

International Nuclear Information System (INIS)

Ueda, Minoru; Yamada, Nobuyuki; Saito, Daiji; Haraoka, Shoichi; Tanetani, Setsuro.

1981-01-01

Echocardiographic and computed tomographic findings of a case of intrapericardial tumor are reported, and two other cases of mediastinal tumor are presented in a discussion of the differential diagnosis of intrapericardial from mediastinal tumors. Case report: A 7-year-old male complained of cough and dyspnea. Cardiomegaly had been pointed out at a mass X-ray examination about a month prior to the admission. Two-dimensional echocardiography revealed a massive anterior pericardial effusion and a fist-sized tumor with cystic structure. The tumor pushed the heart backward at the level of the aortic root. Non-gated computed tomography of the chest disclosed the size and location of the tumor, but failed to clarify the internal structure. The patient underwent successful removal of a tumor, 12 x 10 x 8 cm in size and 350 g in weight, originating from the left atrial wall. Histologically, the tumor was a fibrosarcomatous mesothelioma. Usually, an intrapericardial tumor is easily suspected by echocardiography by the presence of pericardial effusion, although there have been a few reports of intrapericardial tumors without pericardial effusion. Echocardiographic diagnosis of the intrapericardial tumor is difficult in such cases. Identification of the pericardium is necessary to diagnose whether a tumor is intra- or extrapericardial. This identification, however, is not always easy by echocardiography when the ultrasonic beams become tangent to the pericardium. The pericardium between the tumor and the heart could not be identified by echocardiography in our two cases of mediastinal tumor. Computed tomography is helpful in diagnosing the size and location of a mediastinal tumor. (author)

A Case of Malignant Peripheral Nerve Sheath Tumor with Rhabdomyoblastic Differentiation: Malignant Triton Tumor

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Kenichiro Mae

2013-12-01

Full Text Available Malignant peripheral nerve sheath tumors (MPNST constitute a rare variety of soft tissue sarcomas thought to originate from Schwann cells or pluripotent cells of the neural crest. Malignant triton tumor (MTT, a very rare, highly aggressive soft tissue tumor, is a subgroup of MPNST and is comprised of malignant Schwann cells coexisting with malignant rhabdomyoblasts. We herein report the case of a 24-year-old man who presented a subcutaneous mass in his right thigh. The mass was removed surgically in its entirety and radiation therapy was applied locally to prevent tumor regrowth. Nonetheless, the patient died 10 months after surgery from metastases to the lung and brain. He presented neither cafe-au-lait spots nor cutaneous neurofibromas. The histopathology showed a transition from a neurofibroma to an MTT, making this the second report of an MTT arising from a neurofibroma without neurofibromatosis type 1, an autosomal dominant disorder with which 50-70% of tumors reported in previous studies were associated. A histopathological examination using immunostaining with desmin confirmed this diagnosis. MTT has a poorer prognosis than MPNST and should therefore be regarded as a distinct clinical entity.

Investigation of change of tumor optical properties after laser-induced plasmon-resonant photothermal treatment of transplanted tumors in rats

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Genin, Vadim D.; Genina, Elina A.; Bucharskaya, Alla B.; Tuchin, Valery V.; Khlebtsov, Nikolay G.; Terentyuk, Georgy S.; Bashkatov, Alexey N.

2018-04-01

The paper presents the investigation of change of tumor optical properties of the rat tumor doped by gold nanoparticles after laser-induced plasmon-resonant photothermal treatment. To obtain the model tumors the rats have been implanted by suspension of alveolar kidney cancer cells. An hour before the experiment the animals have been injected by the suspension of gold nanorods intratumorally. For irradiation a diode laser with wavelength 808 nm has been used. After the irradiation the tumor has been removed and sliced. Spectra of total and collimated transmission and diffuse reflectance of the samples of different layers of the tumors have been measured in the wavelength range 350-2500 nm. Absorption, scattering, reduced scattering coefficients and scattering anisotropy factor of tumor tissues have been calculated with inverse adding-doubling method. The results of the experiment have shown that after doping the tumor tissue by the plasmon resonant nanoparticles and NIR laser irradiating, there is the decreases of absorption as well as scattering properties of the tumor and surrounding tissues. However, despite the sufficiently high temperature on the surface (about 80°C), the changes in the center of the tumor are insignificant.

Tumoral calcinosis with vitamin D deficiency

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Kannan Subramanian

2008-01-01

Full Text Available A 50-year-old woman presented with recurrent calcified mass in the left gluteal region. The clinical, radiological, and biochemical profile confirmed the diagnosis of tumoral calcinosis. She also had associated vitamin D deficiency. The patient underwent surgical removal of the mass to relieve the sciatic nerve compression and was managed with acetazolamide, calcium carbonate, and aluminium hydroxide gel with which she showed significant improve-ment. The management implications and effect of vitamin D deficiency on phosphate metabolism in the setting of tumoral calcinosis is discussed.

Bone alloplasty and rehabilitation of children with maxillo-facial tumors

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Zhelezny, P. A.; Sadovoy, M. A.; Kirilova, I. A.; Zhelezny, S. P.; Podorozhnaya, B. T.; Zheleznaya, A. P.

2017-09-01

The clinical observations in the treatment and rehabilitation of 117 children with maxillofacial tumors are presented. Malignant tumors were observed in 4 patients, other 113 children had benign tumors and tumor mass. Different bone defects of maxilla of both sub-total perforating and small segmental cavity appeared after the removal of neoplasms. The orthopedic transplants from the laboratory of tissue preservation of Tsivyan Novosibirsk Research Institute of Traumatology and Orthopedics preserved by different methods were used for maxilla defects restoration. Frozen transplants were applied in 48 patients, "Kostma" transplants were used in 14 patients, "Deprodex"—in 28 patients, "Orgamax"—in 27 patients. Orthopedic transplants from mandibular bone were used for chin and condylar process defects restoration. The orthopedic and orthodontic rehabilitation of the patients with the use of removable and unremovable orthodontic equipment and dental implantation systems was carried out in the postoperative period. Good anatomical functional and esthetic results of rehabilitation were received in 92 patients (89.3%) on long dates by 10 years. In some people the face asymmetry, bite disturbance, reduction of masticatory function were registered.

Local recurrence of metastatic brain tumor after surgery

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Shinoura, Nobusada; Yamada, Ryoji; Okamoto, Koichiro; Nakamura, Osamu; Shitara, Nobuyuki; Karasawa, Katsuyuki

2006-01-01

We analyzed factors associated with the local recurrence of brain metastases after surgery. Forty-seven patients with 67 metastatic brain tumors underwent surgery between 1994 and 2001. The survival time in the ''no recurrence'' group (34.7 months) was significantly longer than that in the recurrence group (21.9 months) (p=0.0008; log rank test). The factors affecting the local recurrence of brain metastases after surgery were as follows: cyst (p=0.0156), dural invasion (p=0.0029) of tumors, failure to totally remove tumors (p=0.0040), and lack of post-surgical irradiation (p<0.0001). Sex, age, tumor histology, tumor size, pre-surgical radiation, dose (≥45 vs <45, ≥50 vs <50 Gy) and the method (local vs whole brain) of post-surgical radiation did not affect the local recurrence rate of brain metastases after surgery. To avoid early recurrences of metastatic brain tumors, the factors associated with local recurrence should be considered in providing optimal treatment of tumors by surgery. (author)

A Percutaneous Transtubular Middle Fossa Approach for Intracanalicular Tumors.

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Bernardo, Antonio; Evins, Alexander I; Tsiouris, Apostolos J; Stieg, Philip E

2015-07-01

In cases of small intracanalicular tumors (≤ 1.5 cm), the middle fossa approach (MFA) provides the ability for adequate tumor removal with preservation of existing auditory function. Application of a minimally invasive tubular retractor in this approach may help mitigate the risk of postoperative seizures, aphasia, and venous complications by minimizing intraoperative retraction of the temporal lobe. We propose a minimally invasive microscopic and/or endoscopic percutaneous transtubular MFA for the management of intracanalicular tumors. Subtemporal keyhole craniectomies were performed on 5 preserved cadaveric heads (10 sides), with 6 sides previously injected with a synthetic tumor model. A ViewSite Brain Access System tubular retractor (Vycor Medical, Inc., Boca Raton, Florida, USA) was used to provide minimal temporal retraction and protection of the surrounding anatomy. An extradural dissection of the internal auditory canal was performed under microscopic and endoscopic visualization with a minimally invasive surgical drill and tube shaft instruments, the intracanalicular tumors were removed, and degree of resection was assessed. All 10 approaches were completed successfully through the tubular retractor with minimal retraction of the temporal lobe. Excellent visualization of the structures within the internal auditory canal was achieved with both the microscope and 3-dimensional endoscope. On the 6 synthetic intracanalicular tumors resected, 5 gross total (Grade I) and 1 near total (Grade II) resections were achieved. A percutaneous transtubular MFA is a feasible minimally invasive option for resection of small intracanalicular tumors with potential preservation of auditory function, reduced temporal retraction, and enhanced protection of surrounding structures. Copyright © 2015 Elsevier Inc. All rights reserved.

Intra-operative Ultrasound as a Tool to Assess Free Borders of Primary Vascular Aortic Tumors During Resection

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R.M. Andersen

Full Text Available : Introduction: Primary vascular tumors are rare and, in general, have a poor prognosis. Complete resection is associated with a better prognosis. Radical resection depends on safe discrimination of tumor borders. Technical summary: A 54 year old woman presented with abdominal pain. Imaging revealed a mass in the thoracic aorta, highly suspicious of angiosarcoma which was confirmed post-operatively by histological analysis. Open surgery was performed. Prior to clamping of the aorta, intra-operative ultrasound established clear delineation of the tumor borders. Conclusion: Intra-operative ultrasound was, in this case, a safe and easy method to determine the tumor borders, providing a simple guide to in toto tumor removal. Keywords: Angiosarcoma, Intra-operative ultrasound, In toto tumor removal, Fludeoxyglucose positron emission tomography/computed tomography, Magnetic resonance imaging

Electrochemical lysis at the stage of endoresection for large posterior intraocular tumors

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Yu. A. Belyy

2012-01-01

Full Text Available Purpose: to design the new combined technique of endoresection with intraoperative intraocular electrochemical lysis at the tumor destruction stage for large posterior intraocular tumors.Methods: 3 patients (3 eyes with large choroidal melanomas t3N0M0 (tumor thickness — 8-10 mm, base diameter — 13-15 mm, juxtapapillary localization. Mean age was 55.4 years old. Endoresection with intraoperational intraocular electrochemical lysis of the tumor was performed. Electrochemical lysis was performed with use of the technical unit ECU 300 (Soering, Germany and the original method of combined intratumoral positioning of two platinum electrodes: anode and cathode.Results: the tumor was removal completely in all 3 cases. the anatomical retinal reattachment was reached in all patients. Sclera was safe in all 3 cases. Visual acuity was not changed (NLP. At the place of the removal tumor a surgical choroidal coloboma without pigmentation all over scleral bed and periphery was shown in all cases in distant postoperative period (from 1.5 to 3 years. No local recurrences or metastasis were revealed in all patients.Conclusion: Further investigations in clinical group are necessarily to determinate the real possibilities of the combined method and the indications for endoresection with intraoperative intraocular electrochemical lysis for large intraocular tumors. 

Wilms' tumour: a comparison of surgical aspects in patients with or without pre-operative chemotherapy

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Safdar, C.A.; Aslam, M.; Awan, S.H.; Ahmed, I.; Badshah, S.

2006-01-01

To compare the technical aspects of Wilms' tumour (WT) surgery in patients with and without pre-operative chemotherapy. Patients of WT, presenting between January 1999 and December 2001, were treated, using the NWTSG protocol, with primary surgery (group I). Between January 2001 and December 2004, WT patients were treated according to SIOP protocol, with pre-operative chemotherapy followed by surgery (group II). Volume reduction with chemotherapy, duration of surgery, rupture of tumour, extent of excision, adherence and damage to surrounding structures, blood loss, complications, stay in hospital and event-free survival (EFS) were compared in the two groups. Out of 22 patients in group I, 19 (86.4%) underwent primary surgery. Of the 23 patients in group II, 21 (91.3%) received pre-operative chemotherapy followed by surgery. Average volume reduction in this group was 54% with chemotherapy. Difference in duration of surgery and blood loss was significantly low in group II (p=0.003 and p<0.001, respectively). In group I, rupture (6 vs 2), adherence (14 vs 10) and damage to surrounding structures (5 vs 2) were more. Complete macroscopic excision was possible in 90.5% of WT in group II as compared to 73.7% in group I. Immediate postoperative complications and length of hospital stay were similar in both groups. There was no difference in EFS. (author)

Application of argon-helium cryoablation in resection of intracranial tumors

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Yu-hao ZHOU

2017-07-01

Full Text Available Objective To summarize the curative effect of argon-helium cryoablation in resection of intracranial tumors.  Methods and Results A total of 11 patients with primary intracranial tumors, including 7 cases of glioma and 4 cases of meningioma, were enrolled in this study. The tumor was located in left frontal lobe in 4 cases, left fronto-parietal lobe in 2 cases, left temporal lobe in 2 cases and right temporo-parietal lobe in 3 cases. Argon-helium cryoablation was used to assist intracranial tumor resection. Among 7 cases of glioma, 4 cases were totally removed and 3 cases were partially resected. Four cases of meningioma were totally removed. The average intraoperative blood loss was 80 ml, and average operation time was 80 min. Postoperative clinical symptoms were improved, and head CT or MRI showed no rebleeding. Patients were followed up for an average of 4 years, and none of them suffered from operation-related or postoperative complications such as intracranial infection, or tumor recurrence.  Conclusions Argon - helium cryoablation is suitable for intracranial tumors with different diameters and in different locations. It is safe and effective, with few operation-related or postoperative complications, less rebleeding and low risk of recurrence, which is a highly efficient and relatively low?cost assistant surgical method. DOI: 10.3969/j.issn.1672-6731.2017.06.011

Overexpression of Wilms Tumor 1 Gene as a Negative Prognostic Indicator in Acute Myeloid Leukemia

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Mi, Ruihua; Ding, Jing; Wang, Xianwei; Hu, Jieying; Fan, Ruihua; Wei, Xudong; Song, Yongping; Zhao, Richard Y.

2014-01-01

Chromosomal aberrations are useful in assessing treatment options and clinical outcomes of acute myeloid leukemia (AML) patients. However, 40∼50% of the AML patients showed no chromosomal abnormalities, i.e., with normal cytogenetics aka the CN-AML patients. Testing of molecular aberrations such as FLT3 or NPM1 can help to define clinical outcomes in the CN-AML patients but with various successes. Goal of this study was to test the possibility of Wilms’ tumor 1 (WT1) gene overexpression as an additional molecular biomarker. A total of 103 CN-AML patients, among which 28% had overexpressed WT1, were studied over a period of 38 months. Patient’s response to induction chemotherapy as measured by the complete remission (CR) rate, disease-free survival (DFS) and overall survival (OS) were measured. Our data suggested that WT1 overexpression correlated negatively with the CR rate, DFS and OS. Consistent with previous reports, CN-AML patients can be divided into three different risk subgroups based on the status of known molecular abnormalities, i.e., the favorable (NPM1mt/no FLT3ITD), the unfavorable (FLT3ITD) and the intermediate risk subgroups. The WT1 overexpression significantly reduced the CR, DFS and OS in both the favorable and unfavorable groups. As the results, patients with normal WT1 gene expression in the favorable risk group showed the best clinical outcomes and all survived with complete remission and disease-free survival over the 37 month study period; in contrast, patients with WT1 overexpression in the unfavorable risk group displayed the worst clinical outcomes. WT1 overexpression by itself is an independent and negative indicator for predicting CR rate, DFS and OS of the CN-AML patients; moreover, it increases the statistical power of predicting the same clinical outcomes when it is combined with the NPM1 mt or the FLT3 ITD genotypes that are the good or poor prognostic markers of CN-AML. PMID:24667279

Pancreatoblastoma, a Rare Childhood Tumor: A Case Report

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Asuman ARGON

2017-05-01

Full Text Available Pancreatoblastoma, rarely encountered in the literature, is a malignant exocrine tumor seen in the pancreas. A 5-year-old boy suffering from abdominal pain was sent to our institute for further examination and treatment. Clinical examination was normal but for a palpable abdominal tumor mass. Abdominal Doppler ultrasonography showed a mass with well-defined margins within the body of the pancreas. Laboratory tests, including lactic dehydrogenase, alpha-fetoprotein and cancer antigen 125 were abnormal. The tumor invading the splenic vein and transverse colon was removed totally. We observed a hypercellular tumor in histopathological examination. The tumor had epithelial acinar cells and squamoid morules (corpuscles separated by stromal bands. Adjuvant chemotherapy was used after surgery. However, the patient died 14 months later. All data about pancreatoblastoma have to be collected in order to choose the treatment to elucidate the molecular pathogenesis of the tumor, to diagnose it early and to develop target-specific treatments.

Dying and regeneration of human tumor cells after heterotransplantation to athymic mice

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Köpf-Maler, P.

1986-01-01

The histologic phenomena occurring immediately after heterotransplantation of two human colon adenocarcinomas to athymic mice have been studied. The tumors differed with respect to velocity of growth and passage age. Three phases were discernible in both cases. (1) During the first phase, most inoculated tumor cells died. (2) The second phase was characterized by removal of the necrotic tumor cells by immigrated inflammatory cells and by penetration of the conn...

Role of scrape cytology in the intraoperative diagnosis of tumor

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Kolte Sachin

2010-01-01

Full Text Available Background : Rapid diagnosis of surgically removed specimens has created many controversies and a single completely reliable method has not yet been developed. Histopathology of a paraffin section remains the ultimate gold standard in tissue diagnosis. Frozen section is routinely used by the surgical pathology laboratories for intraoperative diagnosis. The use of either frozen section or cytological examination alone has an acceptable rate (93-97% of correct diagnosis, with regard to interpretation of benign versus malignant. Aim : To evaluate the utility of scrape cytology for the rapid diagnosis of surgically removed tumors and its utilisation for learning cytopathology. Materials and Methods : 75 surgically removed specimens from various organs and systems were studied. Scrapings were taken from each specimen before formalin fixation and stained by modified rapid Papanicolaou staining. Results : Of the 75 cases studied, 73 could be correctly differentiated into benign and malignant tumors, with an accuracy rate of 97.3%. Conclusions : Intraoperative scrape cytology is useful for intraoperative diagnosis of tumor, where facilities for frozen section are not available. The skill and expertise developed by routinely practicing intraoperative cytology can be applied to the interpretation of fine needle aspirate smears. Thus, apart from its diagnostic role, intraoperative cytology can become a very useful learning tool in the field of cytopathology.

A Carcinoid Tumor of the Appendix in a Child: A Case Report

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Laleh Vahedi Larijani

2018-01-01

Full Text Available The appendix is one of the most common sites for carcinoid tumors. Most carcinoids are found in appendices removed incidentally at laparotomy for conditions unrelated to acute appendicitis. We describe the case of a 13-year-old female who presented with abdominal pain in the right lower quadrant (RLQ, with nausea and decreased appetite for the previous 2 days. A physical examination favoreda diagnosis of acute appendicitis. A carcinoid tumor was diagnosed based on the histological examination of the removed appendix. The patient underwent an isolated appendectomy due to the small size of the lesion.

The results of gamma knife radiosurgery for malignant skull base tumors

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Tanaka, Takayuki; Kobayashi, Tatsuya; Kida, Yoshihisa; Oyama, Hirofumi; Niwa, Masahiro [Komaki City Hospital, Aichi (Japan)

1996-03-01

The results of gamma knife radiosurgery for malignant skull base tumors were analyzed using repeated magnetic resonance imagings and neurological examinations. Nineteen malignant skull base tumors were treated and followed up for 22.3 months (5-40 months) using MR imagings. The mean age was 54.4 years old (ranging from 16-85). Ten were male and 9 were female. Prior to the radiosurgery, removal of the tumors in 17 cases, conventional radiation therapy in 7, and chemotherapy in 4 etc. were performed. The pathological diagnoses were chordoma in 6 patients, metastatic tumors in 5, epipharyngeal carcinoma in 2, adenoid cystic carcinoma in 2, and others in 4. The locations of tumors were clivus in 8, parasellar region in 5, epipharynx in 2, paranasal sinus in 2, C-P angle in 1, and intraorbital region in 1 (14 intracranial and 5 extracranial). The mean diameter of the tumor was 33.5 mm. The mean maximum dose was 26.8 Gy and the mean marginal dose was 12.9 Gy during treatment. Repeated MR imagings revealed decrease of tumor size in 12 cases, showing no change in 1, and increase of tumor size in 5 (unknown in 1). Follow-up neurological examinations showed improvement in 3 patients, no change in 9, and deterioration in 7. There were 11 deaths during a mean follow-up period of 17.8 months (5-32 months) and another 8 cases are alive for a mean follow-up of 30.5 months (20-40 months) after the radiosurgery. Although the tumor size was large at the time of treatment, the results of gamma knife radiosurgery were promising. Considering the quality of life of patients with malignant skull base tumors, it is emphasized that gamma knife treatment is the method of choice compared with radical removal of the tumors. (author).

Early-postoperative magnetic resonance imaging in glial tumors: prediction of tumor regrowth and recurrence

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Ekinci, Gazanfer; Akpinar, Ihsan N. E-mail: i.akpinar@mailcity.com; Baltacioglu, Feyyaz; Erzen, Canan; Kilic, Tuerker; Elmaci, Ilhan; Pamir, Necmettin

2003-02-01

Objective: This study investigated the value of early-postoperative magnetic resonance (EPMR) imaging in the detection of residual glial tumor and investigated the role of EPMR for the prediction of tumor regrowth and recurrence. Methods and materials: We retrospectively analyzed pre- and post-operative magnetic resonance imaging results from 50 adult patients who underwent surgical treatment for supratentorial glial tumor. There were glioblastoma multiforme in 25 patients, astrocytoma (grades II and III) in 11 patients, oligodendroglioma (grades II and III) in 9 patients, and oligoastrocytoma (grades II and III) in 5 patients. EPMR imaging was performed within 24 h after surgery. EPMR findings were compared with the neurosurgeon's intraoperative estimation of gross tumor removal. Patterns of contrast enhancement at the resection site, in residual and developing tumor tissue and blood at the resection site were evaluated on EPMR and in follow-up studies. 'Residual tumor' was defined as contrast enhancing mass at the operative site on EPMR. 'Regrowth' was defined as contrast enhancing mass detected on follow-up in the same location as the primary tumor. 'Recurrence' was defined as appearance of a mass lesion in the brain parenchyma distant from the resection bed during follow-up. Results: Nineteen patients showed no evidence of residual tumor, regrowth, or recurrence on EPMR or any of the later follow-up radiological examinations. EPMR identified 20 cases of residual tumor. Follow-up showed tumor regrowth in 10 patients, and tumor recurrence in 1 case. EPMR showed contrast enhancement of the resection bed in 45 of the 50 patients. Four of the 20 residual tumors showed a thick linear enhancement pattern, and the other 16 cases exhibited thick linear-nodular enhancement. No thin linear enhancement was observed in the residual tumor group. Nine of the 10-regrowth tumors showed a thick linear-nodular enhancement pattern, and one

Primary tracheal adenocystic carcinoma and tracheal tumors during pregnancy

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Cem Gundogdu

2011-07-01

Full Text Available Cancer complicates approximately 0.1% of all pregnancies. Primary tracheal carcinoma is one of very rarely seen tumors and the rate of its being seen makes up approximately % 0,2 of all tumors of respiratory tract. The patient, 28 years old, who has 28-weeks-pregnant, was diagnosed with primary tracheal adenocystic carcinoma. Patient was made operation as thoracotomy and tracheal tumor was removed at the 28th week of pregnancy. Patient was delivered with sectio abdominale at the 39th week of pregnancy. Primary tracheal adenocystic carcinoma is very rarely seen tumors and it is the first tracheal ACC with pregnancy case in literature to have been detected and surgically treated during pregnancy. We discussed primary tracheal adenocystic carcinoma and tracheal tumors during pregnancy with literature.

Tumor-induced osteomalacia

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Pablo Florenzano

2017-12-01

Full Text Available Tumor-induced osteomalacia (TIO is a rare paraneoplastic syndrome clinically characterized by bone pain, fractures and muscle weakness. It is caused by tumoral overproduction of fibroblast growth factor 23 (FGF23 that acts primarily at the proximal renal tubule, decreasing phosphate reabsorption and 1α-hydroxylation of 25 hydroxyvitamin D, thus producing hypophosphatemia and osteomalacia. Lesions are typically small, benign mesenchymal tumors that may be found in bone or soft tissue, anywhere in the body. In up to 60% of these tumors, a fibronectin-1(FN1 and fibroblast growth factor receptor-1 (FGFR1 fusion gene has been identified that may serve as a tumoral driver. The diagnosis is established by the finding of acquired chronic hypophosphatemia due to isolated renal phosphate wasting with concomitant elevated or inappropriately normal blood levels of FGF23 and decreased or inappropriately normal 1,25-OH2-Vitamin D (1,25(OH2D. Locating the tumor is critical, as complete removal is curative. For this purpose, a step-wise approach is recommended, starting with a thorough medical history and physical examination, followed by functional imaging. Suspicious lesions should be confirmed by anatomical imaging, and if needed, selective venous sampling with measurement of FGF23. If the tumor is not localized, or surgical resection is not possible, medical therapy with phosphate and active vitamin D is usually successful in healing the osteomalacia and reducing symptoms. However, compliance is often poor due to the frequent dosing regimen and side effects. Furthermore, careful monitoring is needed to avoid complications such us secondary/tertiary hyperparathyroidism, hypercalciuria, and nephrocalcinosis. Novel therapeutical approaches are being developed for TIO patients, such as image-guided tumor ablation and medical treatment with the anti-FGF23 monoclonal antibody KRN23 or anti FGFR medications. The case of a patient with TIO is presented to

Effects of therapeutic irradiation delivered in early childhood upon subsequent lung function

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Wohl, M.E.B.; Griscom, N.T.; Graggis, D.G.; Jaffe, N.

1975-01-01

To determine the long-term effects of therapeutic pulmonary irradiation and treatment with actinomycin D during a period of lung growth, 12 patients treated for Wilms' tumor metastatic to the lung and 8 patients treated for Wilms' tumor with no evidence of pulmonary metastases were studied 7 to 14 years after their initial tumor therapy. All patients had received irradiation to the tumor bed and treatment with actinomycin D. Group 1 had received a single course of bilateral pulmonary irradiation; group 2 had received additional pulmonary irradiation and/or thoracic surgery; group 3 had received no therapeutic irradiation directed primarily to the chest. Total lung capacity (TLC) averaged 71 percent of predicted value in group 1, 58 percent in group 2, and 94 percent in group 3. Diffusing capacity in groups 1 and 2 was reduced to the same extent as lung volume. Quasi-static pressure-volume relationships, studied in three of six patients in group 1, were within the normal range when lung volume was expressed as percentage of observed TLC. Airway resistance, evaluated by spirometry, maximum expiratory flow-volume curves, and resistance of the total respiratory system, was normal or reduced. The data support the hypothesis that therapeutic irradiation during a period of lung growth primarily affects the lung parenchyma and produces a decrease in subsequent size of both the lung and chest wall. No effect of actinomycin D alone upon the lung could be demonstrated

Neurophysiological Identification of Cranial Nerves During Endoscopic Endonasal Surgery of Skull Base Tumors: Pilot Study Technical Report.

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Shkarubo, Alexey Nikolaevich; Chernov, Ilia Valerievich; Ogurtsova, Anna Anatolievna; Moshchev, Dmitry Aleksandrovich; Lubnin, Andrew Jurievich; Andreev, Dmitry Nicolaevich; Koval, Konstantin Vladimirovich

2017-02-01

Intraoperative identification of cranial nerves is crucial for safe surgery of skull base tumors. Currently, only a small number of published papers describe the technique of trigger electromyography (t-EMG) in endoscopic endonasal removal of such tumors. To assess the effectiveness of t-EMG in preventing intraoperative cranial nerve damage in endoscopic endonasal surgery of skull base tumors. Nine patients were operated on using the endoscopic endonasal approach within a 1-year period. The tumors included large skull base chordomas and trigeminal neurinomas localized in the cavernous sinus. During the surgical process, cranial nerve identification was carried out using monopolar and bipolar t-EMG methods. Assessment of cranial nerve functional activity was conducted both before and after tumor removal. We mapped 17 nerves in 9 patients. Third, fifth, and sixth cranial nerves were identified intraoperatively. There were no cases of postoperative functional impairment of the mapped cranial nerves. In one case we were unable to get an intraoperative response from the fourth cranial nerve and observed its postoperative transient plegia (the function was normal before surgery). t-EMG allows surgeons to control the safety of cranial nerves both during and after skull base tumor removal. Copyright © 2016 Elsevier Inc. All rights reserved.

The capabilities of simultaneous use of SPECT and MRI findings at removal of brain tumors using neuronavigation

International Nuclear Information System (INIS)

Makejev, S.S.; Rozumenko, V.D.; Rozumenko, A.V.; Chuvashova, O.Yu.

2010-01-01

The purpose of the work was to determine the capabilities of SPECT and MRI at intra-operative determining of interrelation of the tumor and FIA. MRI revealed the peculiarities of the tumor structure, SPECT - highly proliferating tumors.

Co-administration of α-GalCer analog and TLR4 agonist induces robust CD8+ T-cell responses to PyCS protein and WT-1 antigen and activates memory-like effector NKT cells

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Coelho-dos-Reis, Jordana G.; Huang, Jing; Tsao, Tiffany; Pereira, Felipe V.; Funakoshi, Ryota; Nakajima, Hiroko; Sugiyama, Haruo; Tsuji, Moriya

2016-01-01

In the present study, the combined adjuvant effect of 7DW8-5, a potent α-GalCer-analog, and monophosphoryl lipid A (MPLA), a TLR4 agonist, on the induction of vaccine-induced CD8+ T-cell responses and protective immunity was evaluated. Mice were immunized with peptides corresponding to the CD8+ T-cell epitopes of a malaria antigen, a circumsporozoite protein of Plasmodium yoelii, and a tumor antigen, a Wilms Tumor antigen-1 (WT-1), together with 7DW8-5 and MPLA, as an adjuvant. These immuniza...

Metanephric Adenofibroma associated with Papillary Renal CeU Carcinoma

International Nuclear Information System (INIS)

Roa, Carmen Lucia B; Navarrete, Maria Constanza

2008-01-01

Metanephric adenofibroma is an infrequent biphasic epithelial-stromal renal tumor, occasionally associated with papillary renal cell carcinoma. We describe a case of a girl with a four year clinical history of intermittent hematuria; she was diagnosed, using a left-side tru-cut renal biopsy, with a Wilms' tumor with stromal and epithelial component, with no sign of anaplasia. Later, through the product of the left-side nephrectomy that was performed at the National Cancer institute of Colombia, she was diagnosed with metanephric adenofibroma associated with papillary renal cell carcinoma

Skeletal metastases of carcinomas of prostate in dependence on tumor size and tumor differentiation

International Nuclear Information System (INIS)

Krause, U.

1981-01-01

153 patients with carcinoma of the prostate underwent holebody skeletal scintiscanning. It resulted that the tendency to the development of skeletal metastases increases with increasing dedifferentiation of the tumor. Also the tumor size correlated with the metastase identification. The tumor dedifferentiation also increased with the tumor size. The findings proved that the early diagnosis of a carcinoma of the prostate is a necessary prerequisite, because a radical total removal can only be curative when any metastases are absent. The comparative evaluation of the diagnostic methods proved the superiority of the nuclear medical examination. In 68% of the cases the roentgenologic examination led to correctly positive results. This investigation showed with 98% a high diagnostic specificity and therefore it should be applied in addition to scintiscanning in order to obtain supplementary information. The alkaline and the acid phosphatase offering an almost identical informative value resulted to be not useful for establishing an early diagnosis of skeletal metastases. It was found that the determination of the blood sedimentation rate and of the lactate dehydrogenase do also not render possible the early diagnosis of skeletal metastases. (orig./MG) [de

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.

Development of an acellular tumor extracellular matrix as a three-dimensional scaffold for tumor engineering.

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Wei-Dong Lü

Full Text Available Tumor engineering is defined as the construction of three-dimensional (3D tumors in vitro with tissue engineering approaches. The present 3D scaffolds for tumor engineering have several limitations in terms of structure and function. To get an ideal 3D scaffold for tumor culture, A549 human pulmonary adenocarcinoma cells were implanted into immunodeficient mice to establish xenotransplatation models. Tumors were retrieved at 30-day implantation and sliced into sheets. They were subsequently decellularized by four procedures. Two decellularization methods, Tris-Trypsin-Triton multi-step treatment and sodium dodecyl sulfate (SDS treatment, achieved complete cellular removal and thus were chosen for evaluation of histological and biochemical properties. Native tumor tissues were used as controls. Human breast cancer MCF-7 cells were cultured onto the two 3D scaffolds for further cell growth and growth factor secretion investigations, with the two-dimensional (2D culture and cells cultured onto the Matrigel scaffolds used as controls. Results showed that Tris-Trypsin-Triton multi-step treated tumor sheets had well-preserved extracellular matrix structures and components. Their porosity was increased but elastic modulus was decreased compared with the native tumor samples. They supported MCF-7 cell repopulation and proliferation, as well as expression of growth factors. When cultured within the Tris-Trypsin-Triton treated scaffold, A549 cells and human colorectal adenocarcinoma cells (SW-480 had similar behaviors to MCF-7 cells, but human esophageal squamous cell carcinoma cells (KYSE-510 had a relatively slow cell repopulation rate. This study provides evidence that Tris-Trypsin-Triton treated acellular tumor extracellular matrices are promising 3D scaffolds with ideal spatial arrangement, biomechanical properties and biocompatibility for improved modeling of 3D tumor microenvironments.

A novel method for sample preparation of fresh lung cancer tissue for proteomics analysis by tumor cell enrichment and removal of blood contaminants

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Orre Lotta

2010-02-01

Full Text Available Abstract Background In-depth proteomics analyses of tumors are frequently biased by the presence of blood components and stromal contamination, which leads to large experimental variation and decreases the proteome coverage. We have established a reproducible method to prepare freshly collected lung tumors for proteomics analysis, aiming at tumor cell enrichment and reduction of plasma protein contamination. We obtained enriched tumor-cell suspensions (ETS from six lung cancer cases (two adenocarcinomas, two squamous-cell carcinomas, two large-cell carcinomas and from two normal lung samples. The cell content of resulting ETS was evaluated with immunocytological stainings and compared with the histologic pattern of the original specimens. By means of a quantitative mass spectrometry-based method we evaluated the reproducibility of the sample preparation protocol and we assessed the proteome coverage by comparing lysates from ETS samples with the direct lysate of corresponding fresh-frozen samples. Results Cytological analyses on cytospin specimens showed that the percentage of tumoral cells in the ETS samples ranged from 20% to 70%. In the normal lung samples the percentage of epithelial cells was less then 10%. The reproducibility of the sample preparation protocol was very good, with coefficient of variation at the peptide level and at the protein level of 13% and 7%, respectively. Proteomics analysis led to the identification of a significantly higher number of proteins in the ETS samples than in the FF samples (244 vs 109, respectively. Albumin and hemoglobin were among the top 5 most abundant proteins identified in the FF samples, showing a high contamination with blood and plasma proteins, whereas ubiquitin and the mitochondrial ATP synthase 5A1 where among the top 5 most abundant proteins in the ETS samples. Conclusion The method is feasible and reproducible. We could obtain a fair enrichment of cells but the major benefit of the method

Recurrent malignant variant of phosphaturic mesenchymal tumor with oncogenic osteomalacia

International Nuclear Information System (INIS)

Ogose, A.; Hotta, Tetsuo; Hatano, Hiroshi; Endo, Naoto; Emura, Iwao; Umezu, Hajime; Inoue, Yoshiya

2001-01-01

Phosphaturic mesenchymal tumor is a rare neoplasm which causes osteomalacia or rickets. The tumor typically follows a benign clinical course. Even in the rare malignant cases, local recurrence and distant metastasis are uncommon. We report on an example of a malignant phosphaturic mesenchymal tumor which recurred several times over 16 years concurrently causing hypophosphatemia, bone pain, and osteomalacia. Following each surgery, symptoms and hypophosphatemia improved. The patient died of disease 17 years after the first surgery. Histologically, the initial tumor was composed of small spindle cells with clusters of giant cells, prominent blood vessels, poorly formed cartilaginous areas, and crystalline material. Cytological atypia was minimal. Following multiple recurrences, the tumor demonstrated areas of high-grade sarcoma exhibiting marked pleomorphism, numerous mitotic figures, and p53 overexpression. This case illustrates the potential lethality of incompletely removed phosphaturic mesenchymal tumors. (orig.)

Recurrent malignant variant of phosphaturic mesenchymal tumor with oncogenic osteomalacia

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Ogose, A.; Hotta, Tetsuo; Hatano, Hiroshi; Endo, Naoto [Dept. of Orthopedic Surgery, Niigata University School of Medicine, Asahimachi, Niigata (Japan); Emura, Iwao; Umezu, Hajime [Dept. of Pathology, Niigata University School of Medicine, Niigata (Japan); Inoue, Yoshiya [Dept. of Orthopedic Surgery, Seirei Hamamatsu General Hospital, Hamamatsu (Japan)

2001-02-01

Phosphaturic mesenchymal tumor is a rare neoplasm which causes osteomalacia or rickets. The tumor typically follows a benign clinical course. Even in the rare malignant cases, local recurrence and distant metastasis are uncommon. We report on an example of a malignant phosphaturic mesenchymal tumor which recurred several times over 16 years concurrently causing hypophosphatemia, bone pain, and osteomalacia. Following each surgery, symptoms and hypophosphatemia improved. The patient died of disease 17 years after the first surgery. Histologically, the initial tumor was composed of small spindle cells with clusters of giant cells, prominent blood vessels, poorly formed cartilaginous areas, and crystalline material. Cytological atypia was minimal. Following multiple recurrences, the tumor demonstrated areas of high-grade sarcoma exhibiting marked pleomorphism, numerous mitotic figures, and p53 overexpression. This case illustrates the potential lethality of incompletely removed phosphaturic mesenchymal tumors. (orig.)

Biopsy techniques for intraocular tumors

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Pukhraj Rishi

2016-01-01

Full Text Available Biopsy involves the surgical removal of a tissue specimen for histopathologic evaluation. Most intraocular tumors are reliably diagnosed based on the clinical evaluation or with noninvasive diagnostic techniques. However, accurately diagnosing a small percentage of tumors can be challenging. A tissue biopsy is thus needed to establish a definitive diagnosis and plan the requisite treatment. From fine-needle aspiration biopsy (FNAB to surgical excision, all tissue collection techniques have been studied in the literature. Each technique has its indications and limitations. FNAB has been reported to provide for 88-95% reliable and safe ophthalmic tumor diagnosis and has gained popularity for prognostic purposes and providing eye conserving treatment surgeries. The technique and instrumentation for biopsy vary depending upon the tissue involved (retina, choroid, subretinal space, vitreous, and aqueous, suspected diagnosis, size, location, associated retinal detachment, and clarity of the media. The cytopathologist confers a very important role in diagnosis and their assistance plays a key role in managing and planning the treatment for malignancies.

Virilization caused by an ectopic adrenal tumor located behind the iliopsoas muscle.

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Mavroudis, Konstantinos; Aloumanis, Kyriakos; Papapetrou, Peter D; Voros, Dionisios; Spanos, Iraklis

2007-06-01

Virilization due to androgen-secreting neoplasms in women is a result of androgen overproduction from benign or malignant tumors that are found in the ovaries or rarely in the adrenal glands. Virilizing tumors that arise from ectopic adrenal tissue are extremely rare. We describe a very rare case of an ectopic androgen-producing adrenal tumor. Case report study. Endocrinology outpatient department of university-affiliated teaching hospital. A 45-year-old woman with symptoms of virilization of abrupt onset and rapid progression, with high serum androgen hormone levels and normal glucocorticoid secretion. Basal hormonal levels, stimulation and suppression tests, imaging techniques, and selective venous sampling. Localization and surgical removal of the source of androgen production. An ectopic mass was detected behind the left iliopsoas muscle. The patient was operated on and an oblong-shaped lesion, weighing 6 g, was removed. Histologically, the tissue was identified to be of adrenal origin. Postoperatively the androgen levels decreased to normal levels. This case illustrates difficulties in detecting and localizing the rare contingence of an ectopic adrenocortical androgen-secreting tumor.

Surgical strategy for intra- and extra-vertebral dumbbell-shaped tumors

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SUN Li-yong

2013-12-01

Full Text Available Objective To investigate the clinical features and surgical strategy of intra- and extra-vertebral dumbbell-shaped tumors. Methods Clinical data of 39 patients with intra- and extra-vertebral tumor were retrospectively studied. The tumors were removed via posterior midline approach in 33 patients, and via posterior combined with anterior approach in 6 patients. Thirty patients underwent tumor resection and internal fixation. Lateral mass screw fixation was performed in the level of C3-7, while the pedicular screw fixation was performed in the level of C2 and thoracic and lumbar segment. Results Tumors were totally excised in all the cases. The patients were followed-up for 6 months to 5 years with an average of 18.67 months. Pain relief occured in 29 cases, of whom the average Visual Analogue Scale (VAS score decreased from (7.51 ± 1.05 before surgery to (3.17 ± 1.17 24 h after surgery (P < 0.05. The numbness area emerged or enlarged in 12 cases and was unchanged in 3 cases. The average American Spinal Injury Association (ASIA sensation score decreased from (218.67 ± 2.80 before surgery to (213.33 ± 2.16 24 h after surgery (P < 0.05, but it increased to (216.78 ± 1.47 6 months after operation (P < 0.05. The motor function improved in 18 cases, and ASIA motor function score improved from (92.33 ± 1.63 before surgery to (95.05 ± 1.41 6 months after operation (P < 0.05. No tumor recurrence and secondary spinal deformity were found. Conclusion Most cases of dumbbell-shaped intra- and extra-vertebral tumor can be totally removed with one-session microsurgery. In the cases with bony erosion caused by tumor and facetectomy, concurrent internal fixation and fusion were recommended in order to maintain spinal stability.

[Endoscopic assistance in surgery of cerebellopontine angle tumors].

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Poshataev, V K; Shimansky, V N; Tanyashin, S V; Karnaukhov, V V

2014-01-01

During the period of 2010-2012, 33 patients with cerebellopontine angle tumors were operated on at the Burdenko Neurosurgical Institute (Moscow, Russia) using different types of endoscopic assistance. All patients were operated on via the retrosigmoid suboccipital approach in semi-sitting and prone positions. 30° and 70° endoscopes were used during the surgery. Endoscopic assistance allowed us to increase the completeness of tumor removal and to reduce the risk of postoperative complications by retaining the anatomic integrity of cranial nerves and vascular structures in the base of the posterior cranial fossa. These benefits made it possible to maintain and improve quality of life in patients with CPA tumors in the postoperative period.

Nuclear magnetic resonance evaluation of fifty children with cancer

International Nuclear Information System (INIS)

Cohen, M.D.; Klatte, E.C.; Smith, J.A.; Carr, B.E.; Martin-Simmerman, P.

1985-01-01

Fifty children with cancer have been studied by MR. The patients studied include ten with lymphoma, nine with neuroblastoma, five with rhabdomyosarcoma, six with leukemia, five with Ewings sarcoma, four with Wilms tumor and several with other miscellaneous tumors. The results of scanning show that MR is well tolerated by children. Primary tumor has been identified in every case. Metastases have been identified in many patients. MR has proved helpful in identifying the organ of origin of a tumor. Because of excellent vessel visualization it is helpful in planning surgical resection of a tumor. In addition, in a number of patients MR has proved helpful in monitoring the response of tumor to nonsurgical therapy. With continued improvement in image quality it is believed that MR has a major role to play in pediatric tumor imaging [fr

Clinical significance of anaplasia in childhood rhabdomyosarcoma

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Sidhom, Iman; El Nadi, Enas; Taha, Hala; Elkinaai, Naglaa; Zaghloul, Mohamed S.; Younes, Alaa; Labib, Rania; Sabry, Mohamed

2015-01-01

Background: The presence of anaplastic features has been known to correlate with poor clinical outcome in various pediatric malignancies, including Wilms tumor and medulloblastoma but not in rhabdomyosarcoma. Aim: Aim was to study the frequency of anaplasia at presentation in childhood rhabdomyosarcoma and its relationship to clinical and pathological characteristics as well as to outcome. Patients and Methods: Anaplasia was retrospectively assessed in 105 consecutive pediatric rhabdomy...

Contaminação tumoral em trajeto de biópsia de tumores ósseos malignos primários Tumor contamination in the biopsy path of primary malignant bone tumors

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Marcelo Parente Oliveira

2012-10-01

chondrosarcoma were studied retrospectively. The sample was analyzed to characterize the biopsy technique used, histological type of the tumor, neoadjuvant chemotherapy used, local recurrences and tumor contamination in the biopsy path. RESULTS: Among the 35 patients studied, four cases of contamination occurred (11.43%: one from osteosarcoma, two from Ewing's tumor and one from chondrosarcoma. There was no association between the type of tumor and presence of tumor contamination in the biopsy path (p = 0.65. There was also no association between the presence of tumor contamination and the biopsy technique (p = 0.06. On the other hand, there were associations between the presence of tumor contamination and local recurrence (p = 0.01 and between tumor contamination and absence of neoadjuvant chemotherapy (p = 0.02. CONCLUSION: Tumor contamination in the biopsy path of primary malignant bone tumors was associated with local recurrence. On the other hand, the histological type of the tumor and the type of biopsy did not have an influence on tumor contamination. Neoadjuvant chemotherapy had a protective effect against this complication. Despite these findings, tumor contamination is a complication that should always be taken into consideration, and removal of the biopsy path is recommended in tumor resection surgery.

A Case Report of Primary Cardiac Tumor in A Neonate

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Sh. Rejaei

2008-04-01

Full Text Available Introduction: Primary cardiac tumors are extremely rare in infants and children . Most primary cardiac tumors in pediatric age group are benign, and less than 10% of such tumors are malignant. Many of these tumors are asymptomatic and incidentally diagnosed. The clinical manifestations are very different and includes direct cardiac effect, systemic effect , and embolic phenomena. Every infant or child with an unusual cardiac murmur, unexplained congestive heart failure, or arrhythmia should be evaluated for cardiac tumors. Echocardiography has contributed significantly to the evaluation of these patients. Surgery is the only treatment for primary cardiac tumors that require intervention with a relatively good prognosis. Case Report: The patient was a 20 days old neonate presented with severe congestive heart failure. Evaluation of the patient showed primary cardiac tumor in the left atrium and ventricle. We recommended surgical removal of the tumor but her parents denied surgical intervention at all. Conclusion: After about one year follow up, congestive heart failure symptoms were controlled and the tumor size was decreased.

Removal of vestibular schwannoma and facial nerve preservation using small suboccipital retrosigmoid craniotomy

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CHEN Ling; CHEN Li-hua; LING Feng; LIU Yun-sheng; Madjid Samii; Amir Samii

2010-01-01

Background Vestibular schwannoma, the commonest form of intracranial schwannoma, arises from the Schwann cells investing the vestibular nerve. At present, the surgery for vestibular schwannoma remains one of the most complicated operations demanding for surgical skills in neurosurgery. And the trend of minimal invasion should also be the major influence on the management of patients with vestibular schwannomas. We summarized the microsurgical removal experience in a recent series of vestibular schwannomas and presented the operative technique and cranial nerve preservation in order to improve the rates of total tumor removal and facial nerve preservation.Methods A retrospective analysis was performed in 145 patients over a 7-year period who suffered from vestibular schwannomas that had been microsurgicaily removed by suboccipital retrosigmoid transmeatus approach with small craniotomy. CT thinner scans revealed the tumor size in the internal auditory meatus and the relationship of the posterior wall of the internal acoustic meatus to the bone labyrinths preoperatively. Brain stem evoked potential was monitored intraoperatively. The posterior wall of the internal acoustic meatus was designedly drilled off. Patient records and operative reports, including data from the electrophysiological monitoring, follow-up audiometric examinations, and neuroradiological findings were analyzed.Results Total tumor resection was achieved in 140 cases (96.6%) and subtotal resection in 5 cases. The anatomical integrity of the facial nerve was preserved in 91.0% (132/145) of the cases. Intracranial end-to-end anastomosis of the facial nerve was performed in 7 cases. Functional preservation of the facial nerve was achieved in 115 patients (Grade Ⅰ and Grade Ⅱ, 79.3%). No patient died in this series. Preservation of nerves and vessels were as important as tumor removal dudng the operation. CT thinner scan could show the relationship between the posterior wall of the internal

Efficacy and biological effects of techniques used to induce hypoxia in tumors

International Nuclear Information System (INIS)

Rockwell, S.; Moulder, J.E.; Martin, D.F.

1985-01-01

The authors tested several aspects of the assumptions using BA1112 rat rhabdomyosacromas and EMT6 mouse mammary tumors. Both techniques were effective in producing hypoxia: survival curves for tumors made hypoxic by the 2 techniques were the same, and were indistinguishable from survival curves for hypoxic tumor cells in vitro. Induction of hypoxia for the 30-60 min necessary for irradiation did not alter the yield or clonogenicity of cells suspended from the tumors. Longer clamping was cytotoxic. Clamping did not alter tumor growth unless a major blood vessel was injured. However, clamping BA1112 tumors for 30 min and removing the clamp just before irradiation altered the tumor cell survival curve and TCD/sub 50/. Anesthesia or restrain, often used during clamping, can have major effects

Useful surgical techniques for facial nerve preservation in tumorous intra-temporal lesions.

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Kim, Jin; Moon, In Seok; Lee, Jong Dae; Shim, Dae Bo; Lee, Won-Sang

2010-02-01

The management of the facial nerve in tumorous temporal lesions is particularly challenging due to its complex anatomic location and potential postoperative complications, including permanent facial paralysis. The most important concern regarding surgical treatment of a tumorous temporal lesion is the inevitable facial paralysis caused by nerve injury during the tumor removal, especially in patients with minimal to no preoperative facial nerve dysfunction. We describe successful four cases in which various surgical techniques were developed for the preservation of the facial nerve in treatment of intratemporal tumorous lesions. Copyright (c) 2009 Elsevier Ireland Ltd. All rights reserved.

Tumor Penetrating Theranostic Nanoparticles for Enhancement of Targeted and Image-guided Drug Delivery into Peritoneal Tumors following Intraperitoneal Delivery.

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Gao, Ning; Bozeman, Erica N; Qian, Weiping; Wang, Liya; Chen, Hongyu; Lipowska, Malgorzata; Staley, Charles A; Wang, Y Andrew; Mao, Hui; Yang, Lily

2017-01-01

The major obstacles in intraperitoneal (i.p.) chemotherapy of peritoneal tumors are fast absorption of drugs into the blood circulation, local and systemic toxicities, inadequate drug penetration into large tumors, and drug resistance. Targeted theranostic nanoparticles offer an opportunity to enhance the efficacy of i.p. therapy by increasing intratumoral drug delivery to overcome resistance, mediating image-guided drug delivery, and reducing systemic toxicity. Herein we report that i.p. delivery of urokinase plasminogen activator receptor (uPAR) targeted magnetic iron oxide nanoparticles (IONPs) led to intratumoral accumulation of 17% of total injected nanoparticles in an orthotopic mouse pancreatic cancer model, which was three-fold higher compared with intravenous delivery. Targeted delivery of near infrared dye labeled IONPs into orthotopic tumors could be detected by non-invasive optical and magnetic resonance imaging. Histological analysis revealed that a high level of uPAR targeted, PEGylated IONPs efficiently penetrated into both the peripheral and central tumor areas in the primary tumor as well as peritoneal metastatic tumor. Improved theranostic IONP delivery into the tumor center was not mediated by nonspecific macrophage uptake and was independent from tumor blood vessel locations. Importantly, i.p. delivery of uPAR targeted theranostic IONPs carrying chemotherapeutics, cisplatin or doxorubicin, significantly inhibited the growth of pancreatic tumors without apparent systemic toxicity. The levels of proliferating tumor cells and tumor vessels in tumors treated with the above theranostic IONPs were also markedly decreased. The detection of strong optical signals in residual tumors following i.p. therapy suggested the feasibility of image-guided surgery to remove drug-resistant tumors. Therefore, our results support the translational development of i.p. delivery of uPAR-targeted theranostic IONPs for image-guided treatment of peritoneal tumors.

Retroperitoneal endodermal sinus tumor patient with palliative care needs

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Surbhi Kashyap

2016-01-01

Full Text Available This article is a case reflection of a personal encounter on the palliative care treatment required after the removal of a complicated case of a primary extra-gonadal retro-peritoneal endodermal sinus tumor (yolk sac tumor. This reflection is from the perspective of a recently graduated MD student who spent one month with an Indian pain management and palliative care team at the Institute Rotary Cancer Hospital (IRCH, All India Institute of Medical Sciences (AIIMS, New Delhi

The contribution of radiation therapy to the cancer in children

International Nuclear Information System (INIS)

Watari, Tsutomu

1981-01-01

Majority of patients with tumors generally are sensitive and very useful of radiotherapy. Major cases in pediatric cancer are leukemia, brain tumor, neuroblastoma, malignant lymphoma, Wilms tumor, retinoblastoma, rhabdomyo-sarcoma, osteosarcoma, testicular tumors et al. Others are hemangioma including Kasabach-Merritt Syndrome. Radiation therapy is indicated to all cases of malignant tumor in children. Recently the treatment results improving with the help of well organized chemotherapy. Cancer in children is almost a speciality on its own. Since the total number of patients in any region is not large, treatment is best concentrated at special oncology centers. Long-term results in the majority of cases are disappointing, and the nursing problems after formidable. Surgery, Radiation and Chemotherapy and all valuable, especially in combination - Multidisciplinary therapy and Multidisciplinary team. (author)

Radionecrosis after radiotherapy for brain tumor

International Nuclear Information System (INIS)

Yoshii, Yoshihiko; Maki, Yutaka; Tosa, Junichi; Tsuboi, Koji; Matsumura, Akira

1984-01-01

The neurological deterioration after radiotherapy of brain tumor may depend on radionecrosis or regrowth of the tumor. In the present study, five patients with brain tumor were irradiated with doses of 3,900 to 6,800 rads. The neurological deterioration appeared 3.5 to 46 months after radiotherapy in three patients, who received 5,000 to 5,680 rads, immediately after radiotherapy in one patient, who received 6,800 rads, and during radiotherapy in one patient, who received 3,900 rads. Ring enhancement was observed on sequential CT scans. This enhanced area was surgically removed and the correlation between histology and CT scans and superimposed dose distributions was studied in order to differentiate radionecrosis from regrowth of tumor. The radionecrosis was confirmed at the second operation in five patients, but regrowth of the tumor was also observed in the brain adjacent to radionecrosis in three out of five patients. Coagulation necrosis and fibrinoid necrosis were observed microscopically at the rim of the ring enhancement and necrotic and hyalinized debri were observed in the central low density area of the ring enhancement. Viable tumor cells were noted in the enhanced area adjacent to the ring enhancement on CT scans. Both radionecrosis and regrowth of tumor were observed in the dose distribution area of 3,500 to 6,120 rads on CT scans. This suggested that the superimposed dose distributions could not differentiate radionecrosis from tumor regrowth. Forty-eight cases of cerebral radionecrosis gathered from the literature were reviewed. (J.P.N.)

Microsurgery Resection of Intrinsic Insular Tumors via Transsylvian Surgical Approach in 12 Cases

International Nuclear Information System (INIS)

Wang, Peng; Wu, Ming-can; Chen, Shi-jie; Xu, Xian-ping; Yang, Yong; Cai, Jie

2012-01-01

To investigate the clinical characteristics, operative methods, and diffusion tensor imaging (DTI) in the resection of intrinsic insular gliomas via transsylvian approach. From June 2008 to June 2010, 12 patients with intrinsic insular gliomas were treated via transsylvian microsurgical approach, with preoperative magnetic resonance imaging diffusion tensor imaging (MR DTI) evaluation. The data of these patients were retrospectively analyzed. All patients had astrocytoma, including 8 patients of Grades I to II, 2 patients of Grades III to IV, and 2 patients of mixed glial tumors. The insular tumors were completely removed in 9 patients, whereas they were only partially removed from 3 patients. No death was related to the operations. Two patients had transient aphasia, 2 experienced worsened hemiplegia on opposite sides of their bodies, and 2 had mild hemiplegia and language function disturbance. Most of the insular gliomas are of low grade. By evaluating the damage of the corticospinal tract through DTI and using ultrasonography to locate the tumors during operation, microsurgery treatment removes the lesions as much as possible, protects the surrounding areas, reduces the mobility rate, and improves the postoperative quality of life

Application of infrared spectroscopy for diagnosis of kidney tumor tissue

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Bandzevičiūtė, Rimantė

2016-01-01

Application of Infrared Spectroscopy for Diagnosis of Kidney Tumor Tissue It is possible to apply the technique of an attenuated total reflection of infrared radiation (ATR IR) for the characterisation of the removed tissues during the surgery. Application of this method for interstitium of the removed tissue does not require any specific sample preparation. For this reason ATR IR technique applied for the interstitium allows to get information about tissues immediately after surgical operati...

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)

Recovery from diabetes insipidus and preservation of thyroid function after craniopharyngioma removal and pituitary stalk sectioning.

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Ogawa, Yoshikazu; Niizuma, Kuniyasu; Tominaga, Teiji

2017-11-01

Craniopharyngioma is a slow-growing tumor, but long-term tumor control with maintenance of quality of life is sometimes very difficult to achieve, and hypothalamic disturbance should be strictly avoided in the treatment. However, management of the pituitary gland and/or pituitary stalk varies among surgeons and institutions. This retrospective review identified 44 patients, 24 males and 20 females with craniopharyngiomas who were initially treated by surgery through the extended transsphenoidal approach with pituitary stalk sectioning at a single institute. If the tumor bed involved the posterior lobe of the pituitary gland, pituitary stalk, anterior and/or mid portion of the third ventricle floor, these structures were removed en-bloc together with the tumor. The closest attention was paid to preserve fine arteries running along the surface of optic chiasm and the lateral walls of the third ventricle. Surgical outcome and changes in postoperative endocrinological status were investigated. Gross total removal was achieved in 40 of 44 patients (91%), and all patients could discharge without autonomic and/or thermal disturbances. Tumor remnants were identified with tight adhesion to the perforating arteries in 2 cases, tight adhesion to mammillary bodies in 1, and optic chiasm in 1. Administration of anti-diuretic hormone could be discontinued in 23 of 44 patients (52.3%) with improved diabetes insipidus (DI), although no patient could discontinue glucocorticoid administration. Preservation of thyroid function was achieved in another 23 of 44 patients (52.3%), and recovery from DI was correlated with preservation of thyroid function (p=0.016). Pituitary dysfunction is partially reversible even with pituitary stalk sectioning. Regrowth of tumor in the anterior and/or mid portion of the third ventricle floor including pituitary stalk can possibly be prevented by aggressive tumor removal, and co-achievement of long-term tumor control with maintenance of quality of life

Increased growth rate of vestibular schwannoma after resection of contralateral tumor in neurofibromatosis type 2

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Peyre, Matthieu; Goutagny, Stephane; Imbeaud, Sandrine; Bozorg-Grayeli, Alexis; Felce, Michele; Sterkers, Olivier; Kalamarides, Michel

2011-01-01

Surgical management of bilateral vestibular schwannomas (VS) in neurofibromatosis type 2 (NF2) is often difficult, especially when both tumors threaten the brainstem. When the largest tumor has been removed, the management of the contralateral VS may become puzzling. To give new insights into the growth pattern of these tumors and to determine the best time point for treatment (surgery or medical treatment), we studied radiological growth in 11 VS (11 patients with NF2) over a long period (mean duration, 7.6 years), before and after removal of the contralateral tumor while both were threatening the brainstem. We used a quantitative approach of the radiological velocity of diametric expansion (VDE) on consecutive magnetic resonance images. Before first surgery, growth patterns of both tumors were similar in 9 of 11 cases. After the first surgery, VDE of the remaining VS was significantly elevated, compared with the preoperative period (2.5 ± 2.2 vs 4.4 ± 3.4 mm/year; P = .01, by Wilcoxon test). Decrease in hearing function was associated with increased postoperative growth in 3 cases. Growth pattern of coexisting intracranial meningiomas was not modified by VS surgery on the first side. In conclusion, removal of a large VS in a patient with NF2 might induce an increase in the growth rate of the contralateral medium or large VS. This possibility should be integrated in NF2 patient management to adequately treat the second VS. PMID:21798887

Tumors of the spinal cord and canal

International Nuclear Information System (INIS)

Karlsson, U.L.; Brady, L.W.

1987-01-01

Most spine (primary and secondary) neoplasms should receive curative or palliative radiation therapy. Data from the literature support its use as a beneficial treatment modality. Meningiomas and neurofibromas should be resected and irradiated postoperatively if removed subtotally or if the histopathology is malignant. The prognosis for patients with these tumors is generally good. Intramedullary tumors should be biopsied and irradiated when neoplastic histology has been established. The prognosis for these patients is unsatisfactory for high-grade astrocytomas but is more reasonable for ependymomas and vascular malformations. A favorable exception may be the myxopapillary ependymoma in the lumbosacral region. It should be maximally resected with sparing of cauda equina function but then irradiated postoperatively. The primary intent should be to eradicate the tumor. Radiation therapy is the main treatment modality, with steroid medication, in cases of cord compression

Morbidity and mortality of aggressive resection in patients with advanced neuroendocrine tumors.

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Norton, Jeffrey A; Kivlen, Maryann; Li, Michelle; Schneider, Darren; Chuter, Timothy; Jensen, Robert T

2003-08-01

There is considerable controversy about the treatment of patients with malignant advanced neuroendocrine tumors of the pancreas and duodenum. Aggressive surgery remains a potentially efficacious antitumor therapy but is rarely performed because of its possible morbidity and mortality. Aggressive resection of advanced neuroendocrine tumors can be performed with acceptable morbidity and mortality rates and may lead to extended survival. The medical records of patients with advanced neuroendocrine tumors who underwent surgery between 1997 and 2002 by a single surgeon at the University of California, San Francisco, were reviewed in an institutional review board-approved protocol. Surgical procedure, pathologic characteristics, complications, mortality rates, and disease-free and overall survival rates were recorded. Disease-free survival was defined as no tumor identified on radiological imaging studies and no detectable abnormal hormone levels. Proportions were compared statistically using the Fisher exact test. Kaplan-Meier curves were used to estimate survival rates. Twenty patients were identified (11 men and 9 women). Of these, 10 (50%) had gastrinoma, 1 had insulinoma, and the remainder had nonfunctional tumors; 2 had multiple endocrine neoplasia type 1, and 1 had von Hippel-Lindau disease. The mean age was 55 years (range, 34-72 years). In 10 patients (50%), tumors were thought to be unresectable according to radiological imaging studies because of multiple bilobar liver metastases (n = 6), superior mesenteric vein invasion (n = 3), and extensive nodal metastases (n = 1). Tumors were completely removed in 15 patients (75%). Surgical procedures included 8 proximal pancreatectomies (pancreatoduodenectomy or whipple procedure), 3 total pancreatectomies, 9 distal pancreatectomies, and 3 tumor enucleations from the pancreatic head. Superior mesenteric vein reconstruction was done in 3 patients. Liver resections were done in 6 patients, and an extended periaortic node

[Reimplantation of devitalized tumor-bearing bone in pelvic reconstruction after en-bloc tumor resection].

Science.gov (United States)

Yang, Yi; Guo, Wei; Yang, Rongli; Tang, Xiaodong; Yan, Taiqiang; Ji, Tao; Wei, Ran

2014-10-01

To analyze the clinical outcome of an operative technique using recycling bones to reconstruct pelvis after primary malignant pelvic tumor resection. Fifteen patients who presented with malignant pelvic tumors were treated by wide or marginal resection and reconstruction using recycling bone in our institute from January 2003 to December 2011. The median age was 31 (15-62) years, and the most common diagnosis was chondrosarcoma, followed by Ewing sarcoma. The operative technique consisted of en-bloc excision of the pelvic tumor, removal of soft tissue, curettage of the tumor, incubated in 65 °C 20% hypertonic saline for 30 minutes, reimplantation of recycling bone, and internal fixation with plates, screws and/or total hip replacement. Bone cement was used to augment bone strength when necessary. Bone healing features and function of lower limbs were evaluated with the International Society of Limb Salvage (ISOLS) graft evaluation method and Musculoskeletal Tumor Society (MSTS) score, respectively. Adjuvant therapies were used according to the type and extension of the primary tumor. One patient died of severe peri-operative bleeding 2 days after operation, and the other patients were followed-up for 6 to 96 months (mean 40.4 months), and 5 patients died of local recurrence or metastasis. Eleven operations were followed by complications of any kind. Most mechanical complications were related to the use of hip arthroplasties, where implant breakdown and dislocation were the commonest.Infection was seen in 7 cases (superficial 4 cases and deep 3 cases). Healing and functional scores were fair. The median ISOLS score and MSTS score were 81.0% (range 30.0% to 95.0%) and 60.0% (range 23.0% to 93.0%), respectively. Recycling reconstruction technique is valid for young patients with low-grade chondrosarcoma or other chemo-sensitive tumor in pelvis. Although many complications are seen, this method remains our treatment of choice.

[Influence of anesthesia procedure on malignant tumor outcome].

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Fukui, K; Werner, C; Pestel, G

2012-03-01

Malignant tumors are the second major cause of death in Germany. The essential therapy of operable cancer is surgical removal of primary tumors combined with adjuvant therapy. However, several consequences of surgery may promote metastasis, such as shedding of tumor cells into the circulation, decrease in tumor-induced antiangiogenesis factors, excessive release of growth factors for wound healing and suppression of immunity induced by surgical stress. In the last decade it has become clear that cell-mediated immunity controls the development of metastasis. Various perioperative factors, such as surgical stress, certain anesthetic and analgesic drugs and pain can suppress the patients' immune system perioperatively. On the other hand, by modifications of the anesthesia technique (e.g. regional anesthesia) and perioperative management to minimize immunosuppression, anesthesiologists can play a considerable role for a better outcome in patients having malignant tumors. Sufficient clinical evidence is not yet available to prove or disprove the hypothesis that anesthesia practice can improve cancer prognosis. Despite difficulties in study design, several prospective randomized trials are currently running and the results are awaited to elucidate this topic.

Endoscopic resection of subepithelial tumors.

Science.gov (United States)

Schmidt, Arthur; Bauder, Markus; Riecken, Bettina; Caca, Karel

2014-12-16

Management of subepithelial tumors (SETs) remains challenging. Endoscopic ultrasound (EUS) has improved differential diagnosis of these tumors but a definitive diagnosis on EUS findings alone can be achieved in the minority of cases. Complete endoscopic resection may provide a reasonable approach for tissue acquisition and may also be therapeutic in case of malignant lesions. Small SET restricted to the submucosa can be removed with established basic resection techniques. However, resection of SET arising from deeper layers of the gastrointestinal wall requires advanced endoscopic methods and harbours the risk of perforation. Innovative techniques such as submucosal tunneling and full thickness resection have expanded the frontiers of endoscopic therapy in the past years. This review will give an overview about endoscopic resection techniques of SET with a focus on novel methods.

Endoscopic removal of a dislocated tomour prothesis from the stomach

International Nuclear Information System (INIS)

Rueckauer, K.; Dinkel, E.

1985-01-01

Endoscopic pertubation of oesophagogustric neoplasms is an established method of palliative treatment. The dislocated plastic prosthesis may be removed with difficulties from the stomach endoscopically. A simple technique for endoscopic removal of the prosthesis is described. The tube can be precisely centred within the oesophageal lumen by use of an intestinal decompression tube and additional guidance by the endoscopic retraction forceps. Thus gross damge of the exophytic tumor tissue with bleeding or perforation sequelae can be avoided. Injury to the patient does not exceed that caused by an ordinary gastroscopy. (orig.) [de

Maxillary Swing Approach for Removal of Palatal Carcinoma: A Modified Procedure

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Tsutomu Nomura

2018-01-01

Full Text Available Introduction. We report a modification of the maxillary swing approach to remove a palatal tumor while preserving the anterior alveolar area. Methods. Case report using clinical records. Results. The patient was a 54-year-old male. TNM grade was T4bN0M0, and invasion to the base of the pterygoid process was seen. Two courses of induction chemotherapy were administered prior to the operation. Because there was no evidence of anterior maxillary invasion, the maxillary swing approach was chosen. The left anterior maxilla was cut and swung laterally, preserving the blood supply. After removal of the palatal tumor, the maxilla was repositioned and the defect was restored with an anterior lateral thigh flap. Postoperative course was typical, and facial appearance, speech, and masticatory function were satisfactory. Conclusions. This technique is particularly useful for preserving appearance as well as speech and mastication.

Nonislet Cell Tumor Hypoglycemia in a Patient with Adrenal Cortical Carcinoma

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Se Won Kim

2016-01-01

Full Text Available Nonislet cell tumor hypoglycemia (NICTH is a rare but serious paraneoplastic syndrome in which a tumor secretes incompletely processed precursors of insulin-like growth factor-II (IGF-II, causing hypoglycemia. Here, we report an exceptional case of NICTH caused by nonfunctioning adrenocortical carcinoma in a 39-year-old male with recurrent hypoglycemia. The patient’s serum IGF-II/IGF-I ratio had increased to 27.8. The serum level of the IGF-II/IGF-I ratio was normalized after removal of the tumor, and the hypoglycemic attacks no longer occurred after the operation.

Review of juxtaglomerular cell tumor with focus on pathobiological aspect

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Pan Chin-Chen

2011-08-01

Full Text Available Abstract Juxtaglomerular cell tumor (JGCT generally affects adolescents and young adults. The patients experience symptoms related to hypertension and hypokalemia due to renin-secretion by the tumor. Grossly, the tumor is well circumscribed with fibrous capsule and the cut surface shows yellow or gray-tan color with frequent hemorrhage. Histologically, the tumor is composed of monotonous polygonal cells with entrapped normal tubules. Immunohistochemically, tumor cells exhibit a positive reactivity for renin, vimentin and CD34. Ultrastructurally, neoplastic cells contain rhomboid-shaped renin protogranules. Genetically, losses of chromosomes 9 and 11 were frequently observed. Clinically, the majority of tumors showed a benign course, but rare tumors with vascular invasion or metastasis were reported. JGCT is a curable cause of hypertensive disease if it is discovered early and surgically removed, but may cause a fatal outcome usually by a cerebrovascular attack or may cause fetal demise in pregnancy. Additionally, pathologists and urologists need to recognize that this neoplasm in most cases pursues a benign course, but aggressive forms may develop in some cases.

Macrophages From Irradiated Tumors Express Higher Levels of iNOS, Arginase-I and COX-2, and Promote Tumor Growth

International Nuclear Information System (INIS)

Tsai, C.-S.; Chen, F.-H.; Wang, C.-C.; Huang, H.-L.; Jung, Shih-Ming; Wu, C.-J.; Lee, C.-C.; McBride, William H.; Chiang, C.-S.; Hong, J.-H.

2007-01-01

Purpose: To investigate the effects of single and fractionated doses of radiation on tumors and tumor-associated macrophages (TAMs), and to elucidate the potential of TAMs to influence tumor growth. Methods and Materials: A murine prostate cell line, TRAMP-C1, was grown in C57Bl/6J mice to 4-mm tumor diameter and irradiated with either 25 Gy in a single dose, or 60 Gy in 15 fractions. The tumors were removed at the indicated times and assessed for a variety of markers related to TAM content, activation status, and function. Results: In tumors receiving a single radiation dose, arginase (Arg-I), and cycloxygenase-2 (COX-2) mRNA expression increased as a small transient wave within 24 h and a larger persistent wave starting after 3 days. Inducible nitric oxide synthase (iNOS) mRNA was elevated only after 3 days and continued to increase up to 3 weeks. After fractionated irradiation, Arg-1 and COX-2 mRNA levels increased within 5 days, whereas iNOS was increased only after 10 fractions of irradiation had been given. Increased levels of Arg-I, COX-2, and, to a lesser extent, iNOS protein were found to associate with TAMs 1-2 weeks after tumor irradiation. Function of TAMs were compared by mixing them with TRAMP-C1 cells and injecting them into mice; TRAMP-C1 cells mixed with TAMs from irradiated tumors appeared earlier and grew significantly faster than those mixed with TAMs from unirradiated tumors or TRAMP-C1 alone. Conclusions: Tumor-associated macrophages in the postirradiated tumor microenvironment express higher levels of Arg-1, COX-2, and iNOS, and promote early tumor growth in vivo

 An Uncommon Presentation of Giant Cell Tumor

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Gopal Malhotra

2011-09-01

Full Text Available  Giant Cell Tumors commonly occur at the ends of long bones. However in rare cases, they can occur in the bones of the hands and feet. Tumors in these locations occur in younger patients; in addition, these tumors are more commonly multifocal and are associated with a higher risk for local recurrence than tumors at the ends of long bones. Since lesions in the small bones may be multifocal, a patient with a giant cell tumor of the small bones should undergo a skeletal survey to exclude similar lesions elsewhere. Primary surgical treatment ranges from curettage or excision with or without bone grafting to amputation. The success of surgical treatment depends on the completeness with which the tumor was removed. We are presenting a case report of a 34 year old female, who presented with a swelling in the right hand, following trauma. X-ray of the hand showed an osteolytic expansile lesion at the base of the 1st metacarpal bone. The lesion was initially curetted and then treated by local resection with bone grafting. Histological examination revealed a typical benign giant cell tumor composed of closely packed stromal cells with a variable admixture of giant cells. Follow up at the end of one year did not reveal any recurrence of the tumor.

X-ray pathological-anatomical diagnosis of bone tumors

International Nuclear Information System (INIS)

Adler, C.P.

1983-01-01

The diagnosis of bone tumors is particularly difficult and requires specific knowledge and experience. This is done not only of clinical workers and X-ray specialists but also of pathologists, who are mostly unable to gather enough experience because those diseases are relatively rare. The specialities of the diagnosis of bone tumors are pointed out and the indispensable co-operation between the fields of work of clinical specialists, radiologists and pathologists is emphasized. Bone growths are classified according to the proposals of the World Health Organization, forming the basis for the subsequent therapy. An absolute pre-requisite for an exact diagnosis in the synopsis of the X-ray structures and the histologic findings. In various cases the dignity of a bone tumor cannot be determined; in such instances radical removal is recommended to preclude recidivation and possible malignity. In difficult cases a reference centre for bone tumors should be consulted. (orig.) [de

Reconstruction of segmental bone defect of long bones after tumor resection by devitalized tumor-bearing bone.

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Qu, Huayi; Guo, Wei; Yang, Rongli; Li, Dasen; Tang, Shun; Yang, Yi; Dong, Sen; Zang, Jie

2015-09-24

The reconstruction of an intercalary bone defect after a tumor resection of a long bone remains a challenge to orthopedic surgeons. Though several methods have been adopted to enhance the union of long segmental allografts or retrieved segmental autografts to the host bones, still more progresses are required to achieve a better union rate. Several methods have been adopted to devitalize tumor bone for recycling usage, and the results varied. We describe our experiences of using devitalized tumor-bearing bones for the repairing of segmental defects after tumor resection. Twenty-seven eligible patients treated from February 2004 to May 2012 were included. The segmental tumor bone (mean length, 14 cm) was resected, and then devitalized in 20% sterile saline at 65 °C for 30 min after the tumor tissue was removed. The devitalized bone was implanted back into the defect by using nails or plates. Complete healing of 50 osteotomy ends was achieved at a median time of 11 months (interquartile range (IQR) 9-13 months). Major complications included bone nonunion in four bone junctions (7.4%), devitalized bone fracture in one patient (3.7%), deep infection in three patients (11.1%), and fixation failure in two patients (7.4%). The bone union rates at 1 and 2 years were 74.1 and 92.6%, respectively. The average functional score according to the Musculoskeletal Tumor Society (MSTS) 93 scoring system was 93 % (IQR 80-96.7%). Incubation in 20% sterile saline at 65 °C for 30 min is an effective method of devitalization of tumor-bearing bone. The retrieved bone graft may provide as a less expensive alternative for limb salvage. The structural bone and the preserved osteoinductivity of protein may improve bone union.

Anterior Trans Cervicothoracic Approach for Complete Resection of Cervicothoracic Mediastinal Neurogenic Tumors

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Reza Bagheri

2013-10-01

Full Text Available Introduction:Neurogenic mediastinal tumors comprise a wide range of benign and malignant diseases. A group of these tumors, located at thoracic apex, sometimes spread to cervical spaces causing numerous surgical difficulties. In thoracotomy approaches, due to proximity of the tumors to major blood vessels, complete removal of these tumors from cervical spaces is impossible or may cause intraoperative severe bleeding or other dangerous incidents Because of the adjacent major vessels  that are not visible.The aim of this study is to report cases of surgical treatment of such tumors using Anterior Trans Cervicothoracic Approach (ATCA. Materials and Methods:All patients with neurogenic tumors and cervicomediastinal (CM spread who underwent surgey with ATCA technique during 2005-2011 were included in our study. Then they were evaluated in terms of age, sex, clinical symptoms, radiological and pathological findings, technical success rate of the surgery, surgical complications and first-year relapse rate after the surgery. Results:Our study included 10 patients from whom 9 were female and 1 was male (M/F= 1/9 and the mean age was 27 years. The most common symptoms were pain and feeling of a lump. All patients were operated by this technique successfully. The most common pathological finding was neurofibroma (in 5 patients and surgical complications occurred in 2 patients (20% (Wound infection in 1 patient and brachial plexus injury in another patient. There was no mortality. Disease relapse was reported in 1 patient  ganglioneuroblastoma who underwent surgical resection for the second time. Conclusion: Considering the successful removal of the tumors and favorable exposure of major vessels in cervicomediastinal spaces, this technique is recommended to resect mediastinal tumors with spread to cervical spaces. However, a more definite conclusion requires further studies.

Enhancing predicted efficacy of tumor treating fields therapy of glioblastoma using targeted surgical craniectomy: A computer modeling study

DEFF Research Database (Denmark)

Korshoej, Anders Rosendal; Saturnino, Guilherme Bicalho; Rasmussen, Line Kirkegaard

2016-01-01

the potential of the intervention to improve the clinical efficacy of TTFields therapy of brain cancer. Methods: We used finite element analysis to calculate the electrical field distribution in realistic head models based on MRI data from two patients: One with left cortical/subcortical glioblastoma and one......Objective: The present work proposes a new clinical approach to TTFields therapy of glioblastoma. The approach combines targeted surgical skull removal (craniectomy) with TTFields therapy to enhance the induced electrical field in the underlying tumor tissue. Using computer simulations, we explore...... with deeply seated right thalamic anaplastic astrocytoma. Field strength was assessed in the tumor regions before and after virtual removal of bone areas of varying shape and size (10 to 100 mm) immediately above the tumor. Field strength was evaluated before and after tumor resection to assess realistic...

Papillary endothelial hyperplasia (Masson's tumor) in children.

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Liné, A; Sanchez, J; Jayyosi, L; Birembaut, P; Ohl, X; Poli-Mérol, M-L; François, C

2017-06-01

The intravascular papillary endothelial hyperplasia (IPEH/Masson's tumor) is a rare benign tumor of the skin and subcutaneous vessels. We report, in four pediatric cases, clinical presentation, care (diagnostic and surgical) of Masson's tumor in children. Two boys (two years) and two girls (four and six years) showed a pain subcutaneous tumor (one to five centimeters). They were in the transverse abdominal muscle, between two metatarsals, at the front of thigh and in the axilla. Imaging performed (MRI, Doppler ultrasound) evoked either a hematoma, a lymphangioma or hemangioma. The indication for removal was selected from pain and/or parental concern. The diagnosis was histologically. A lesion persisted in residual form (incomplete initial resection), and is currently not scalable for eleven years. This tumor is characterized by excessive proliferation and papillary endothelial cells in the vessels, following a thrombotic event. It is found mainly in adults (no specific age), and preferentially localizes in the face and limbs. The clinical differential diagnosis of this tumor is angiosarcoma. The imagery has not allowed in our series to diagnose but still essential to eliminate differential diagnoses. Only surgical excision with histological examination can differentiate. Our study emphasizes the possibility of pediatric cases with two cases of unusual locations (abdominal and axilla). Clinical presentations we met, now lead us to direct our histologist looking for a Masson tumor in any child with a subcutaneous tumor and/or intramuscular pain, sudden onset, and vascular appearance (after excluding an arteriovenous malformation). Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Familial Investigations of Childhood Cancer Predisposition

Science.gov (United States)

2018-01-03

Acute Leukemia; Adenomatous Polyposis; Adrenocortical Carcinoma; AML; BAP1 Tumor Predisposition Syndrome; Carney Complex; Choroid Plexus Carcinoma; Constitutional Mismatch Repair Deficiency Syndrome; Diamond-Blackfan Anemia; DICER1 Syndrome; Dyskeratosis Congenita; Emberger Syndrome; Familial Acute Myeloid Leukemia; Familial Adenomatous Polyposis; Fanconi Anemia; Familial Cancer; Familial Wilms Tumor; Familial Neuroblastoma; GIST; Hereditary Breast and Ovarian Cancer; Hereditary Paraganglioma-Pheochromocytoma Syndrome; Hodgkin Lymphoma; Juvenile Polyposis; Li-Fraumeni Syndrome; Lynch Syndrome; MDS; Melanoma Syndrome; Multiple Endocrine Neoplasia Type 1; Multiple Endocrine Neoplasia Type 2; Neuroblastoma; Neurofibromatosis Type 1; Neurofibromatosis Type II; Nevoid Basal Cell Carcinoma Syndrome; Non Hodgkin Lymphoma; Noonan Syndrome and Other Rasopathy; Overgrowth Syndromes; Pancreatic Cancer; Peutz-Jeghers Syndrome; Pheochromocytoma/Paraganglioma; PTEN Hamartoma Tumor Syndrome; Retinoblastoma; Rhabdoid Tumor Predisposition Syndrome; Rhabdomyosarcoma; Rothmund-Thomson Syndrome; Tuberous Sclerosis; Von Hippel-Lindau Disease

A gastrointestinal stromal tumor (GIST masquerading as an ovarian mass

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Beneduce Pasquale

2004-05-01

Full Text Available Abstract Background Malignant gastrointestinal stromal tumors (GIST are rare mesenchymal tumors originating in the wall of the gastrointestinal tract. Myogenic gastrointestinal stromal tumor, a distinctive morphologic variant is characterized by an unusually prominent myxoid stromal background. Case presentation We report a case of myxoid variant of GIST in a 42 years old woman presenting as an epigastric mass associated to an ovarian cyst and elevated CA-125. Histologically, the lesions was composed of a proliferation of spindle cells in an abundant myxoid stroma, without evidence of atypia or anaplasia. Immunohistochemical stains showed strong positive staining with muscle actin, positive staining with CD34 and weak positive staining with CD117, while showed negative for S-100. Conclusion At surgery every effort should be made to identify the origin of the tumor. A complete surgical removal of the tumor should be obtained, as this is the only established treatment that offers long term survival.

Laparoscopic Management of Sclerosing Stromal Tumors of the Ovary Combined with Ectopic Pregnancy.

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Liu, Hua-Qian; Liu, Qiang; Sun, Xue-Bing; Chang, Wen-Min

2015-01-01

Like other stromal-derived gynecological tumors, a sclerosing stromal tumor of the ovary (SSTO) is a rare benign tumor that is difficult to distinguish from a malignant ovarian tumor in clinical practice. An SSTO is routinely treated with laparotomy. Here, we present two extremely rare cases of SSTO with contralateral and ipsilateral tubal pregnancies, in which laparoscopic surgery was performed to remove the tumors. After surgery, one patient (case 1) became pregnant twice within 29 months, and the other patient (case 2) did not become pregnant within 6 months postoperatively. These two cases suggest that laparoscopic management is not only useful in treating SSTO and complicating diseases, but it may also help to reduce unnecessary surgical injury to the ovary. © 2015 S. Karger AG, Basel.

The association of congenital neuroblastoma and congenital heart disease

International Nuclear Information System (INIS)

Bellah, R.; D'Andrea, A.; Children's Hospital, Boston, MA; Darillis, E.; Fellows, K.E.

1989-01-01

Several authors have reported an association between neuroblastoma and congenital heart disease; others contend that, unlike specific wellknown associations between malignancy and congenital defects (Wilm's tumor and aniridia, leukemia and Down's syndrome), no real relationship exists. We present three cases of cyanotic congenital heart disease in which subclinical neuroblastoma was found. We speculate that abnormal neural crest cell migration and development may be a common link between cardiac malformations and congenital neuroblastoma. (orig.)

Benign Pigmented Dermal Basal Cell Tumor in a Namibian Cheetah (Acinonyx jubatus

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Sonja K. Heinrich

2016-01-01

Full Text Available A 3.5-year-old wild born cheetah (Acinonyx jubatus, living in a large enclosure on a private Namibian farm, developed a large exophytic nodular neoplasm in its skin at the height of the left shoulder blade. We describe the clinical appearance, the surgical removal, and histological examination of the tumor, which was diagnosed as a moderately pigmented benign basal cell tumor. A three-year follow-up showed no evidence of recurrence after the surgery. Although neoplasia is reported in nondomestic felids, only very few concern cheetahs. So far, no case of basal cell tumor was described in this species.

Value of gamma knife radiosurgery for tumors invading cavernous sinus

International Nuclear Information System (INIS)

Jokura, Hidefumi; Yoshimoto, Takashi

1999-01-01

The usefulness of radiosurgery for cavernous sinus tumors was evaluated based on our experience and recent published reports from other institutes. Twenty-six meningiomas involving the cavernous sinus were treated by radiosurgery. The length of follow-up average 3 years. Tumors regressed in 40% and remained stable in 56% of cases. A total of 96% of the tumors were controlled with only a few minor complications. We believe surgical resection to reduce the volume of the tumor without causing new neurological deficits, followed by radiosurgery on the tumor located in the cavernous sinus is the best choice in many cases. Twenty-five pituitary adenomas with cavernous sinus invasion were treated by a combination of transsphenoidal removal and radiosurgery. All the tumors are controlled in terms of volume during the follow-up (average of 34 months). There were no new neurological deficits, including visual disturbance. Hormone elevation was able to be corrected at an early stage without pituitary insufficiency more by radiosurgery than by fractionated radiation. However, to obtain good results by radiosurgery, it must be preceded by complete surgical decompression of optic nerves and chiasma from the tumor. (author)

Effect of misonidazole on radiosensitivity of Ehrlich ascites tumor cells

International Nuclear Information System (INIS)

Takeuchi, Hiroshi

1986-01-01

The effect of Misonidazole on radiosensitivity of Ehrlich ascites tumor cells was studied in vivo. Ehrlich ascites tumor cells growing intraperitoneally (ICR/SIC mice) for either 1, 4, 6 or 10 days were irradiated in vivo (whole body irradiation) with or without Misonidazole. Immediately after irradiation tumor cells were transplanted intraperitoneally into new animals. Four days later, the propagated surviving cells were removed and counted for analyses. Enhancement ratio of Misonidazole at the surviving fraction of 0.1 were 1.0 (for 1-day-old), 1.3 (for 4-day-old), 1.9 (for 6-day-old), 1.9 (for 10-day-old) and 2.8 (for anoxic cells) respectively. The gradual increase of the enhancement ratio of the ascites tumore cells during intraperitoneal growth from 1 through 10 days might be attributed to an increase of hypoxic tumor cells. Cytotoxicity was not observed at 0.1 mg per gram body weight of Misonidazole but was at 1 mg per gram body weight of Misonidazole in 6-day-old and 10-day-old Ehrlich ascites tumor cells which were supposed to contain hypoxic cells. These results suggest that Misonidazole may prove an effective radiosensitizer for hypoxic tumor cells. (author)

Regulation of radiation-induced apoptosis by early growth response-1 gene in solid tumors

International Nuclear Information System (INIS)

Ahmed, M.

2003-01-01

Ionizing radiation exposure is associated with activation of certain immediate-early genes that function as transcription factors. These include members of jun or fos and early growth response (EGR) gene families. In particular, the functional role of EGR-1 in radiation-induced signaling is pivotal since the promoter of EGR-1 contains radiation-inducible CArG DNA sequences. The Egr-1 gene belongs to a family of Egr genes that includes EGR-2, EGR-3, EGR-4, EGR-α and the tumor suppressor, Wilms' tumor gene product, WT1. The Egr-1 gene product, EGR-1, is a nuclear protein that contains three zinc fingers of the C 2 H 2 subtype. The EGR-1 GC-rich consensus target sequence, 5'-GCGT/GGGGCG-3' or 5'-TCCT/ACCTCCTCC-3', has been identified in the promoter regions of transcription factors, growth factors, receptors, cell cycle regulators and pro-apoptotic genes. The gene targets mediated by Egr-1 in response to ionizing radiation include TNF-α , p53, Rb and Bax, all these are effectors of apoptosis. Based on these targets, Egr-1 is a pivotal gene that initiates early signal transduction events in response to ionizing radiation leading to either growth arrest or cell death in tumor cells. There are two potential application of Egr-1 gene in therapy of cancer. First, the Egr-1 promoter contains information for appropriate spatial and temporal expression in-vivo that can be regulated by ionizing radiation to control transcription of genes that have pro-apoptotic and suicidal function. Secondly, EGR-1 protein can eliminate 'induced-radiation resistance' by inhibiting the functions of radiation-induced pro-survival genes (NFκB activity and bcl-2 expression) and activate pro-apoptotic genes (such as bax) to confer a significant radio-sensitizing effect. Together, the reported findings from my laboratory demonstrate clearly that EGR-1 is an early central gene that confers radiation sensitivity and its pro-apoptotic functions are synergized by abrogation of induced radiation

Endoscopic-modified medial maxillectomy and its limitation for a solitary fibrous tumor of the lacrimal sac and nasolacrimal duct.

Science.gov (United States)

Moriyama, Munehito; Kodama, Satoru; Hirano, Takashi; Suzuki, Masashi

2017-06-01

Solitary fibrous tumor (SFT) is an uncommon neoplasm that usually arises from the pleura. Recently, SFTs have been reported in the head and neck region located in subsites such as the orbit. SFTs of the lacrimal sac are extremely rare, with only six cases reported in the English literature. We describe a SFT arising from the right lacrimal sac and extending along the nasolacrimal duct into the nasal cavity. Although, the tumor could not be removed by endoscopic-modified medial maxillectomy (EMMM) alone, combined approach with EMMM and a small external incision achieved the complete removal of the tumor. The patient has remained disease-free 24 months after surgery. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

FISH studies in a girl with sporadic aniridia and an apparently balanced de novo t(11;13(p13;q33 translocation detect a microdeletion involving the WAGR region

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J.C. Llerena Jr.

2000-09-01

Full Text Available Conventional cytogenetic studies on a female infant with sporadic aniridia revealed what appeared to be a balanced de novo t(11;13 (p13;q33 translocation. Fluorescence in situ hybridization (FISH investigations, however, detected the presence of a cryptic 11p13p14 deletion which included the WAGR region and involved approximately 7.5 Mb of DNA, including the PAX6 and WT1 genes. These results account for the patient's aniridia, and place her at high risk for developing Wilms' tumour. The absence of mental retardation in the patient suggests that the position of the distal breakpoint may also help to refine the mental retardation locus in the WAGR contiguous gene syndrome (Wilms', aniridia, genital anomalies and mental retardation.O estudo citogenético convencional em uma menina com aniridia esporádica resultou em uma aparente translocação balanceada t(11;13(p13;q33 de novo. Entretanto, o estudo citogenético pela hibridação in situ fluorescente (FISH detectou a presença de uma deleção críptica 11p13p14, incluindo a região WAGR e envolvendo aproximadamente 7.5 Mb de DNA, deletando os genes PAX6 e WT1. Estes resultados correlacionam-se com o quadro clínico da paciente e a coloca em alto risco de desenvolver tumor de Wilms. A ausência de retardo mental na paciente indica que a posição distal do ponto de quebra poderá refinar o mapeamento do locus retardo mental na síndrome de genes contíguos WAGR (Wilms, aniridia, anomalias genitais e retardo mental.

Partial removal and post-operative irradiation for craniopharyngioma

International Nuclear Information System (INIS)

Nishioka, Takeshi; Shirato, Hiroki; Arimoto, Takuro; Kamata, Tadashi; Suzuki, Keishiro; Kitahara, Toshihiro.

1992-01-01

From 1971 to 1990, sixteen patients with craniopharyngioma were treated by postoperative radiotherapy (RT). Fourteen patients underwent partial removal and RT as primary treatment. In two patients, partial removal and RT were performed for treatment of recurrence after total removal. Total dose was between 40 Gy and 60 Gy. Five year survival and 5-year relapse free rates after RT were 100% and 71.7%, respectively. Computed tomography raised suspicion of radiation necrosis in two patients, each treated with two opposing lateral ports. The following hormonal replacements were needed: adrenal in 73% of the patients, thyroid in 66%, growth hormone in 50% of the children, and antidiuretic hormone in 21%. The amounts of thyroid and adrenal hormones for substitution have increased gradually with elapsed time after RT. To minimize these complications and to achieve tumor control, a multiport technique including radiosurgery, and a total dose of 50-55 Gy with 2 Gy per fraction is suggested to be optimal. (author)

Partial removal and post-operative irradiation for craniopharyngioma

Energy Technology Data Exchange (ETDEWEB)

Nishioka, Takeshi; Shirato, Hiroki (Obihiro Kousei Hospital, Hokkaido (Japan)); Arimoto, Takuro; Kamata, Tadashi; Suzuki, Keishiro; Kitahara, Toshihiro

1992-12-01

From 1971 to 1990, sixteen patients with craniopharyngioma were treated by postoperative radiotherapy (RT). Fourteen patients underwent partial removal and RT as primary treatment. In two patients, partial removal and RT were performed for treatment of recurrence after total removal. Total dose was between 40 Gy and 60 Gy. Five year survival and 5-year relapse free rates after RT were 100% and 71.7%, respectively. Computed tomography raised suspicion of radiation necrosis in two patients, each treated with two opposing lateral ports. The following hormonal replacements were needed: adrenal in 73% of the patients, thyroid in 66%, growth hormone in 50% of the children, and antidiuretic hormone in 21%. The amounts of thyroid and adrenal hormones for substitution have increased gradually with elapsed time after RT. To minimize these complications and to achieve tumor control, a multiport technique including radiosurgery, and a total dose of 50-55 Gy with 2 Gy per fraction is suggested to be optimal. (author).

Recurrent spinal primitive neuroectodermal tumor with brain and bone metastases: A case report.

Science.gov (United States)

Chen, Frank; Chiou, Shyh-Shin; Lin, Sheng-Fung; Lieu, Ann-Shung; Chen, Yi-Ting; Huang, Chih-Jen

2017-11-01

Primary spinal primitive neuroectodermal tumor (PNET) is relatively rare in all age groups, and the prognosis in most cases of spinal PNETs appears to be poor, with a median patient survival of 1 to 2 years. We present a case with recurrent spinal PNET with brain and bone metastases that was successfully treated by multimodality treatment. A 14-year-old teenage girl had suffered from progressive left upper back pain with bilateral lower legs weakness and numbness for 1 year. After treatment, left neck mass was noted 3 years later. Initially, magnetic resonance imaging (MRI) showed neurogenic tumor involving intradural extramedullary space of T5-T10. Pathology report showed PNET (World Health Organization grade IV) featuring lobules of neoplastic cells with round regular nuclei, high nucleus-to-cytoplasm ratio, and fibrillary cytoplasm. At the time of tumor recurrence, chest MRI then showed recurrent tumor at T2-T3 level of the epidural space with right neural foramina invasion. Brain MRI showed extensive bilateral calvarial metastases and leptomeningeal metastases in the right frontoparietal regions. Bone scan showed multiple bone metastases. T-spine tumor removal and adjuvant radiotherapy (RT) to T-spine tumor bed were performed in the initial treatment. After clinical tumor recurrence, tumor removal was done again. She then received chemotherapy followed by whole brain irradiation with hippocampal sparing with 35 gray in 20 fractions. After treatment, follow-up images showed that the disease was under control. There was no neurological sequela. She has survived more than 7 years from diagnosis and more than 4 years from recurrence to date. Multimodality treatments including operation, RT, and chemotherapy should be considered in the initial treatment planning, and salvage chemotherapy was useful in this case.

99mTc-MIBI-SPECT-studies in the evaluation of brain tumors

International Nuclear Information System (INIS)

Ambrus, E.; Pavics, L.; Gruenwald, F.; Barath, B.; Tiszlavicz, L.; Bender, H.; Menzel, C.; Almasi, L.; Lang, J.; Bodosi, M.; Biersack, H.J.; Csernay, L.

1994-01-01

Brain SPECT studies were performed 5 and 60 minutes after 99m Tc-MIBI administration in 41 patients with brain tumors confirmed by CT and surgical removal (13 meningeomas, 8 astrocytomas grades I-III, 10 glioblastomas, 10 metastases). 99m Tc-MIBI uptake was found in 32 out of 41 brain tumors. According to the semiquantitative SPECT analysis, the tumor/non tumor radios revealed a statistically significant difference in the early tracer uptake between meningeomas and astrocytomas (+4.73±2.91 vs -1.75±0.75, p 99m Tc-MIBI uptake and its changes with time. We concluded that the combination of an early and late 99m Tc-MIBI brain SPECT may be helpful in the non invasive histological classification of brain tumors and the determination of the grade of theirs malignancy. (orig.) [de

Effect of uracil on the intracellular distribution of 1-(2-tetrahydrofuryl)-5-fluorouracil in the mouse tumor cells

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Fujimoto, J.; Kosaki, G. (Osaka Univ. (Japan). Faculty of Medicine)

1980-12-01

Fifteen ..mu..g/g 1-(2-tetrahydrofuryl)-5-fluoro (-6-/sup 3/H) uracil (/sup 3/H-FT) were injected i.p. into mice bearing Fujimoto ascites tumor. Tumor cells were removed at various intervals and the autoradiographs were prepared. The specimens were treated first with chloroform before being coated with emulsion, so as to remove /sup 3/H-FT in the specimens and to preserve 5-fluoro (-6-/sup 3/H) uracil (/sup 3/H-FU) which was released from /sup 3/H-FT. Autoradiographs revealed a higher concentration of silver grains which localized over the nucleus, especially over the nucleoli, of tumor cells than over the cytoplasm. When 33.6 ..mu..g uracil/g was coadministered with 15.0 ..mu..g /sup 3/H-FT/g into tumor-bearing mice, intracellular distribution of /sup 3/H-FU released from /sup 3/H-FT was unchanged and an incorporation of /sup 3/H-FU into the tumor cells was higher than when /sup 3/H-FT alone.

Tumor cells have decreased ability to metabolize H2O2: Implications for pharmacological ascorbate in cancer therapy

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Claire M. Doskey

2016-12-01

Full Text Available Ascorbate (AscH− functions as a versatile reducing agent. At pharmacological doses (P-AscH−; [plasma AscH−] ≥≈20 mM, achievable through intravenous delivery, oxidation of P-AscH− can produce a high flux of H2O2 in tumors. Catalase is the major enzyme for detoxifying high concentrations of H2O2. We hypothesize that sensitivity of tumor cells to P-AscH− compared to normal cells is due to their lower capacity to metabolize H2O2. Rate constants for removal of H2O2 (kcell and catalase activities were determined for 15 tumor and 10 normal cell lines of various tissue types. A differential in the capacity of cells to remove H2O2 was revealed, with the average kcell for normal cells being twice that of tumor cells. The ED50 (50% clonogenic survival of P-AscH− correlated directly with kcell and catalase activity. Catalase activity could present a promising indicator of which tumors may respond to P-AscH−.

Radiological evaluation of the intraabdominal masses in childhood

International Nuclear Information System (INIS)

Kim, Y. J.; Oh, K. K.; Park, C. Y.

1980-01-01

The spectrum of types of cancer in childhood differs strikingly from that in adults. Leukemia, central nervous system tumors, embryonal tumors, and sarcomas are much more common in children than are adenocarcinoma and carcinomas, which constitute the majority of cancers in adults. In children under 15 years of age, intraabdominal tumors are the third most common one, preceded only by leukemia and brain tumors. It is well recognized that these tumors remain silent until they assume huge size, and early detection and treatment are important. X-ray examination is the most important method among diagnostic approaches. 150 cases of intraabdominal masses were reviewed in respect of age, sex, incidence, site of origin and radiographic findings. The results are summarized as follows; 1. The most common site was retroperitoneum (80 cases: 53.3%), followed by intraperitoneum (47 cases: 31.3%), and pelvic cavity (23 cases: 15.4%). The kidney was the single most common site of origin (33%). 2. Hydronephrosis was most common (23 cases: 15.3%) and Wilms' tumor (22 cases: 14.7%), teratoma (21 cases: 14.0%), and neuroblastoma (18 cases: 12.0%) were descending order of frequency. One renal cell carcinoma in 9 year old female patient and bilateral duplications of pelvis with ureteroceles in 5 month old twin infants were found. 3. Male outnumbered female in most childhood abdominal diseases, however female was predominant in teratoma (1:20) and choledochal cyst (3:7). 97 cases were under the age of 5 years. 4. Radiographic findings were as follow, a. Simple abdomen: In retroperitoneal tumors, mass shadows were the most common finding (89.7%) and obliteration of psoas shadows was noted in 73%. Calcifications were seen in 6 cases (100%) of teratoma as bone, teeth or amorphous, 1 case (4.5%) of Wilms' tumor as amorphous, and 5 cases (33%) or neuroblastoma as amorphous or psammomatous. Fat shadow was seen in 5 cases (83%) of teratoma. In intraperitoneal tumors, mass was the most common

Hyperparathyroidism-jaw Tumor Syndrome: An Overlooked Cause of Severe Hypercalcemia.

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Mathews, Joseph Wolfgang; Winchester, Rhonda; Alsaygh, Nebras; Bartlett, Anne M; Luttrell, Louis

2016-09-01

Ossifying fibromas of the maxillofacial bones are an uncommon form of benign neoplasm usually treated by surgical excision. Up to 30% of patients with hyperparathyroidism-jaw tumor syndrome, a rare form of multiple endocrine neoplasia resulting from autosomal dominant inactivating mutation of the Hrpt2 tumor suppressor gene, initially present with ossifying fibromas. Coincident hypercalcemia because of the presence of parathyroid adenoma is common in these patients, of whom 15% may have or may develop parathyroid carcinoma. The authors present a case of severe postsurgical hypercalcemia after removal of a large maxillary ossifying fibroma in a patient with previously unrecognized hyperparathyroidism-jaw tumor AU3 syndrome. Copyright © 2016 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

Cecal Carcinoid Tumor in a Nigerian Man: A Case Report and ...

African Journals Online (AJOL)

2016-10-10

Oct 10, 2016 ... a case of a 56-year-old Nigerian man with a cecal carcinoid tumor that was surgically removed. ... per 100,000.[3,4]. Other studies have found carcinoid ... with symptoms of pain, anorexia, or weight loss.[8]. Approximately ...

Phosphoinositide 3-kinase accelerates postoperative tumor growth by inhibiting apoptosis and enhancing resistance to chemotherapy-induced apoptosis. Novel role for an old enemy.

LENUS (Irish Health Repository)

Coffey, J Calvin

2012-02-03

Tumor removal remains the principal treatment modality in the management of solid tumors. The process of tumor removal may potentiate the resurgent growth of residual neoplastic tissue. Herein, we describe a novel murine model in which flank tumor cytoreduction is followed by accelerated local tumor recurrence. This model held for primary and recurrent tumors generated using a panel of human and murine (LS174T, DU145, SW480, SW640, and 3LL) cell lines and replicated accelerated tumor growth following excisional surgery. In investigating this further, epithelial cells were purified from LS174T primary and corresponding recurrent tumors for comparison. Baseline as well as tumor necrosis factor apoptosis-inducing ligand (TRAIL)-induced apoptosis were significantly reduced in recurrent tumor epithelia. Primary and recurrent tumor gene expression profiles were then compared. This identified an increase and reduction in the expression of p110gamma and p85alpha class Ia phosphoinositide 3-kinase (PI3K) subunits in recurrent tumor epithelia. These changes were further confirmed at the protein level. The targeting of PI3K ex vivo, using LY294002, restored sensitivity to TRAIL in recurrent tumor epithelia. In vivo, adjuvant LY294002 prolonged survival and significantly attenuated recurrent tumor growth by greatly enhancing apoptosis levels. Hence, PI3K plays a role in generating the antiapoptotic and chemoresistant phenotype associated with accelerated local tumor recurrence.

Glomus jugulare tumor with intra- and extracranial extension

International Nuclear Information System (INIS)

Morisako, Toshitaka; Goya, Tomokazu; Wakisaka, Shinichiro; Kinoshita, Kazuo

1987-01-01

A case of glomus jugulare tumor with intra- and extracranial extension is described. The patient was a 63-year-old woman who complained of gait and memory disturbances. On admission neurological examination revealed recent memory disturbance, left deafness, left XI, XIIth cranial nerve palsies, and slight ataxic gait. Roentgenogram of the skull showed an enlarged left jugular foramen with bone erosion. Plain X-ray computerized tomography scan (X-CT) indicated obstructive hydrocephalus and X-CT with contrast enhancement revealed a mass lesion in the left posterior cranial fossa extending through enlarged left jugular foramen to the extracranial space toward the level of C 2 . Cerebral angiography demonstrated a large mass with blood supply from branches of left external carotid and vertebral arteries. The tumor stain was not remarkable. Left internal jugular vein was completely obstructed at the level of the second cervical vertebral body. Magnetic resonance imaging (MRI) clearly showed the tumor extending from the anterolateral portion to the second cervical vertebral body through the enlarged jugular foramen to the posterior cranial fossa. Brain stem and cerebellar hemisphere which were markedly compressed by the mass were clearly visualized. At first a ventriculo-peritoneal shunt was made and four weeks later subtotal removal of the tumor was undertaken. Histopathology of tumor specimen showed typical glomus jugulare tumor. MRI was considered to be very useful for the diagnosis and treatment of the glomus jugulare tumor with intra- and extracranial extension. (author)

Papillary Ependymoma WHO Grade II of the Aqueduct Treated by Endoscopic Tumor Resection

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Andreas M. Stark

2009-01-01

Full Text Available Papillary ependymoma is a rare tumor that may be located along the ventricular walls or within the spinal cord. We report the case of a 54-year-old patient with a papillary ependymoma WHO grade II arising at the entrance of the aqueduct. The tumor caused hydrocephalus. The tumor was completely removed via a right-sided endoscopic approach with restoration of the aqueduct. The free cerebrospinal fluid passage through the aqueduct was not only visualized by endoscopy but also controlled by intraoperative high-field magnetic resonance imaging. Therefore, an additional endoscopic third ventriculostomy was unneccessary.

Intraoperative Tumoral Bleeding of Hypervascular Medulloblastoma after Ventricular Drainage: A Case Report.

Science.gov (United States)

Ryu, Han-Seung; Jung, Tae-Young; Han, Moon-Soo; Kim, Seul-Ki; Lee, Kyung-Hwa

2017-01-01

We report a rare case of intraoperative tumoral bleeding of a hypervascular medulloblastoma. A 12-year-old girl presented with dizziness and nausea. Brain magnetic resonance (MR) images revealed an approximately 4.2-cm enhanced mass on the cerebellar vermis associated with mild perilesional edema and increased cerebral blood volume. Angiography showed tumoral staining and developed occipital and circular dural sinuses in the venous phase. A suboccipital craniotomy was performed. To relieve the intracranial pressure, cerebrospinal fluid (CSF) was drained via a lateral ventricular catheter in the occipital horn. During the opening of the dura, the brain swelling had progressed, and brain computed tomography revealed an intratumoral hemorrhage with brainstem compression. The patient was in a stuporous mental state. A reoperation was performed, and the mass was totally removed. The pathologic findings revealed a medulloblastoma with abnormal enlarged arterial vascular structures. Postoperatively, the patient recovered to an alert mental state. She underwent chemotherapy and radiotherapy. There was no recurrence after 1 year. Pre-resectional CSF drainage should not be routinely performed in posterior fossa tumors, especially with increased cerebral blood volume on MR perfusion images. Complete removal should be performed quickly while CSF drainage should be performed slowly. An intratumoral hemorrhage should be considered in posterior fossa tumors when severe brain swelling suddenly develops after CSF drainage. © 2016 S. Karger AG, Basel.

Achados clínicos e genéticos de cinco pacientes com anomalias relacionadas ao gene WT1

OpenAIRE

Andrade, Juliana Gabriel R. de; Guaragna, Mara Sanches; Soardi, Fernanda Caroline; Guerra-Júnior, Gil; Mello, Maricilda Palandi de; Maciel-Guerra, Andréa Trevas

2008-01-01

AIM: To present phenotypic variability of WT1-related disorders. METHODS: Description of clinical and genetic features of five 46,XY patients with WT1 anomalies. RESULTS: Patient 1: newborn with genital ambiguity; he developed Wilms tumor (WT) and chronic renal disease and died at the age of 10 months; the heterozygous 1186G>A mutation compatible with Denys-Drash syndrome was detected in this child. Patients 2 and 3: adolescents with chronic renal disease, primary amenorrhea and hypergonadotr...

Clinical Investigation Program (Tripler Army Medical Center)

Science.gov (United States)

1991-10-01

8217 I have learned an immense amount during this last year. Clinicians have no idea of the real world of medical research, finance, and politics. We (I...91. 10 Pfanner TP, Person DA, Berenberg JL, Lockett LJ: Eosinophilia Myalgia Syndrome: A Case Report and One Year Follow-Up. American College of ...protocol as yet. This is a non therapeutic study designed to gather epidemiologic and late effects data on long term (>Syrs) survivors of Wilms’ tumor

Conjunctival tumors in children: histopathologic diagnosis in 165 cases

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Martin A. Zimmermann-Paiz

2015-12-01

Full Text Available ABSTRACT Purpose: Conjunctival tissue tumors have a varied presentation, and few series studies on pediatric patients have been published. The objective of this paper is to report the histopathologic diagnoses (spanning over 1988-2013 of conjunctival tumors in children younger than 14 years. Methods: We conducted a retrospective, descriptive, and observational study by reviewing the database of all children in whom a conjunctival tumor was surgically removed at Hospital de Ojos y Oídos "Dr. Rodolfo Robles V.," Benemérito Comité Pro Ciegos y Sordos de Guatemala. The data pertaining to gender, age, and histopathologic diagnosis of all cases was collected. The same ocular pathologist made all diagnoses. Results: One hundred sixty-five cases were found, with a mean age of 7.88 years, being 91 (55.15% male subjects. Melanocytic lesions were the most common tumors found (30.91% of cases, with only one case (0.60% being malignant. Conclusions: Melanocytic lesions were the most common tumors found, and of all the cases, only one was malignant; this was in a patient with xeroderma pigmentosum. These findings are consistent with those reported in other studies regarding the frequencies of the histopathology of conjunctival tumors in the pediatric population.

A case report of phosphaturic mesenchymal tumor-induced osteomalacia.

Science.gov (United States)

Wu, Weiqian; Wang, Chongyang; Ruan, Jianwei; Chen, Feng; Li, Ningjun; Chen, Fanghu

2017-12-01

Tumor-induced osteomalacia (TIO) is a rare and often misdiagnosed syndrome. Surgical resection is currently the first line treatment for TIO. Here we report the case of a 49-year-old woman presented with intermittent pain in the right chest and bilateral hip that had persisted for over two years. She was diagnosed of TIO caused by a phosphaturic mesenchymal tumor based on the following examinations. Laboratory tests revealed high serum alkaline phosphatase, high urinary phosphorus, hypophosphatemia and normal serum calcium levels. 18-FDG PET/CT indicated a systemic multi-site symmetrical pseudo fracture and a tumor in the 7th right rib. Curettage of the tumor was performed, and pathological analysis also confirmed our diagnoses as a phosphaturic mesenchymal tumor. At seven months post-surgery, the symptoms were relieved, proximal muscle strength was improved and serum levels of phosphorus and alkaline phosphatase normalized. The bilateral femoral neck and bilateral pubic bone fractures were blurred in the pelvic plain X-ray, suggesting that the fracture was healing. This case report strengthened the importance of recognition of this rare disease to avoid delay of diagnosis and surgical removal of the causative tumor is recommended. Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.

Treatment of oral soft tissues benign tumors using laser

Science.gov (United States)

Crisan, Bogdan; Baciut, Mihaela; Crisan, Liana; Bran, Simion; Rotar, Horatiu; Dinu, Cristian; Moldovan, Iuliu; Baciut, Grigore

2014-01-01

The present study aimed to assess the efficacy and indications of surgical laser therapy in the treatment of oral soft tissues benign tumors compared to classic surgery. A controlled clinical study was conducted in a group of 93 patients presenting various forms of oral soft tissues benign tumors. These patients were examined pre-and postoperatively and the oral benign tumors were measured linearly and photographed. The surgery of laser-assisted biopsy excision of oral benign tumors was carried out using a diode laser device of 980 nm. In patients who received surgical laser treatment, therapeutic doses of laser to biostimulate the operated area were administered on the first day after the surgery. The interventions of conventional excision of oral soft tissues benign tumors consisted in removing them using scalpel. In patients who have received therapeutic doses of laser for biostimulation of the operated area, a faster healing of wound surfaces and tumor bed was observed during the first days after surgery. Two weeks after the surgical treatment, good healing without scarring or discomfort in the area of excision was documented. Surgical treatment of oral soft tissues benign tumors with laser assisted postoperative therapy confirms the benefits of this surgical procedure. A faster healing process of the excision area due to laser biostimulation of low intensity has been observed in patients with surgical laser assisted treatment in the postoperative period.

[Lung metastases: tumor reduction as an oncologic concept].

Science.gov (United States)

Dienemann, H; Hoffmann, H; Trainer, C; Muley, T

1998-01-01

The principle of surgery for lung metastases is the removal of all lesions in the lung that are either visible or detectable by palpation. This may be combined with complete dissection of all ipsilateral lymph nodes. Therefore, "tumor reduction" rather than "complete" or "radical resection" may be an adequate description of this surgical approach. Since the dissemination of--macroscopically not detectable--tumor cells represents the major mannerism of every metastatic disease, any local therapy appears to be a discrepancy. However, in most cases the rationale of surgery for lung metastases is the lack of effective systemic therapy and the low morbidity of surgery, along with up to 60% 5-year survival rates.

Tumor-induced osteomalacia with elevated fibroblast growth factor 23: a case of phosphaturic mesenchymal tumor mixed with connective tissue variants and review of the literature.

Science.gov (United States)

Hu, Fang-Ke; Yuan, Fang; Jiang, Cheng-Ying; Lv, Da-Wei; Mao, Bei-Bei; Zhang, Qiang; Yuan, Zeng-Qiang; Wang, Yan

2011-11-01

Tumor-induced osteomalacia (TIO), or oncogenic osteomalacia (OOM), is a rare acquired paraneoplastic disease characterized by renal phosphate wasting and hypophosphatemia. Recent evidence shows that tumor-overexpressed fibroblast growth factor 23 (FGF23) is responsible for the hypophosphatemia and osteomalacia. The tumors associated with TIO are usually phosphaturic mesenchymal tumor mixed connective tissue variants (PMTMCT). Surgical removal of the responsible tumors is clinically essential for the treatment of TIO. However, identifying the responsible tumors is often difficult. Here, we report a case of a TIO patient with elevated serum FGF23 levels suffering from bone pain and hypophosphatemia for more than three years. A tumor was finally located in first metacarpal bone by octreotide scintigraphy and she was cured by surgery. After complete excision of the tumor, serum FGF23 levels rapidly decreased, dropping to 54.7% of the preoperative level one hour after surgery and eventually to a little below normal. The patient's serum phosphate level rapidly improved and returned to normal level in four days. Accordingly, her clinical symptoms were greatly improved within one month after surgery. There was no sign of tumor recurrence during an 18-month period of follow-up. According to pathology, the tumor was originally diagnosed as "lomangioma" based upon a biopsy sample, "proliferative giant cell tumor of tendon sheath" based upon sections of tumor, and finally diagnosed as PMTMCT by consultation one year after surgery. In conclusion, although an extremely rare disease, clinicians and pathologists should be aware of the existence of TIO and PMTMCT, respectively.

En bloc resection for treatment of tumor-induced osteomalacia: a case presentation and a systematic review.

Science.gov (United States)

Meng, Tong; Zhou, Wang; Li, Bo; Yin, Huabin; Li, Zhenxi; Zhou, Lei; Kong, Jinhai; Yan, Wangjun; Yang, Xinghai; Liu, Tielong; Song, Dianwen; Xiao, Jianru

2015-05-08

Tumor-induced osteomalacia (TIO) is a rare disorder, which is commonly found in craniofacial locations and in the extremities. To the best of our knowledge, only 16 cases have been described in the spine, and this is the first report to describe a case of patient with TIO in the thoracic spine combined with a mesenchymal hamartoma which had confused the therapeutic strategies to date. We report the case of a 60-year-old patient with hypophosphatemia and presented with limb weakness. Treating with phosphate did not correct the hypophosphatemia and an (111)In pentetreotide scintigraphy (octreotide scan) revealed an increased uptake at the right forearm. The tumor was resected totally, and the histopathology revealed a mesenchymal hamartoma, but we noticed that hypophosphatemia was not corrected after the tumor resection. Then a whole-body magnetic resonance imaging (WB-MRI) was performed and the results revealed tumorous tissues at the right T1 vertebral pedicle. The tumor was removed with an en bloc method, and the pathology showed phosphaturic mesenchymal tumor. Follow-up at 1 year after surgery revealed no recurrence, and the serum phosphorus level of the patient was normal. Tumor-induced osteomalacia is exceedingly rare with only 16 cases in spine published in the literature. It is difficult to find and leads to years of suffering debilitating complications. In this regard, the WB-MRI is a better method to locate the real tumor. Treating with phosphate can only relieve symptoms, and a complete surgical removal remains the gold standard treatment.

Successful Insular Glioma Removal in a Deaf Signer Patient During an Awake Craniotomy Procedure.

Science.gov (United States)

Metellus, Philippe; Boussen, Salah; Guye, Maxime; Trebuchon, Agnes

2017-02-01

Resection of tumors located within the insula of the dominant hemisphere represents a technical challenge because of the complex anatomy, including the surrounding vasculature, and the relationship to functional (motor and language) structures. We report here the case of a successful resection of a left insular glioma in a native deaf signer during an awake craniotomy. The patient, a congenitally deaf right-handed patient who is a native user of sign language, presented with a seizure 1 week before he was referred to our department. Magnetic resonance imaging revealed a left heterogeneous insular tumor enhanced after intravenous gadolinium infusion. Because of its deep and dominant hemisphere location, an awake craniotomy was decided. The patient was evaluated intraoperatively using object naming, text reading, and sign repetition tasks. An isolated inferior frontal gyrus site evoked repeated object naming errors. A transopercular parietal approach was performed and allowed the successful removal of the tumor under direct electric stimulation and electrocorticography. To our knowledge, this is the first report of successful removal of a left insular tumor without any functional sequelae in a native deaf signer using intraoperative direct cerebral stimulation during an awake craniotomy. The methodology used also provides the first evidence of the actual anatomo-functional organization of language in deaf signers. Copyright © 2016 Elsevier Inc. All rights reserved.

Bronchoscopic management of a rare benign endobronchial tumor

Directory of Open Access Journals (Sweden)

K. Madan

2012-09-01

Full Text Available Benign endobronchial tumors are uncommon. Bronchoscopic removal is the preferred modality of treatment although surgery may be required in some cases. Rigid bronchoscopy is usually recommended in the management of these tumors. However, flexible bronchoscopy is also used in many centers. We present a case of endobronchial lipoma, where an unusual complication during flexible bronchoscopic resection using snare forceps necessitated urgent rigid bronchoscopy. This case highlights the importance of rigid bronchoscopy in the management of endobronchial tumors. We believe that with a large benign endobronchial tumor in tracheal or main-stem bronchus, physicians should initially employ rigid bronchoscopy, switching to flexible if more peripheral treatment is required. Resumo: Os tumores endobrônquicos benignos são raros. A remoção broncoscópica é a modalidade de tratamento preferida, embora a cirurgia possa ser necessária em alguns casos. A broncoscopia rígida é geralmente considerada a modalidade preferencial na abordagem destes tumores. No entanto, a broncoscopia flexível também é utilizada em muitos centros. Apresentamos um caso de lipoma endobrônquico, onde uma complicação invulgar durante a ressecção broncoscópica flexível utilizando pinças de laço necessitou de uma urgente broncoscopia rígida. Neste caso, destaca-se a importância da broncoscopia rígida na abordagem dos tumores endobrônquicos. Acreditamos que com um tumor endobrônquico benigno de grandes dimensões da traqueia ou do brônquio principal, os médicos devem inicialmente utilizar a broncoscopia rígida, alternando para a flexível, se for necessário um tratamento mais periférico. Keywords: Bronchoscopy, Lipoma, Endobronchial tumor, Electrocautery, Interventional pulmonology, Palavras-chave: Broncoscopia, Lipoma, Tumor endobrônquico, Electrocautério, Pneumologia interventiva

Tumor-induced hypophosphatemic osteomalacia Report of a cases associated with peripheral giant cell granuloma of gingiva

International Nuclear Information System (INIS)

Lee, Sang Rae; Kim, Won Chul; Lee, Sang Hoon; Kim, Mee Kyung; Lee, Byung Do

1987-01-01

The authors observed a patient who referred to the Department of Oral Radiology, due to diffuse skeletal pain, muscular weakness and unknown tumor mass on the buccal gingiva of upper right molar region. The patient was found to have peripheral reparative giant cell granuloma and osteomalacia. After removal of the tumor, the clinical, radiologic, and laboratory findings of the patient was rapidly normalized with remarkable improvement of bone pain. The results were as follows: 1. After removal of the tumor, the patient improved the clinical findings such as bone pain, trismus, muscular weakness and he could walk. 2. In postoperative x-ray findings at 1 and 2 months intervals, the lamina dura of all dentition and bony trabeculae in upper and lower arches were regenerating and the bone density increased. 3. In periodic recall check, no occurrence of osteomalacia was existed and the laboratory findings of the patient showed gradual improvement.

Tumor-induced hypophosphatemic osteomalacia Report of a cases associated with peripheral giant cell granuloma of gingiva

Energy Technology Data Exchange (ETDEWEB)

Lee, Sang Rae; Kim, Won Chul; Lee, Sang Hoon; Kim, Mee Kyung; Lee, Byung Do [Dept. of Oral Radiology, College of Dentistry, Kyung Hee University, Seoul (Korea, Republic of)

1987-11-15

The authors observed a patient who referred to the Department of Oral Radiology, due to diffuse skeletal pain, muscular weakness and unknown tumor mass on the buccal gingiva of upper right molar region. The patient was found to have peripheral reparative giant cell granuloma and osteomalacia. After removal of the tumor, the clinical, radiologic, and laboratory findings of the patient was rapidly normalized with remarkable improvement of bone pain. The results were as follows: 1. After removal of the tumor, the patient improved the clinical findings such as bone pain, trismus, muscular weakness and he could walk. 2. In postoperative x-ray findings at 1 and 2 months intervals, the lamina dura of all dentition and bony trabeculae in upper and lower arches were regenerating and the bone density increased. 3. In periodic recall check, no occurrence of osteomalacia was existed and the laboratory findings of the patient showed gradual improvement.

[Usefulness of neuroendoscopy and a neuronavigator for removal of clival chordoma].

Science.gov (United States)

Miyagi, A; Maeda, K; Sugawara, T

1998-02-01

We report a case of large clival chordoma. The patient was a 56-year-old male who was admitted to our hospital with left eye ptosis and diplopia of 2 months duration. On admission, neurological examinations revealed oculomotor nerve palsy of the left eye. Skull radiographs with polytomographs demonstrated marked destruction of the clivus. A plain computed tomography (CT) scan revealed a large iso-attenuated mass in the clivus, extending anteriorly into the sphenoidal sinus, superiorly into the suprasellar cistern, bilaterally into the petrous apex, posteriorly into the prepontine cistern and caudally into the foramen magnum. An enhanced CT scan demonstrated a slightly enhanced tumor. A high-resolution bone-window CT scan revealed marked destruction of the clivus and bilateral petrous apex. Magnetic resonance imaging (MRI) scans disclosed a large enhanced mass extending superiorly into the suprasellar cistern, bilaterally into the petrous apex and inferiorly into the foramen magnum. The tumor extended so widely that we decided on a one-stage operation via a transsphenoidal sublabial transseptal approach and transoral transpalatal approach. At surgery, we employed a neuronavigator and Codman 4-mm rigid neuroendoscope with 0 degree, 30 degrees and 70 degrees angled lenses. The tumor was very soft and suckable, and could be easily removed by applying CUSA, a pituitary curette and suction. The neuronavigator was particularly useful because the surgeon had a real-time two-dimensional representation of the position of the tip of this device in the corresponding imaging space intraoperatively. The neuroendoscope also proved useful, since remnant tumor tissues that could not be seen under an operating microscope were frequently recognized near or around the entrance of the tumor cavity, cavernous sinus region and petroclival junction area. The surgeon was able to remove these remnants safely by checking on the neuroendoscope monitor. The tumor was excised completely. The

Clinical application of preoperative embolization of tumor feeding artery combined with intraoperative balloon occlusion of the abdominal aorta in the resection of sacral tumors

International Nuclear Information System (INIS)

Chen Wenhua; Wang Qi; He Zhongming; Zhou Jian; Wang Yimin; Wang Jie

2012-01-01

Objective: To investigate the clinical application of preoperative embolization of tumor feeding artery combined with intraoperative balloon occlusion of the abdominal aorta in performing the surgical resection of sacral tumors. Methods: Conventional surgical excision of sacral tumors was employed in 24 patients with sacral tumors (control group), while preoperative embolization of tumor feeding artery combined with intraoperative balloon occlusion of the abdominal aorta was carried out in 32 patients with sacral tumors (study group). The operation time, blood loss during the surgery and the one-year recurrence rate of both groups were documented, and the results were statistically analyzed. Results: Angiography showed that in the study group the sacral tumors were supplied by several vessels, and these feeding arteries were occluded separately. The tumors were successfully removed in all patients with the help of intraoperative balloon occlusion of the abdominal aorta. During the surgery, the surgical area was clearly exposed and the blood loss wa remarkably reduced. After the surgery, no ectopic vascular embolization, renal ischemia, limb ischemia or other complications occurred. Statistically significant difference in the operation time, blood loss during the surgery and the one-year recurrence rate existed between the two groups (P<0.05). Conclusion: Preoperative embolization of tumor feeding artery combined with intraoperative balloon occlusion of the abdominal aorta can effectively shorten the operation time, reduce the blood loss during the surgery and provide a clear surgical field, and thus the surgical safety can be significantly ensured. (authors)

Osteomalacia por tumor secretor de FGF-23 Ostemalacia due to a tumor secreting FGF-23

Directory of Open Access Journals (Sweden)

Ariel Sánchez

2013-02-01

Full Text Available Se presenta un caso de osteomalacia oncogénica en un varón de 50 años, con fuertes dolores óseos y gran debilidad muscular durante 4 años. Tenía varias deformidades vertebrales dorsales en cuña, fracturas en ambas ramas iliopubianas y en una rama isquiopubiana, y una zona de Looser en la meseta tibial derecha. Se localizó un tumor de 2 cm de diámetro en el hueco poplíteo derecho mediante centellograma con octreótido marcado con tecnecio. El tumor fue extirpado quirúrgicamente. La microscopía mostró un tumor mesenquimático fosfatúrico, de tejido conectivo mixto. La inmunotinción demostró FGF-23. Hubo rápida mejoría, con consolidación de las fracturas pelvianas y de la pseudofractura tibial y normalización de las alteraciones bioquímicas.A case of oncogenic osteomalacia in a 50-year-old male is here presented. He suffered severe bone pain and marked muscular weakness of 4 years' duration. There were several vertebral deformities in the thoracic spine, bilateral fractures of the iliopubic branches, another fracture in the left ischiopubic branch, and a Looser's zone in the right proximal tibia. An octreotide-Tc scan allowed to identify a small tumor in the posterior aspect of the right knee. It was surgically removed. Microscopically, it was a phosphaturic mesenchymal tumor-mixed connective tissue (PMT-MCT. Expression of FGF-23 was documented by immune-peroxidase staining. There was rapid improvement, with consolidation of the pelvic fractures and the tibial pseudo-fracture. The laboratory values returned to normal.

Tumor mammae - a dignostic challenge

International Nuclear Information System (INIS)

Roekke, O.; Due, J.; Saeboee-Larsen, J.; Stoermer, J.; Eide, T.J.; Rosenlund, A.F.

1990-01-01

The study includes all patients referred for surgical examination with suspected breast cancer (n=439) during a single year. All women were examined clinically. Mammography was performed in 372 cases (88%), and 195 (46%) women underwent biopsy. 37 (8.7%) of the admitted women had breast cancer, which was verified by histological examination. This corresponds to an incidence rate of 0.8 per 1000 women, and an incidence rate of 2.0 per 1000 in women above 40 years. In four patients who were not suspected of having breast cancer after clinical examination, breast cancer was revealed by mammography. However, mammography alone is insufficient, since three mammograms were false negative in cases where cancers were diagnosed clinically. Therefore, both mammography and clinical investigation are necessary in the examination of women with breast tumors. When breast cancer cannot be ruled out by physical and mammographic examination, a representative specimen of the tumor should be removed for histological or cytological examination. 9 refs., 1 fig., 6 tabs

SURGERY FOR TUMORS OF THE FOURTH VENTRICLE: THE CHARACTERISTICS OF ACCESSES AND THE ROLE OF ENDOSCOPIC TECHNIQUES

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V. B. Karakhan

2012-01-01

Full Text Available The use of current accesses to the tumors of the fourth ventricle, which fill and compress from the outside its cavity, was assessed in 28 patients. Original associated endomicrosurgical techniques were used. Two groups and five topographic types of fourth ventricle tumors are identified. Basic accesses — telovelar and supracerebellar — eliminate the necessity of dissecting the vermis cerebelli. The key endoscopic technique is to provide a simultaneous survey of the lower and upper poles of a tumor during its removal. The technique of trochlear removal of metastatic nodes from the fourth ventricle is shown. The benefits of endoscopic techniques are to early examine the vulnerable vascular and neural structures blocked by a tumor at the access step; to reduce the volume of an access itself and the traction of cerebellar and truncal structures; to completely survey the Sylvian aqueduct without additionally displacing or dissecting the vermis cerebelli; to maintain optical sharpness within sight of differently remote microstructures. Overall, incorporation of the endoscopic method realizes the principle of mini-invasive neurosurgery. 

Application of Adoptive T-Cell Therapy Using Tumor Antigen-Specific T-Cell Receptor Gene Transfer for the Treatment of Human Leukemia

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Toshiki Ochi

2010-01-01

Full Text Available The last decade has seen great strides in the field of cancer immunotherapy, especially the treatment of melanoma. Beginning with the identification of cancer antigens, followed by the clinical application of anti-cancer peptide vaccination, it has now been proven that adoptive T-cell therapy (ACT using cancer antigen-specific T cells is the most effective option. Despite the apparent clinical efficacy of ACT, the timely preparation of a sufficient number of cancer antigen-specific T cells for each patient has been recognized as its biggest limitation. Currently, therefore, attention is being focused on ACT with engineered T cells produced using cancer antigen-specific T-cell receptor (TCR gene transfer. With regard to human leukemia, ACT using engineered T cells bearing the leukemia antigen-specific TCR gene still remains in its infancy. However, several reports have provided preclinical data on TCR gene transfer using Wilms' tumor gene product 1 (WT1, and also preclinical and clinical data on TCR gene transfer involving minor histocompatibility antigen, both of which have been suggested to provide additional clinical benefit. In this review, we examine the current status of anti-leukemia ACT with engineered T cells carrying the leukemia antigen-specific TCR gene, and discuss the existing barriers to progress in this area.

Tumor location and IDH1 mutation may predict intraoperative seizures during awake craniotomy.

Science.gov (United States)

Gonen, Tal; Grossman, Rachel; Sitt, Razi; Nossek, Erez; Yanaki, Raneen; Cagnano, Emanuela; Korn, Akiva; Hayat, Daniel; Ram, Zvi

2014-11-01

Intraoperative seizures during awake craniotomy may interfere with patients' ability to cooperate throughout the procedure, and it may affect their outcome. The authors have assessed the occurrence of intraoperative seizures during awake craniotomy in regard to tumor location and the isocitrate dehydrogenase 1 (IDH1) status of the tumor. Data were collected in 137 consecutive patients who underwent awake craniotomy for removal of a brain tumor. The authors performed a retrospective analysis of the incidence of seizures based on the tumor location and its IDH1 mutation status, and then compared the groups for clinical variables and surgical outcome parameters. Tumor location was strongly associated with the occurrence of intraoperative seizures. Eleven patients (73%) with tumor located in the supplementary motor area (SMA) experienced intraoperative seizures, compared with 17 (13.9%) with tumors in the other three non-SMA brain regions (p awake craniotomy compared with patients who have a tumor in non-SMA frontal areas and other brain regions. The IDH1 mutation was more common in SMA region tumors compared with other brain regions, and may be an additional risk factor for the occurrence of intraoperative seizures.

[Intracranial collision tumor--A case report (author's transl)].

Science.gov (United States)

Fukaya, T; Yoshida, J; Banno, T; Kageyama, N; Ito, M

1976-06-01

A 21-year-old man with nasopharyngeal tumor was first admitted to the Nagoya University Hospital on April 15, 1972. He had difficulty in speaking and swallowing, and developed double vision prior to admission. A soft and yellow tumor was found in the nasopharynx and revealed typical features of chordoma. The patient underwent Co60 irradiation after the operation. On January 25, 1973, the patient developed double vision of severe degree. Microscopic examination of the specimen which was obtained at the time of the second operation in February 9, 1973, disclosed a coexistence (collision) of chordoma and hemangioblastoma. The two different tumors were situated in juxtaposition on histological examination. Co60 irradiation was added during his second hospitalization. Three months after the second operation, he developed symptoms of meningitis and was hospitalized for the third time on June 3, 1973, at which time the tumor tissue extended through the right frontal and middle fossa. The third operation was done with frontal craniotomy and tumor was partially removed. The histological diagnosis was hemangioblastoma. Postoperatively the patient went downhill and died on September 19, 1973. The report of a collision tumor of intracranial chordoma and hemangioblastoma is not found in the previous literature. There have been many theories as to the origin of collision tumor. Some investigators have proposed that the existence of hyperplastic blood vessels within the glioblastoma is responsible for the collision tumor of sarcoma and glioblastoma. Since the advent of radiotherapy, several examples of sarcoma have been discovered at postmortem examination in patient irradiated for treatment of cerebral neoplasm, both gliogeneous and nongliogenous, suggesting a possible relationship between the tumor and the radiation therapy. In our case, the chordoma showed neither hyperplastic blood vessels nor malignant pattern on histological examination. It was suspected that post

Skull base tumor model.