Sample records for national wilms tumor

  1. Wilms Tumor (United States)

    Wilms tumor is a rare type of kidney cancer. It causes a tumor on one or both kidneys. It usually affects ... are at risk should be screened for Wilms tumor every three months until they turn eight. Symptoms ...

  2. Wilms Tumor (United States)

    ... Wilms tumor is 1 pound at diagnosis. Some children also may have nausea, stomach pain, high blood pressure (hypertension), blood in the urine, loss of appetite, or fever. Even though Wilms tumors often are ...

  3. Wilms' Tumor (United States)

    ... team and have training in child development, recreation, psychology or social work. If your child must remain ... conditions/wilms-tumor/basics/definition/CON-20043492 . Mayo Clinic Footer Legal Conditions and Terms Any use of ...

  4. Max Wilms and his tumor. (United States)

    Raffensperger, John


    The most common cancer of the kidney in infants and children is named for Max Wilms, a German surgeon. How did this eponym come about? There were excellent reviews of this lesion before Wilms, a second year surgical assistant, published "Die Mischgeschwulste Der Niere" or The Mixed Tumors of the Kidney in 1899. At thirty two years of age, he demonstrated a masterful knowledge of pathology and embryology. Wilms' career was cut short when he became septic after operating on a prisoner of war during WWI. The survival rate for children with Wilms tumor was dismal until William Ladd, at the Boston Children's hospital introduced rational surgical treatment. By mid century, Robert Gross achieved a 47% survival rate with surgery combined with postoperative radiation. Sydney Farber treated Wilms tumors with Actinomycin-d and opened the door to cancer chemotherapy. With protocols developed by the National Wilms Tumor Study Group, the survival rate of children with Wilms tumors reached 90% by the end of the twentieth century.

  5. Wilms tumors: genotypes and phenotypes

    NARCIS (Netherlands)

    H. Segers (Heidi)


    textabstractWilms tumor, or nephroblastoma, represents about 90% of all pediatric renal tumors and about 7% of all pediatric malignancies. Most Wilms tumors are unilateral, although in 5-10 % of the patients both kidneys are infected. Wilms tumor typically occurs between the age of 2 and 4 years,

  6. Wilms tumors: genotypes and phenotypes


    Segers, Heidi


    textabstractWilms tumor, or nephroblastoma, represents about 90% of all pediatric renal tumors and about 7% of all pediatric malignancies. Most Wilms tumors are unilateral, although in 5-10 % of the patients both kidneys are infected. Wilms tumor typically occurs between the age of 2 and 4 years, and 90% of the patients are diagnosed before the age of 7 years. Above the age of 18 years, Wilms tumor is rare, representing less than 1% of all adult renal tumors. Most pediatric Wilms tumor patien...

  7. Drugs Approved for Wilms Tumor (United States)

    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.

  8. What Is Wilms Tumor? (United States)

    ... children. It is named after Max Wilms, a German doctor who wrote one of the first medical ... Cancer Atlas Press Room Cancer Statistics Center Volunteer Learning Center Follow Us Twitter Facebook Instagram Cancer Information, ...

  9. Identification and Management of High Risk Wilms Tumors

    NARCIS (Netherlands)

    J.S. Dome (Jeffrey)


    textabstractPediatric renal tumors comprise approximately 5% of malignancies in children under 15 years old and 3.6% of malignancies in children under 20 years old (1). Among 9731 patients registered with the National Wilms Tumor Study Group (NWTSG) (1969-2002), Wilms tumor composed the vast majorit

  10. Laparoscopic Nephrectomy for Wilms Tumor


    Andolfi C; Randi B; Ruggeri G.; Lima M.


    Wilms tumor is the most frequent primary renal malignancy in children. The surgical resection is traditionally performed through laparotomy. The advent of laparoscopic surgery for benign renal lesions has led the surgeon to use a minimal invasive approach for the nephroblastoma. We describe a 9-months-old girl who presented with a left renal mass. A laparoscopic resection of the tumor with left radical nephroureterectomy was performed. The specimens were removed in an endoscopic bag through a...

  11. Laparoscopic Nephrectomy for Wilms Tumor

    Directory of Open Access Journals (Sweden)

    Andolfi C


    Full Text Available Wilms tumor is the most frequent primary renal malignancy in children. The surgical resection is traditionally performed through laparotomy. The advent of laparoscopic surgery for benign renal lesions has led the surgeon to use a minimal invasive approach for the nephroblastoma. We describe a 9-months-old girl who presented with a left renal mass. A laparoscopic resection of the tumor with left radical nephroureterectomy was performed. The specimens were removed in an endoscopic bag through a low suprapubic incision. The patient had an uncomplicated recovery. Minimally invasive techniques may be considered for resection of nephroblastoma if oncologic principles are carefully followed.

  12. Paraneoplastic Cushing Syndrome Due To Wilm's Tumor. (United States)

    Faizan, Mahwish; Manzoor, Jaida; Saleem, Muhammad; Anwar, Saadia; Mehmood, Qaiser; Hameed, Ambreen; Ali, Agha Shabbir


    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, along with histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed.

  13. Anatomical basis for Wilms tumor surgery

    Directory of Open Access Journals (Sweden)

    Trobs R


    Full Text Available Wilms tumor surgery requires meticulous planning and sophisticated surgical technique. Detailed anatomical knowledge can facilitate the uneventful performance of tumor nephrectomy and cannot be replaced by advanced and sophisticated imaging techniques. We can define two main goals for surgery: (1 exact staging as well as (2 safe and complete resection of tumor without spillage. This review aims to review the anatomical basis for Wilms tumor surgery. It focuses on the surgical anatomy of retroperitoneal space, aorta, vena cava and their large branches with lymphatics. Types and management of vascular injuries are discussed.

  14. Innovations in the management of Wilms' tumor. (United States)

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


    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.

  15. Intrarenal neuroblastoma mimics Wilms' tumor; Neuroblastoma intrarenal mimetizando tumor de Wilms

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    Muniz, Maria T. Cartaxo; Soares, Andrezza B.; Freitas, Elizabete M. [Pernambuco Univ., Recife, PE (Brazil). Hospital Universitario Oswaldo Cruz. Inst. de Ciencias Biologicas]. E-mail:; Araujo, Marcela [Centro Infantil Boldrini, Campinas, SP (Brazil). Lab. de Biologia Molecular; Pureza, Leda M.M.; Morais, Adriana; Antunes, Consuelo; Salles, Terezinha de J. Marques; Borges, Josenilda C.; Morais, Vera L.L. de [Pernambuco Univ., Recife, PE (Brazil). Hospital Universitario Oswaldo Cruz; Romualdo Filho, Jose [Pernambuco Univ., Recife, PE (Brazil). Hospital Universitario Oswaldo Cruz. Centro Integrado de Anatomia Patologica; Magalhaes, Mario H. [Instituto Nacional de Cancer, Rio de Janeiro, RJ (Brazil). Dept. de Patologia


    This work reports the case history of a child with intrarenal neuroblastoma, initially diagnosed as Wilms' tumor. The patient, a one year and three months old girl, presented a hard abdominal mass on the left flank that extended to the meso gastric region, plus fever and paleness. The ultrasound of the entire abdomen revealed an intrarenal mass. Biopsy with fine needle in many points of the tumor revealed Wilms' tumor. The scarcely of the material, however, made immunohistoquemistry impossible at that moment. Because of the child's severe condition the SIOP protocol was started. As no clinical response was observed, an exploratory laparotomy was indicated with partial resection of the tumor and bone marrow aspiration (MO). The histopathologic study revealed a malignant neoplasia of small cells, poorly differentiated. IHQ was negative for WT-1 and positive for NB-84, synaptofisin, cromogranine. N-myc amplification was observed by molecular biology. The bone marrow aspiration identified metastatic small round cells infiltration. Intrarenal neuroblastoma is a rare entity that clinically and radiographically resembles Wilms' tumor. The objective of this case report is to show the importance of immunohistochemical and molecular analysis in the diagnosis of intrarenal neuroblastoma. (author)

  16. What Are the Key Statistics about Wilms Tumor? (United States)

    ... Survival rates for Wilms tumor, by stage and histology .” Visit the American Cancer Society’s Cancer Statistics Center ... Our Volunteers More ACS Sites Bookstore Shop Cancer Atlas Press Room Cancer Statistics Center Volunteer Learning Center ...

  17. Uniparental disomy occurs infrequently in Wilms tumor patients

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    Grundy, P.; Wilson, B.; Telzerow, P.; Zhou, W.; Paterson, M.C. (Univ. of Alberta, Edmonton (Canada))


    Wilms tumors commonly exhibit loss of heterozygosity for polymorphic DNA markers located on the short arm of chromosome 11 at band p15. In some instances, the deleted region does not include 11p13, the location of the WT1 gene, suggesting the existence of a second Wilms tumor gene on 11p. Both the exclusive loss of the maternally derived allele in Wilms tumors and the recent description of constitutional paternal isodisomy for this region in patients with either the Beckwith-Wiedemann syndrome (BWS) or isolated hemihypertrophy have suggested that this second locus is subject to sex-specific genomic imprinting. Given that one of these isodisomic patients had minimal congenital anomalies (hemihypertrophy), the authors hypothesized that a proportion of Wilms tumors which had not lost heterozygosity for 11p markers (about 60% of all cases) might have arisen consequent to 11p paternal heterodisomy and that patients constitutionally homozygous at 11p15 might harbor paternal isodisomy. They have analyzed 40 Wilms tumor cases to determine the parental origin of the child's 11p15 alleles. Paternal heterodisomy could be excluded in all 28 unilateral and 8/9 bilateral potential candidates. It is intriguing that somatic mosaicism for 11p paternal isodisomy was detected in one child with bilateral Wilms tumor and macroglossia. Isodisomy could only be excluded in one of the three possible cases. Thus, 11p paternal hetero- and isodisomy appear to be uncommon causes of non-anomaly-associated Wilms tumors but may be more frequent in Wilms tumor patients with BWS-associated anomalies. 44 refs., 1 fig., 2 tabs.

  18. CITED1 Expression in Wilms' Tumor and Embryonic Kidney

    Directory of Open Access Journals (Sweden)

    Harold N. Lovvorn, III


    Full Text Available Wilms' tumors, or nephroblastomas, are thought to arise from abnormal postnatal retention and dysregulated differentiation of nephrogenic progenitor cells that originate as a condensed metanephric mesenchyme within embryonic kidneys. We have previously shown that the transcriptional regulator CITED1 (CBP/p300-interacting transactivators with glutamic acid [E]/aspartic acid [D]-rich C-terminal domain is expressed exclusively in these nephrogenic progenitor cells and is downregulated as they differentiate to form nephronic epithelia. In the current study, we show that CITED1 expression persists in blastemal cell populations of both experimental rat nephroblastomas and human Wilms' tumors, and that primary human Wilms' tumors presenting with disseminated disease show the highest level of CITED1 expression. Unlike the predominantly cytoplasmic subcellular localization of CITED1 in the normal developing kidney, CITED1 is clearly detectable in the nuclear compartment of Wilms' tumor blastema. These findings indicate that CITED1 is a marker of primitive blastema in Wilms' tumors and suggest that persistent expression and/or altered subcellular localization of CITED1 in the condensed metanephric mesenchyme could play a role in Wilms' tumor initiation and pathogenesis.

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

    Directory of Open Access Journals (Sweden)

    Caridad Verdecia Cañizares


    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.

  20. Perirenal hemorrhage as first presentation of Wilms tumor

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    Byerly, Douglas [The Ohio State University College of Medicine and Public Health, Columbus, Ohio (United States); Coley, Brian [Columbus Children' s Hospital, Department of Radiology, Columbus, OH (United States); Ruymann, Frederick [Columbus Children' s Hospital, Section of Hematology/Oncology, Department of Pediatrics, Columbus, Ohio (United States)


    Wilms tumor typically presents as an abdominal mass, though occasionally patients present with other manifestations. We report a case of a child presenting with a perirenal hemorrhage and an initially occult Wilms tumor, found only on subsequent renal arteriography. Symptoms in this patient were caused by the presence of perirenal and subcapsular hemorrhage rather than the tumor itself. Despite an unusual presentation, we need to consider underlying neoplasia in children with renal hemorrhage and the absence of a history of trauma. Follow-up studies might help clarify initial negative imaging results. (orig.)

  1. SIX2 Effects on Wilms Tumor Biology

    Directory of Open Access Journals (Sweden)

    Janene Pierce


    Full Text Available Wilms tumor (WT blastema retains gene expression profiles characteristic of the multipotent nephron progenitor pool, or cap mesenchyme (CM, in the developing kidney. As a result, WT blastema and the CM are believed to represent contextual analogues of one another. Sine oculis homeobox 2 (SIX2 is a transcription factor expressed specifically in the CM, provides a critical mechanism for CM self-renewal, and remains persistently active in WT blastema, although its purpose in this childhood malignancy remains unclear. We hypothesized that SIX2, analogous to its function in development, confers a survival pathway to blastema, the putative WT stem cell. To test its functional significance in WT biology, wild-type SIX2 was overexpressed in the human WT cell line, WiT49. After validating this model, SIX2 effects on anchorage-independent growth, proliferation, invasiveness, canonical WNT pathway signaling, and gene expression of specific WNT pathway participants were evaluated. Relative to controls, WiT49 cells overexpressing SIX2 showed significantly enhanced anchorage-independent growth and early-passage proliferation representing surrogates of cell survival. Interestingly, overexpression of SIX2 generally repressed TCF/LEF-dependent canonical WNT signaling, which activates and coordinates both differentiation and stem pathways, but significantly heightened canonical WNT signaling through the survivin promoter, a mechanism that exclusively maintains the stem state. In summary, when overexpressed in a human WT cell line, SIX2 enhances cell survival and appears to shift the balance in WNT/β-catenin signaling away from a differentiation path and toward a stem cell survival path.

  2. Mutational Analysis of the Wilms' Tumor (WTI) Gene. (United States)

    King-Underwood, L; Pritchard-Jones, K


    Mutations of the Wilms' tumor (WT1) gene have been shown to underlie a proportion of cases of Wilms' tumor, an embryonal kidney cancer occurring mainly in childhood. The WTl gene comprtses ten exons spanning approx 50 kb of genomrc DNA. The messenger RNA is approx 3 kb in length and encodes a zinc finger protein. The four zinc fingers, which he at the C-terminal end of the protein, are encoded by separate exons 7-10. The 5' end of the gene is extremely GC-rich, with areas approaching a 70% GC content. This makes this region difficult to amplify in polymerase chain reactions.

  3. Lin28 sustains early renal progenitors and induces Wilms tumor. (United States)

    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


    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.

  4. Structure of the Wilms Tumor Suppressor

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    Stoll, R.; Lee, B.M.; Debler, E.W.; Laity, J.H.; Wilson, I.A.; Dyson, H.J.; Wright, P.E.


    The zinc finger domain of the Wilms tumor suppressor protein (WT1) contains four canonical Cys{sub 2}His{sub 2} zinc fingers. WT1 binds preferentially to DNA sequences that are closely related to the EGR-1 consensus site. We report the structure determination by both X-ray crystallography and NMR spectroscopy of the WT1 zinc finger domain in complex with DNA. The X-ray structure was determined for the complex with a cognate 14 base-pair oligonucleotide, and composite X-ray/NMR structures were determined for complexes with both the 14 base-pair and an extended 17 base-pair DNA. This combined approach allowed unambiguous determination of the position of the first zinc finger, which is influenced by lattice contacts in the crystal structure. The crystal structure shows the second, third and fourth zinc finger domains inserted deep into the major groove of the DNA where they make base-specific interactions. The DNA duplex is distorted in the vicinity of the first zinc finger, with a cytidine twisted and tilted out of the base stack to pack against finger 1 and the tip of finger 2. By contrast, the composite X-ray/NMR structures show that finger 1 continues to follow the major groove in the solution complexes. However, the orientation of the helix is non-canonical, and the fingertip and the N terminus of the helix project out of the major groove; as a consequence, the zinc finger side-chains that are commonly involved in base recognition make no contact with the DNA. We conclude that finger 1 helps to anchor WT1 to the DNA by amplifying the binding affinity although it does not contribute significantly to binding specificity. The structures provide molecular level insights into the potential consequences of mutations in zinc fingers 2 and 3 that are associated with Denys-Drash syndrome and nephritic syndrome. The mutations are of two types, and either destabilize the zinc finger structure or replace key base contact residues.

  5. Clinical Application of {sup 18}F-FDG PET in Wilms Tumor

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    Seok, Ju Won [College of Medicine, Chung-Ang University, Seoul (Korea, Republic of)


    Wilms Tumor is a great therapeutic success story within pediatric oncology. Therefore, accurate initial staging is needed to assess tumor spread and to assign patients appropriately to the different risk branches. However, it is recognized that FDG-PET can provide useful information about tumor and has better accuracy than CT and MRI for staging, but its role in Wilms tumor is unclear. According to clinical research data, FDG PET may be useful for the management of selected patients with Wilms tumors.

  6. Wilms tumor arising in extracoelomic paravertebral soft tissues.

    LENUS (Irish Health Repository)

    Mulligan, Linda


    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.

  7. Infrequent mutation of the WT1 gene in 77 Wilms' Tumors


    Gessler, Manfred; König, A.; Arden, K; Grundy, P; Orkin, S H; Sallan, S; Peters, C.; Ruyle, S.; Mandell, J.; Li, F.; Cavenee, W; Bruns, G A


    Homozygous deletions in Wilms' tumor DNA have been a key step in the identification and isolation of the WTI gene. Several additional loci are also postulated to contribute to Wilms' tumor formation. To assess the frequency of WTI alterations we have analyzed the WTI locus in a panel of 77 Wilms' tumors. Eight tumors showed evidence for large deletions of several hundred or thousand kilobasepairs of DNA, some of which were also cytogenetically detected. Additional intragenic mutations were de...

  8. [Wilms' tumor, multiple intestinal parasitosis and typhoid fever]. (United States)

    Franco Vega, G; Llausás Vargas, A; Kumate, J


    The case was that of a 21-month-old infant who presented a great inoperable Wilm's tumor that was treated with vincristine to the point of practically disappearing. Severe typhoid fever that was complicated by multiple intestinal parasitoses (ascariasis, trichuriasis, giardiasis and strongyloidiasis) appeared. Possibly, tumoral necrosis, salmonellosis and the parasitoses formed a sac that opened to the hepatic angle of the colon. Finally, multiple liver metastases were discovered and confirmed pathologically. The patient died 36 hours after surgical reexamination and liver biopsies, from causes not clearly explained. Comments are made on the diagnostic problems originated by rareness of the association of typhoid fever resistant to chloramphenicol, intestinal parasitoses and a great Wilms' tumor and the possible influence of chemotherapy and radiotherapy in the evolution of the case.

  9. Genetic and chromosomal alterations in Kenyan Wilms Tumor

    DEFF Research Database (Denmark)

    Lovvorn, Harold N; Pierce, Janene; Libes, Jaime


    Wilms tumor (WT) is the most common childhood kidney cancer worldwide and poses a cancer health disparity to black children of sub-Saharan African ancestry. Although overall survival from WT at 5 years exceeds 90% in developed countries, this pediatric cancer is alarmingly lethal in sub-Saharan A......Wilms tumor (WT) is the most common childhood kidney cancer worldwide and poses a cancer health disparity to black children of sub-Saharan African ancestry. Although overall survival from WT at 5 years exceeds 90% in developed countries, this pediatric cancer is alarmingly lethal in sub...... prognostic indicator of WT biology in developed countries, was detected in 32% of KWT analyzed, and 89% of these children are deceased. Similarly, LOH at 11q was detected in 32% of KWT, and 80% of these patients are deceased. From this genomic analysis, KWT biology appears uniquely aggressive and treatment-resistant....

  10. [A case of Wilm's tumor with full symptomatic WAGR syndrome]. (United States)

    Januszkiewicz, D; Daszkiewicz, P


    The authors of this paper presented a case of a baby with full-symptomatic WAGR syndrome (Wilms tumor, aniridia, genital tract malformation and mental retardation) treated in the I Department of Pediatrics, Institute of Pediatrics, Medical Academy Poznań. The etiology of this syndrome was discussed (deletion of the 13th band of the 11th chromosome short arm). The reason for treatment failure was analysed.

  11. A Pathologic Link between Wilms Tumor Suppressor Gene, WT1, and IFI16

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    Marianne K-H. Kim


    Full Text Available The Wilms tumor gene (WT1 is mutated or deleted in patients with heredofamilial syndromes associated with the development of Wilms tumors, but is infrequently mutated in sporadic Wilms tumors. By comparing the microarray profiles of syndromic versus sporadic Wilms tumors and WT1-inducible Saos-2 osteosarcoma cells, we identified interferon-inducible protein 16 (IFI16, a transcriptional modulator, as a differentially expressed gene and a candidate WT1 target gene. WT1 induction in Saos-2 osteosarcoma cells led to strong induction of IFI16 expression and its promoter activity was responsive to the WT1 protein. Immunohistochemical analysis showed that IFI16 and WT1 colocalized in WT1-replete Wilms tumors, but not in normal human midgestation fetal kidneys, suggesting that the ability of WT1 to regulate IFI16 in tumors represented an aberrant pathologic relationship. In addition, endogenous IFI16 and WT1 interacted in vivo in two Wilms tumor cell lines. Furthermore, IFI16 augmented the transcriptional activity of WT1 on both synthetic and physiological promoters. Strikingly, short hairpin RNA (shRNA-mediated knockdown of either IFI16 or WT1 led to decreased growth of Wilms tumor cells. These data suggest that IFI16 and WT1, in certain cellular context including sporadic Wilms tumors, may support cell survival.

  12. Adhesion molecules in Wilms tumor (part II : beta-catenin expression and significance

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    Basta-Jovanović Gordana M.


    Full Text Available Beta-catenin is a glicoprotein which has an important role in cell-cell adhesion, as well as in cell signal transmition, in u regulation of gen expression and in interaction with axin and APC (adenomatous poliposis coli. Its oncogenic role in several types of carcinomas in human population is well known. It is very likely that b-catenin as an protooncogen plays an importante role in genesis of Wilms tumor. It is well known that in 15% Wilms tumors there are b-catenin mutations, which indicates that there is a disorder in Wnt signal paththat plays an important role in Wilms tumor genesis. The aim of our study was to investigate b-catenin expression in Wilms tumor, to compaire it with the expression in normal renal tissue as well as to see if there is a positive correlation between b-catenin expression in Wilms tumor with tumor stage, histologic type and/ or prognostic group.

  13. Occurrence of Wilms' tumor in a child with hereditary spherocytosis. (United States)

    Özyörük, Derya; Demir, Hacı Ahmet; Emir, Suna; Karakuş, Esra; Tunç, Bahattin


    Hereditary spherocytosis (HS) is the most frequent cause of congenital hemolytic anemia. It is an autosomal dominant genetic disorder characterized by cell membrane abnormalities, specifically in red blood cells. Although the association between benign, borderline and malignant tumors and HS is not clear, various tumors such as splenoma, adrenal myolipoma, pancreatic schwannoma, ganglioneuroma, extramedullary hematopoiesis, myeloproliferative disorders, multiple myeloma, B-cell lymphoma and acute lymphoblastic leukemia have been presented in case reports concerning HS patients. Here we describe a 6-year-old boy with HS who presented with a mass in the left kidney. Tru-cut biopsy revealed Wilms' tumor (WT). To the best of our knowledge, this is the first case of WT associated with HS to be reported in the literature.

  14. A Case of Bilateral Cystic Partially Differentiated Nephroblastoma vs Cystic Wilms' Tumor: Highlighting a Diagnostic Dilemma. (United States)

    Stout, Thomas E; Au, Jason K; Hicks, J M; Gargollo, Patricio C


    Cystic partially differentiated nephroblastoma (CPDN) is a rare multicystic renal tumor along the spectrum of cystic nephroma and cystic Wilms' tumor. There have only been two previously reported cases of bilateral CPDN in the literature. We present here a case of bilateral CPDN vs cystic Wilms' tumor treated with neoadjuvant and adjuvant chemotherapy in addition to a bilateral partial nephrectomy. We also review the relevant literature regarding CPDN in an effort to aid in diagnosis and management of these rare cystic renal tumors.

  15. Sorafenib Tosylate in Treating Younger Patients With Relapsed or Refractory Rhabdomyosarcoma, Wilms Tumor, Liver Cancer, or Thyroid Cancer (United States)


    Childhood Hepatocellular Carcinoma; Papillary Thyroid Cancer; Previously Treated Childhood Rhabdomyosarcoma; Recurrent Childhood Liver Cancer; Recurrent Childhood Rhabdomyosarcoma; Recurrent Thyroid Cancer; Recurrent Wilms Tumor and Other Childhood Kidney Tumors

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

    KAUST Repository

    Gadd, Samantha


    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.

  17. CT appearances of involvement of the peritoneum, mesentery and omentum in Wilms` tumor

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    Slasky, B.S. [Dept. of Radiology, Hadassah Univ. Hospital, Jerusalem (Israel); Bar-Ziv, J. [Dept. of Radiology, Hadassah Univ. Hospital, Jerusalem (Israel); Freeman, A.I. [Dept. of Pediatric Hematology/Oncology, Hadassah Univ. Hospital, Jerusalem (Israel); Peylan-Ramu, N. [Dept. of Pediatric Hematology/Oncology, Hadassah Univ. Hospital, Jerusalem (Israel)


    Background. Peritoneal involvement by Wilms` tumor indicates stage III disease. CT is the single preferred modality in determining the extent and staging of Wilms` tumor; however, the CT appearances of Wilms` tumor involvement of the peritoneum have not been specifically addressed in the literature. Objective. The objective of this study was to demonstrate the CT manifestations when there is involvement of the peritoneum, mesentery and/or omentum in Wilms` tumor. Materials and methods. Four cases of Wilms` tumor form the basis of this report. They were examined on Elscint CT scanners. Results. Masses (``dropped metastases``) in the pelvis were present in all four patients. Three patients had masses in the mesentery of the small bowel and sigmoid colon. Infiltration of the greater omentum was identified in two patients as a mantle of tumor separating bowel from the anterior abdominal wall. Ascites was present in two patients. In one patient broad-based solid masses of varying sizes were noted on the parietal and on the visceral surfaces of the peritoneum, and in a different patient a discrete mass was noted in the lesser omentum. Conclusion. The peritoneal spaces, recesses, ligaments and folds are invisible unless invaded by disease which is well demonstrated on CT. (orig.). With 6 figs., 1 tab.

  18. 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.)


    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

  19. Wilms tumor: "State-of-the-art" update, 2016. (United States)

    Irtan, Sabine; Ehrlich, Peter F; Pritchard-Jones, Kathy


    Despite an impressive increase in survival rate over the past decades, there is still a need to improve the survival of specific subgroups of Wilms tumor (anaplastic, metastatic, and bilateral) and to decrease the late effects of treatment in terms of renal function and heart toxicity. We aim to explore new areas of improvement, from diagnosis to treatment: in the field of radiology the increased use of MRI and exploration of its diffusion-weighted imaging capabilities to predict WT histology at diagnosis and for preoperative assessment; in biology the emergence of new biomarkers that could be integrated into the decision-making process; and surgical techniques with more accurate indication of nephron-sparing surgery that is no longer reserved for bilateral WT and the minimally invasive approach. The long-term outcome of patients with WT should thus be a strong indicator of the improvement in adapting and personalizing the treatment to each individual. Copyright © 2016 Elsevier Inc. All rights reserved.

  20. Diffusion-weighted MRI for differentiating Wilms tumor from neuroblastoma. (United States)

    Aslan, Mine; Aslan, Ahmet; Arıöz Habibi, Hatice; Kalyoncu Uçar, Ayşe; Özmen, Evrim; Bakan, Selim; Kuruğoğlu, Sebuh; Adaletli, İbrahim


    Wilms tumor (WT) and neuroblastoma (NB) are the most common pediatric abdominal malignant neoplasms of the kidney and adrenal gland. Differentiating them from each other is essential since their treatments are different. Here, we aimed to show the diffusion characteristics of WT and NB for differentiation. Diffusion-weighted imaging (DWI) of 17 histopathologically diagnosed lesions (10 NB and 7 WT in 8 female and 9 male patients) was evaluated retrospectively. The apparent diffusion coefficient (ADC) value for each tumor was calculated using region-of-interest (ROI) measurements by two observers. The mean ADC values were compared, and receiver operating characteristics (ROC) curve analysis was performed. Intraclass correlation was evaluated for the reliability of ADC measurement. The mean ADC values measured by two observers were 0.787±0.09 ×10-3 mm2/s and 0.768±0.08 ×10-3 mm2/s for WT, and 0.524±0.16 ×10-3 mm2/s and 0.529±0.16 ×10-3 mm2/s for NB, respectively (P = 0.006 and P = 0.011). Intraclass correlation coefficient was 0.955. Utilizing ROC curve analysis, a cutoff ADC value of ≤0.645 ×10-3 mm2/s was obtained to differentiate NB from WT. ADC values of NBs were significantly lower than WT with a perfect interobserver agreement. We suggest that DWI may have a role in differentiating the two tumors.

  1. Cushing syndrome secondary to CRH-producing Wilms tumor in a 6 year old. (United States)

    Lee, Moon Hee; Cho, Uiju; Lee, Jae-Wook; Cho, Won-Kyoung; Jung, Min Ho; Chung, Nak Gyun; Cho, Bin; Choi, Yeong Jin; Lee, Myung Duk; Suh, Byung-Kyu


    Abstract Cushing syndrome is caused by prolonged exposure to elevated serum cortisol. It is uncommon in children, and etiology includes pituitary adenoma, adrenal tumor, and exogenous glucocorticoid administration. Rarely, it is paraneoplastic in origin. We present a case of paraneoplastic Cushing syndrome due to Wilms tumor that secreted corticotropin-releasing hormone (CRH). A 6-year-old male presented with polyphagia and weight gain. He showed Cushingoid appearance, hypertension, and palpable left flank mass. Serum cortisol and adrenocorticotropic hormone (ACTH) levels were elevated. Computed tomography showed a neoplasm originating from the left kidney. Pathologic diagnosis of Wilms tumor was made upon nephroureterectomy. Immunohistochemical staining was positive for CRH and negative for ACTH. All features of Cushing syndrome disappeared after surgery. This represents a rare case of Cushing syndrome secondary to Wilms tumor in which CRH production has been demonstrated.

  2. 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)


    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.

  3. Desmoplastic small round cell tumor of the kidney mimicking Wilms tumor: a case report and review of the literature. (United States)

    da Silva, Rogério Cardoso; Medeiros Filho, Plínio; Chioato, Lucimara; Silva, Tácio R B; Ribeiro, Sérgio M; Bacchi, Carlos E


    Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant neoplasm usually present with the widespread abdominal serosal involvement and affects mainly adolescents and young adults. When presenting within visceral organs, as kidney, the diagnosis of DSRCT imposes significant difficulties. We present a case of primary DSRCT of the kidney in a 10-year-old boy mimicking clinically and pathologically Wilms tumor. The tumor showed morphologic and immunohistochemical features of DSRCT and the presence of the Ewing sarcoma and Wilm tumor 1 fusion transcripts resulting from the t(11;22) (p13;q12) reciprocal translocation. DSRCT should be considered in the differential diagnosis of Wilm tumor and other small blue-round cell tumors of the kidney.

  4. 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)


    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.)

  5. Clinical relevance of Wilms tumor 1 gene mutations in childhood acute myeloid leukemia.

    NARCIS (Netherlands)

    Hollink, I.H.; Heuvel-Eibrink, M.M. van den; Zimmermann, M.; Balgobind, B.V.; Arentsen-Peters, S.T.; Alders, M.; Willasch, A.; Kaspers, G.J.L.; Trka, J.; Baruchel, A.; Graaf, S.S.N. de; Creutzig, U.; Pieters, R.; Reinhardt, D.; Zwaan, C.M.


    Wilms tumor 1 (WT1) mutations have recently been identified in approximately 10% of adult acute myeloid leukemia (AML) with normal cytogenetics (CN-AML) and are associated with poor outcome. Using array-based comparative genome hybridization in pediatric CN-AML samples, we detected a WT1 deletion in

  6. Clinical relevance of Wilms tumor 1 gene mutations in childhood acute myeloid leukemia

    NARCIS (Netherlands)

    I.H.I.M. Hollink (Iris); M.M. van den Heuvel-Eibrink (Marry); M. Zimmermann (Martin); B.V. Balgobind (Brian); S.T.C.J.M. Arentsen-Peters (Susan); M. Alders (Mariëlle); A.M. Willasch; G.J. Kaspers (Gertjan); J. Trka (Jan); A. Baruchel (André); S.S.N. de Graaf (Siebold); U. Creutzig; R. Pieters (Rob); D. Reinhardt (Dirk); C.M. Zwaan (Christian Michel)


    textabstractWilms tumor 1 (WT1) mutations have recently been identified in approximately 10% of adult acute myeloid leukemia (AML) with normal cytogenetics (CN-AML) and are associated with poor outcome. Using array-based comparative genome hybridization in pediatric CN-AML samples, we detected a WT1

  7. Tumor-infiltrating Cytotoxic T Lymphocytes as Independent Prognostic Factor in Epithelial Ovarian Cancer With Wilms Tumor Protein 1 Overexpression

    NARCIS (Netherlands)

    Vermeij, Renee; de Bock, Geertruida H.; Leffers, Ninke; ten Hoor, Klaske A.; Schulze, Ute; Hollema, Harry; van der Burg, Sjoerd H.; van der Zee, Ate G. J.; Daemen, Toos; Nijman, Hans W.


    Immune response characterization at the primary tumor site enables the design of therapeutic vaccination strategies with higher efficacy in epithelial ovarian cancer (EOC). In this study, we related Wilms tumor protein 1 (WT1) overexpression, a well-established immunotherapeutic target, to clinicopa

  8. Expression of vascular endothelial growth factor (VEGF) and VEGF-C in serum and tissue of Wilms tumor

    Institute of Scientific and Technical Information of China (English)

    WANG Lei; ZHANG Da; CHEN Xin-rang; FAN Yu-xia; WANG Jia-xiang


    Background Angiogenesis and lymphogenesis which were promoted by vascular endothelial growth factor (VEGF)and VEGF-C are important in the growth and metastasis of solid tumors.The high level of VEGF and VEGF-C were distributed in numerous types of cancers,but their distribution and expression in Wilms tumor,the most common pediatric tumor of the kidney,was unclear.Methods To learn about the distribution,mass spectroscopy and immunohistochemistry were used to measure the level of VEGF and VEGF-C in serum and tissue of Wilms tumor.Results The expression level of VEGF in serum of Wilms tumor was the same as in pre-surgery and control,so it was the same case of VEGF-C.Both of these factors were chiefly located in Wilms tumor tissue,but not in borderline and normal.In addition,the higher clinical staging and histopathologic grading were important elements in high expression of VEGF and VEGF-C.Gender,age and the size of tumor have not certainly been implicated in expression level of VEGF and VEGF-C.Conclusions The lymph node metastasis and growth of tumors resulted from angiogenesis and lymphogenesis which were promoted by VEGF and VEGF-C in Wilms tumor.The autocrine and paracrine process of VEGF and VEGF-C were the principal contributor to specific tissues of Wilms tumor but not to the entire body.

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

    Directory of Open Access Journals (Sweden)

    O. H. Radhika Krishna


    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.

  10. Immunohistochemical Expression of Ki67 and p53 in Wilms Tumor and Its Relationship with Tumor Histology and Stage at Presentation. (United States)

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


    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.

  11. 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

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    Silva, Eduardo Just da Costa e, E-mail: [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


    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)

  12. Wilms tumor arising in a child with X-linked nephrogenic diabetes insipidus. (United States)

    El-Kares, Reyhan; Hueber, Pierre-Alain; Blumenkrantz, Miriam; Iglesias, Diana; Ma, Kim; Jabado, Nada; Bichet, Daniel G; Goodyer, Paul


    We report on a child with X-linked nephrogenic diabetes insipidus (NDI) who developed Wilms tumor (WT). Nephrogenic diabetes insipidus is caused by mutations of the arginine vasopressin receptor (AVPR2) or aquaporin-II (AQP2) genes. Wilms tumor is also genetically heterogeneous and is associated with mutations of WT1 (15-20%), WTX (20-30%) and other loci. The boy presented at 5 months with failure to thrive, polyuria, hypernatremia and abdominal mass. Analysis of leukocyte DNA showed a novel missense mutation (Q174H) of the AVPR2 gene, which was not present in his mother. In cells (WitS) isolated from the tumor, WTX mRNA expression and coding sequence were intact. However, we identified a 44-kb homozygous deletion of the WT1 gene spanning exons 4 to 10. The WT1 deletion was not present in leukocyte DNA from the patient or his mother. We also noted strong beta-catenin (CTNNB1) expression in the tumor cells and identified a heterozygote missense Ser45Cys mutation of exon 3 of CTNNB1. However, the mutation was absent both in the constitutional DNA of the patient and his mother. The concurrence of WT and NDI has not been previously reported and may be unrelated. Nevertheless, this case nicely illustrates the sequence of events leading to sporadic Wilms tumor.

  13. Birth characteristics and Wilms tumors in children in the Nordic countries

    DEFF Research Database (Denmark)

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


    -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 parental age and birth order. In our large-scale, registry......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...

  14. Synchronous bilateral Wilms tumor: a report from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP). (United States)

    Indolfi, Paolo; Jenkner, Alessandro; Terenziani, Monica; Crocoli, Alessandro; Serra, Annalisa; Collini, Paola; Biasoni, Davide; Gandola, Lorenza; Bisogno, Gianni; Cecchetto, Giovanni; Di Martino, Martina; D'Angelo, Paolo; Bianchi, Maurizio; Conte, Massimo; Inserra, Alessandro; Pession, Andrea; Spreafico, Filippo


    The optimal management of bilateral Wilms tumor (BWT) is challenging, and their survival is lower than for unilateral tumors. This report discusses a large series of BWTs treated in Italy in the last 2 decades. This analysis concerns patients with synchronous BWT registered at Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers between 1990 and 2011; details on their treatment and outcome are presented and discussed. Ninety BWTs were registered in the AIEOP Wilms tumor database. Preoperative chemotherapy was given for a median 12 weeks before definitive tumor resection was attempted. Forty-eight percent of the patients had preservation of bilateral renal parenchyma. The proportion of bilateral nephron-sparing surgeries was not higher in the 37 patients initially given doxorubicin/vincristine/actinomycin D (32%) than in the 43 children receiving vincristine/actinomycin D alone (58%). The 4-year disease-free survival rate was 66.5% ± 5% and overall survival was 80% ± 5% for the cohort as a whole. The 4-year disease-free survival (overall survival) for 18 children with diffuse anaplasia or postchemotherapy blastemal-type tumors was 51% ± 13% (62% ± 13%), as opposed to 72% ± 3% (88% ± 4%) for 68 children with a favorable histology (log-rank P = .04 [P = .007]). These results provide further evidence that the optimal duration and choice of drugs for preoperative chemotherapy remain an open question. Outcome remained significantly worse for BWT than for unilateral Wilms tumor. To enable the conservative treatment of as many affected kidneys as possible, only centers with experience in BWT should manage such cases. Copyright © 2013 American Cancer Society.

  15. Anesthesia Management of a 20-Month-Old Patient with Giant Unilateral Wilms Tumor

    Directory of Open Access Journals (Sweden)

    Nune Matinyan


    Full Text Available Wilms tumour (WT (or nephroblastoma is one of the most common malignant kidney tumors in children. On subsequent stages clinically it is often characterized by abdominal hypertension syndrome, which, in turn, leads to development of respiratory insufficiency. Other symptoms comprise renal deficiency, hypertension, and abnormalities of hemostasis and hemogram. Treatment includes rounds of preoperative chemotherapy and subsequent surgery. We report a case of perioperative management for nephrectomy in 20-month-old patient with a giant unilateral WT. The complexity of anesthesia was determined by the size of tumor, increased intra-abdominal pressure, respiratory deficiency, and hypercoagulation.

  16. Wilms tumor 1 peptide vaccination combined with temozolomide against newly diagnosed glioblastoma: safety and impact on immunological response. (United States)

    Hashimoto, Naoya; Tsuboi, Akihiro; Kagawa, Naoki; Chiba, Yasuyoshi; Izumoto, Shuichi; Kinoshita, Manabu; Kijima, Noriyuki; Oka, Yoshihiro; Morimoto, Soyoko; Nakajima, Hiroko; Morita, Satoshi; Sakamoto, Junichi; Nishida, Sumiyuki; Hosen, Naoki; Oji, Yusuke; Arita, Norio; Yoshimine, Toshiki; Sugiyama, Haruo


    To investigate the safety of combined Wilms tumor 1 peptide vaccination and temozolomide treatment of glioblastoma, a phase I clinical trial was designed. Seven patients with histological diagnosis of glioblastoma underwent concurrent radiotherapy and temozolomide therapy. Patients first received Wilms tumor 1 peptide vaccination 1 week after the end of combined concurrent radio/temozolomide therapy, and administration was continued once per week for 7 weeks. Temozolomide maintenance was started and performed for up to 24 cycles, and the observation period for safety encompassed 6 weeks from the first administration of maintenance temozolomide. All patients showed good tolerability during the observation period. Skin disorders, such as grade 1/2 injection-site reactions, were observed in all seven patients. Although grade 3 lymphocytopenia potentially due to concurrent radio/temozolomide therapy was observed in five patients (71.4 %), no other grade 3/4 hematological or neurological toxicities were observed. No autoimmune reactions were observed. All patients are still alive, and six are on Wilms tumor 1 peptide vaccination without progression, yielding a progression-free survival from histological diagnosis of 5.2-49.1 months. Wilms tumor 1 peptide vaccination was stopped in one patient after 12 injections by the patient's request. The safety profile of the combined Wilms tumor 1 peptide vaccination and temozolomide therapy approach for treating glioblastoma was confirmed.

  17. Recurrent DGCR8, DROSHA, and SIX homeodomain mutations in favorable histology Wilms tumors. (United States)

    Walz, Amy L; Ooms, Ariadne; Gadd, Samantha; Gerhard, Daniela S; Smith, Malcolm A; Guidry Auvil, Jaime M; Guidry Auvil, Jamie M; Meerzaman, Daoud; Chen, Qing-Rong; Hsu, Chih Hao; Yan, Chunhua; Nguyen, Cu; Hu, Ying; Bowlby, Reanne; Brooks, Denise; Ma, Yussanne; Mungall, Andrew J; Moore, Richard A; Schein, Jacqueline; Marra, Marco A; Huff, Vicki; Dome, Jeffrey S; Chi, Yueh-Yun; Mullighan, Charles G; Ma, Jing; Wheeler, David A; Hampton, Oliver A; Jafari, Nadereh; Ross, Nicole; Gastier-Foster, Julie M; Perlman, Elizabeth J


    We 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 that tumors with SIX1/2 and/or miRNAPG mutations show a pre-induction metanephric mesenchyme gene expression pattern and are significantly associated with both perilobar nephrogenic rests and 11p15 imprinting aberrations. Significantly decreased expression of mature Let-7a and the miR-200 family (responsible for mesenchymal-to-epithelial transition) in miRNAPG mutant tumors is associated with an undifferentiated blastemal histology. The combination of SIX and miRNAPG mutations in the same tumor is associated with evidence of RAS activation and a higher rate of relapse and death.

  18. Heterogeneity of Disease Classified as Stage III in Wilms Tumor: A Report From the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP)

    Energy Technology Data Exchange (ETDEWEB)

    Spreafico, Filippo, E-mail: [Pediatric Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano (Italy); Gandola, Lorenza [Department of Radiology/Radiotherapy, Fondazione IRCCS Istituto Nazionale Tumori, Milano (Italy); D' Angelo, Paolo [Pediatric Oncology, Ospedale dei Bambini G. di Cristina, Palermo (Italy); Terenziani, Monica [Pediatric Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano (Italy); Collini, Paola [Department of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale Tumori, Milano (Italy); Bianchi, Maurizio [Pediatric Department, Ospedale Infantile Regina Margherita, Torino (Italy); Provenzi, Massimo [Pediatric Unit, Ospedali Riuniti Bergamo, Bergamo (Italy); Indolfi, Paolo [Pediatric Oncology, Pediatric Department, II University, Napoli (Italy); Pession, Andrea [Pediatric Hematology and Oncology Unit ' Lalla Seragnoli' , Bologna University, Bologna (Italy); Nantron, Marilina [Department of Hematology and Oncology, Istituto G. Gaslini, Genova (Italy); Di Cataldo, Andrea [Pediatric Hematology and Oncology, Catania University, Catania (Italy); Marchiano, Alfonso [Department of Radiology/Radiotherapy, Fondazione IRCCS Istituto Nazionale Tumori, Milano (Italy); Catania, Serena; Fossati Bellani, Franca [Pediatric Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano (Italy); Piva, Luigi [Pediatric Surgery Unit, Urology, Fondazione IRCCS Istituto Nazionale Tumori, Milano (Italy)


    Purpose: We analyzed whether the prognosis can differ among Wilms tumors (WT) labeled as Stage III according to currently adopted classification systems. Methods and Materials: Patients with nonanaplastic Stage III WT consecutively registered in two Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) trials (CNR-92, TW-2003) were the subjects in the present analysis. The steady mainstay of therapy was primary nephrectomy, followed by three-drug chemotherapy with vincristine, dactinomycin, doxorubicin, and abdominal radiotherapy (RT). Results: Ninety-nine WT patients met the criteria for classification as Stage III according to a revised version of the National Wilms Tumor Study-3 staging system (51 patients in CNR-92, 48 patients in TW-2003). Regional lymph nodes (LN) were not biopsied in 16 patients. After a median follow-up of 66 months, the 4-year disease-free survival (DFS) and overall survival (OS) rates were 85% {+-} 4% and 92% {+-} 3%, respectively, for the whole group. For 38 children with positive LN, the 4-year DFS rate was 73% {+-} 7%, as opposed to 98% {+-} 2% for the 45 children with Stage III WT according to the other criteria but with negative biopsied LN (p = 0.001). The subgroup with the worst prognosis consisted of children more than 2 years old with positive LN (DFS 67% {+-} 8%). A delay between surgery and RT > 30 days had an adverse impact on the abdominal tumor relapse rate. Conclusions: This study provides further evidence that Stage III tumors with LN metastases might be distinguished from WTs meeting the other criteria for classification as Stage III. The worse outcome of the former may warrant a prospective study on the effects of intensified therapy. A subclassification of Stage III tumors is discussed.

  19. Heterogeneity of disease classified as stage III in Wilms tumor: a report from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP). (United States)

    Spreafico, Filippo; Gandola, Lorenza; D'Angelo, Paolo; Terenziani, Monica; Collini, Paola; Bianchi, Maurizio; Provenzi, Massimo; Indolfi, Paolo; Pession, Andrea; Nantron, Marilina; Di Cataldo, Andrea; Marchianò, Alfonso; Catania, Serena; Fossati Bellani, Franca; Piva, Luigi


    We analyzed whether the prognosis can differ among Wilms tumors (WT) labeled as Stage III according to currently adopted classification systems. Patients with nonanaplastic Stage III WT consecutively registered in two Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) trials (CNR-92, TW-2003) were the subjects in the present analysis. The steady mainstay of therapy was primary nephrectomy, followed by three-drug chemotherapy with vincristine, dactinomycin, doxorubicin, and abdominal radiotherapy (RT). Ninety-nine WT patients met the criteria for classification as Stage III according to a revised version of the National Wilms Tumor Study-3 staging system (51 patients in CNR-92, 48 patients in TW-2003). Regional lymph nodes (LN) were not biopsied in 16 patients. After a median follow-up of 66 months, the 4-year disease-free survival (DFS) and overall survival (OS) rates were 85% ± 4% and 92% ± 3%, respectively, for the whole group. For 38 children with positive LN, the 4-year DFS rate was 73% ± 7%, as opposed to 98% ± 2% for the 45 children with Stage III WT according to the other criteria but with negative biopsied LN (p = 0.001). The subgroup with the worst prognosis consisted of children more than 2 years old with positive LN (DFS 67% ± 8%). A delay between surgery and RT > 30 days had an adverse impact on the abdominal tumor relapse rate. This study provides further evidence that Stage III tumors with LN metastases might be distinguished from WTs meeting the other criteria for classification as Stage III. The worse outcome of the former may warrant a prospective study on the effects of intensified therapy. A subclassification of Stage III tumors is discussed. Copyright © 2012 Elsevier Inc. All rights reserved.

  20. 3D-visualization by MRI for surgical planning of Wilms tumors; 3-D-Visualisierung in der MRT zur Operationsplanung von Wilms-Tumoren

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    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


    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.)

  1. Unusual association of non-anaplastic Wilms tumor and Cornelia de Lange syndrome: case report. (United States)

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


    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.

  2. Birth characteristics and Wilms tumors in children in the Nordic countries

    DEFF Research Database (Denmark)

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


    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...

  3. Identification and analysis of mutations in WTX and WT1 genes in peripheral blood and tumor tissue of children with Wilms' tumor

    Institute of Scientific and Technical Information of China (English)

    WANG Hui; SHEN Ying; SUN Ning; JIANG Ye-ping; LI Ming-lei; SUN Lin


    Background Wilms' tumor (nephroblastoma) is the most common pediatric kidney cancer.Only one Wiims' tumor gene is known,WT1 at 11 p 13,which is mutated in 5%-10% of Wilms' tumors.Recently,mutations were reported in WTX at Xq11.1 in Wilms' tumors.This study investigated the mutation proportion,type,and distribution in WTX and WT1 in children with Wilms' tumor.The role of WTX/WT1 in the development of Wilms' tumor,and the relationship between clinical phenotype and genotype,were also studied.Methods Wilms' tumor specimens (blood samples from 70 patients and tumor tissue samples from 52 patients) were used.A long fragment of WTX and 10 exons and intron sequences of WT1 were amplified by polymerase chain reaction (PCR) from extracted genomic DNA and sequenced.A chi-square test compared the difference between the WTX mutation group and the no mutation group.The relationship between the mutations and clinical phenotype was analyzed.Results WTX mutations were found in 5/52 tumor tissues and in 2/70 peripheral blood samples (five cases in total,all point mutations).Two patients had a WTX mutation in both samples.WT1 mutations were found in 2/52 tumor tissues and in 4/70 peripheral blood samples (five cases in total,all point mutations).One patient had a WT1 mutation in both samples.Ten cases had WTX or WT1 mutation (19.2% of Wilms' tumors).No overlapping WTXand WT1 mutations were found.No significant differences in clinical parameters were found between patients with and without a WTX mutation.Conclusions WTX mutations occur early in Wilms' tumor development,but at e low proportion.There was no evidence that WTX is the main cause of Wilms' tumor.Clinical parameters of patients with WTX mutations are not related to the mutation,indicating a limited impact of WTX on tumor progression.WTX and WT1 mutations occur independently,suggesting a relationship between their gene products.

  4. Discrepant outcomes in two Brazilian patients with Bloom syndrome and Wilms' tumor: two case reports. (United States)

    Moreira, Marilia Borges; Quaio, Caio Robledo Dc; Zandoná-Teixeira, Aline Cristina; Novo-Filho, Gil Monteiro; Zanardo, Evelin Aline; Kulikowski, Leslie Domenici; Kim, Chong Ae


    Bloom syndrome is a rare, autosomal recessive, chromosomal instability disorder caused by mutations in the BLM gene that increase the risk of developing neoplasias, particularly lymphomas and leukemias, at an early age. Case 1 was a 10-year-old Brazilian girl, the third child of a non-consanguineous non-Jewish family, who was born at 36 weeks of gestation and presented with severe intrauterine growth restriction. She had Bloom syndrome and was diagnosed with a unilateral Wilms' tumor at the age of 3.5 years. She responded well to oncological treatment and has remained disease-free for the last 17 years. Case 2 was a 2-year-old Brazilian girl born to non-Jewish first-degree cousins. Her gestation was marked by intrauterine growth restriction. She had Bloom syndrome; a unilateral stage II Wilms' tumor was diagnosed at the age of 4 years after the evaluation of a sudden onset abdominal mass. Surgical removal, neoadjuvant chemotherapy and radiotherapy were not sufficient to control the neoplasia. The tumor recurred after 8 months and she died from clinical complications. Our study reports the importance of rapid diagnostics and clinical follow-up of these patients.

  5. Somatic mutations in DROSHA and DICER1 impair microRNA biogenesis through distinct mechanisms in Wilms tumors (United States)

    Rakheja, Dinesh; Chen, Kenneth S.; Liu, Yangjian; Shukla, Abhay A.; Schmid, Vanessa; Chang, Tsung-Cheng; Khokhar, Shama; Wickiser, Jonathan E.; Karandikar, Nitin J.; Malter, James S.; Mendell, Joshua T.; Amatruda, James F.


    Wilms tumor is the most common childhood kidney cancer. Here we report the whole-exome sequencing of 44 Wilms tumors, identifying missense mutations in the microRNA (miRNA)-processing enzymes DROSHA and DICER1 and novel mutations in MYCN, SMARCA4 and ARID1A. Examination of tumor miRNA expression, in vitro processing assays, and genomic editing in human cells demonstrate that DICER1 and DROSHA mutations influence miRNA processing through distinct mechanisms. DICER1 RNase IIIB mutations preferentially impair processing of miRNAs deriving from the 5′ arm of pre-miRNA hairpins, while DROSHA RNase IIIB mutations globally inhibit miRNA biogenesis through a dominant-negative mechanism. Both DROSHA and DICER1 mutations impair expression of tumor-suppressing miRNAs including the let-7 family, important regulators of MYCN, LIN28 and other Wilms tumor oncogenes. These results provide new insights into the mechanisms through which mutations in miRNA biogenesis components reprogram miRNA expression in human cancer and suggest that these defects define a distinct subclass of Wilms tumors. PMID:25190313

  6. Induction of WT1 (Wilms' Tumor Gene)-Specific Cytotoxic T Lymphocytes by WT1 Peptide Vaccine and the Resultant Cancer Regression

    National Research Council Canada - National Science Library

    Yoshihiro Oka; Akihiro Tsuboi; Tetsuya Taguchi; Tadashi Osaki; Taiichi Kyo; Hiroko Nakajima; Olga A. Elisseeva; Yusuke Oji; Manabu Kawakami; Kazuhiro Ikegame; Naoki Hosen; Satoshi Yoshihara; Fei Wu; Fumihiro Fujiki; Masaki Murakami; Tomoki Masuda; Sumiyuki Nishida; Toshiaki Shirakata; Shin-ichi Nakatsuka; Ayako Sasaki; Keiko Udaka; Hiroo Dohy; Katsuyuki Aozasa; Shinzaburo Noguchi; Ichiro Kawase; Haruo Sugiyama; Takashi Sugimura


    The Wilms' tumor gene WT1 is overexpressed in leukemias and various types of solid tumors, and the WT1 protein was demonstrated to be an attractive target antigen for immunotherapy against these malignancies...

  7. Effectiveness of enteral and parenteral nutrition in the nutritional management of children with Wilms' tumors. (United States)

    Rickard, K A; Kirksey, A; Baehner, R L; Grosfeld, J L; Provisor, A; Weetman, R M; Boxer, L A; Ballantine, T V


    The effectiveness of enteral and parenteral feeding in supporting a satisfactory nutrition status and/or reversing protein-energy malnutrition was evaluated in nine children, ages 1 to 7 years (eight female), with Wilms' tumors. At the onset of treatment, eight patients received comprehensive enteral nutrition (CEN) which included intense nutritional counseling and oral supplements while one received total parenteral nutrition (TPN). Despite CEN, the initial, intense treatment period was associated with a decreased energy intake (64 +/- 27% Recommended Dietary Allowances), dramatic weight loss (22 +/- 7% by 26 +/- 17 days from the beginning of treatment), decreased skinfold thickness (total lymphocyte count were 3.02 +/- 0.45 g/dl, 155 +/- 40 mg/dl, and, 655 +/- 437/mm3, respectively; all children had abnormal anthropometric measurements and anergy to recall skin test antigens. TPN for 28 or more days supported weight gain (+ 2.44 kg), increased serum albumin (+ 0.58 +/- 0.47 g/dl) and transferrin (+ 76 +/- 34 mg/dl), and reversed anergy despite low total lymphocyte counts. During maintenance treatment, nutritional status was maintained or restored with CEN in the group who responded. These preliminary data document the severity of protein-energy malnutrition which accompanies initial, intense treatment of children with Wilms' tumors, the nutritional and immunological benefits of TPN during continuing intense treatment and the effectiveness of CEN in maintaining a satisfactory nutritional status during maintenance treatment.

  8. Surgical management of Wilms tumor with intravascular extension: a single-institution experience. (United States)

    Aspiazu, Diego; Fernandez-Pineda, Israel; Cabello, Rosa; Ramirez, Gema; Alvarez-Madrid, Antonio; De Agustin, Juan Carlos


    The purpose of this study was to retrospectively analyze the clinical presentation, treatment, and outcomes of children with Wilms tumor (WT) and intravascular extension who were treated at a single institution. A retrospective review was conducted of medical records of all children with Wilms tumor and intravascular extension treated at Virgen del Rocio Children's Hospital between 1992 and 2010. Seven patients (median age 3.4 years, range 2-8.1 years) were identified. At diagnosis, 6 of the 7 patients (85.7%) presented with tumor thrombus that reached the right atrium (RA) and 1 patient with infrahepatic inferior vena cava (IVC) thrombus. All patients received neoadjuvant chemotherapy (SIOP 2001 protocol) with vincristine, doxorubicin, and actinomycin D. Regression of the intravascular extension of the tumor was documented in all patients. Postchemotherapy level of extension was suprahepatic IVC in 1 patient, infrahepatic IVC in 2 patients, renal vein (RV) in 1 patient, and RA in 3 patients. Nephrectomy and thrombectomy were performed in all cases, requiring cardiopulmonary bypass for the 4 patients who presented with suprahepatic IVC and RA thrombus. The other 3 patients with infrahepatic IVC and RV involvement underwent cavotomy and thrombus extraction. Computed tomography, ultrasonography, and echocardiography were used for diagnosis and follow-up. All patients remain disease-free with a median follow-up of 6.3 years (range, 2-19 years). Neoadjuvant chemotherapy for WT with intravascular extension may facilitate the resection by decreasing the extent of the tumor thrombus. Cardiopulmonary bypass is indicated for suprahepatic IVC and RA involvement. Accurate diagnostic imaging is necessary.

  9. Germline mutations in DIS3L2 cause the Perlman syndrome of overgrowth and Wilms tumor susceptibility

    NARCIS (Netherlands)

    Astuti, Dewi; Morris, Mark R.; Cooper, Wendy N.; Staals, Raymond H. J.; Wake, Naomi C.; Fews, Graham A.; Gill, Harmeet; Gentle, Dean; Shuib, Salwati; Ricketts, Christopher J.; Cole, Trevor; van Essen, Anthonie J.; van Lingen, Richard A.; Neri, Giovanni; Opitz, John M.; Rump, Patrick; Stolte-Dijkstra, Irene; Mueller, Ferenc; Pruijn, Ger J. M.; Latif, Farida; Maher, Eamonn R.


    Perlman syndrome is a congenital overgrowth syndrome inherited in an autosomal recessive manner that is associated with Wilms tumor susceptibility. We mapped a previously unknown susceptibility locus to 2q37.1 and identified germline mutations in DIS3L2, a homolog of the Schizosaccharomyces pombe di

  10. Topotecan distribution in an anephric infant with therapy resistant bilateral Wilms tumor with a novel germline WT1 gene mutation

    NARCIS (Netherlands)

    R.T. Lugtenberg (Rieneke); K. Cransberg (Karlien); W.J. Loos (Walter); A. Wagner (Anja); M. Alders (Mariëlle); M.M. van den Heuvel-Eibrink (Marry)


    textabstractThe therapeutic strategy for bilateral Wilms tumor (WT) remains a challenge. Especially in cases with chemotherapy resistant disease, bilateral nephrectomy is sometimes inevitable. For optimal cure rates stage V WT patients benefit from adjuvant treatment; however, there are limited data

  11. Association between TP53 gene Arg72Pro polymorphism and Wilms' tumor risk in a Chinese population. (United States)

    Fu, Wen; Zhuo, Zhen-Jian; Jia, Wei; Zhu, Jinhong; Zhu, Shi-Bo; Lin, Ze-Feng; Wang, Feng-Hua; Xia, Huimin; He, Jing; Liu, Guo-Chang


    Wilms' tumor is one of the most prevalent pediatric malignancies, ranking fourth in childhood cancer worldwide. TP53 is a critical tumor suppressor gene, which encodes a 53 kDa protein, p53. The p53 functions to protect against cancer by regulating cell cycle and apoptosis and maintaining DNA integrity. TP53 gene is highly polymorphic. Several TP53 gene polymorphisms have been considered to be associated with cancer risk. Of them, a nonsynonymous polymorphism, Arg72Pro (rs1042522 C>G), has been most extensively studied for the association with cancer risk; however, few studies have investigated its effect on Wilms' tumor. Because of the central role of p53 in cell cycle control, the TP53 gene Arg72Pro polymorphism is also a good potential candidate predisposition locus for this pediatric cancer. We genotyped this polymorphism in 145 patients and 531 cancer-free controls recruited from Chinese children by Taqman methodology. Overall, our result suggested a lack of association between the TP53 gene Arg72Pro polymorphism and Wilms' tumor. In the stratified analysis, we found that carriers of CG/GG genotypes had a significantly increased Wilms' tumor risk in children not older than 18 months (adjusted odds ratio =2.04, 95% confidence interval =1.003-4.13, P=0.049) compared with CC genotype carriers. Our study indicated that the TP53 gene Arg72Pro polymorphism may have a weak, age-related effect on Wilms' tumor risk in Chinese children. These findings need further validations in other populations with larger sample size.

  12. Wilms' tumor protein Wt1 regulates the Interleukin-10 (IL-10) gene. (United States)

    Sciesielski, Lina K; Kirschner, Karin M; Scholz, Holger; Persson, Anja Bondke


    We identified the Wilms' tumor protein, Wt1, as a novel transcriptional activator of the immunosuppressant cytokine interleukin-10 (IL-10). Silencing of Wt1 by RNA interference reduced IL-10 mRNA levels by approximately 90%. IL-10 transcripts were increased more than 15-fold upon forced expression of Wt1. Electrophoretic mobility shift assay and chromatin immunoprecipitation revealed a cis-element that was responsible for activation of the IL-10 promoter by Wt1 in murine macrophages. Mutation of the Wt1 binding motif abrogated stimulation of the IL-10 promoter by tumor necrosis factor-α (TNFα). These results suggest a novel immune regulatory function of Wt1 in controlling IL-10 gene expression.

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

    Directory of Open Access Journals (Sweden)

    Laxmi Chilukamarri


    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.

  14. Fine structure analysis of the WT1 gene in sporadic Wilms tumors

    Energy Technology Data Exchange (ETDEWEB)

    Varanasi, R.; Bardeesy, N.; Ghahremani, M.; Pelletier, J. [McGill Univ., Montreal (Canada); Petruzzi, M.-J.; Nowak, N.; Shows, T.B. [State Univ. of New York, Buffalo, NY (United States); Adam, M.A. [Merck-Frosst Center for Therapeutic Research, Point Claire-Dorval (Canada); Grundy, P. [Cross Cancer Institute, Edmonton (Canada)


    Molecular genetic studies indicate that the etiology of Wilms tumor (WT) is complex, involving at least three loci. Germ-line mutations in the tumor suppressor gene, WT1, have been documented in children with WTs and urogenital developmental anomalies. Sporadic tumors constitute the majority (>90%) of WT cases and previous molecular analyses of the WT1 gene have focused only on the DNA-binding domain. Using the single-strand conformational polymorphism (SSCP) assay, the authors analyzed the structural integrity of the entire WT1 gene in 98 sporadic WTs. By PCR-SSCP they find that mutations in the WT1 gene are rare, occurring in only six tumors analyzed. In one sample, two independent intragenic mutations inactivated both WT1 alleles, providing a singular example of two different somatic alterations restricted to the WT1 gene. This case is consistent with the existence of only one tumor suppressor gene at 11p13 involved in the pathogenesis of WTs. The data, together with the previously ascertained occurrence of large deletions/insertions in WT1, define the frequency at which the WT1 gene is altered in sporadic tumors. 36 refs., 3 figs.

  15. The Perlman syndrome: familial renal dysplasia with Wilms tumor, fetal gigantism and multiple congenital anomalies. 1984. (United States)

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


    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.

  16. Laparoscopic nephron-sparing resection of synchronous Wilms tumors in a case of hyperplastic perilobar nephroblastomatosis. (United States)

    Rauth, Thomas P; Slone, Jeremy; Crane, Gabriella; Correa, Hernan; Friedman, Debra L; Lovvorn, Harold N


    Diffuse hyperplastic perilobar nephroblastomatosis (DHPLN) is a rare precursor lesion of Wilms tumor (WT). Because of the increased risk to develop WT in either kidney, current management algorithms of DHPLN merit nephron-sparing strategies, beginning with chemotherapy and close radiographic monitoring into late childhood. After resolution of DHPLN, subsequent detection of a renal nodule mandates resection to exclude WT. Here, we report the case of a 4-year-old girl who developed 2 synchronous nodules in the right kidney more than 2 years after completion of therapy for DHPLN. Because of the early detection and peripheral location of these 2 nodules, laparoscopic nephron-sparing resection of each was performed using ultrasonic dissection. Both nodules were determined on pathology to be favorable histology WT with negative surgical margins. The child was placed on vincristine and actinomycin D therapy for 18 weeks.

  17. Race disparities in peptide profiles of North American and Kenyan Wilms tumor specimens. (United States)

    Libes, Jaime M; Seeley, Erin H; Li, Ming; Axt, Jason R; Pierce, Janene; Correa, Hernan; Newton, Mark; Hansen, Erik; Judd, Audra; McDonald, Hayes; Caprioli, Richard M; Naranjo, Arlene; Huff, Vicki; O'Neill, James A; Lovvorn, Harold N


    Wilms tumor (WT) is the most common childhood kidney cancer worldwide and arises in children of black African ancestry with greater frequency and severity than other race groups. A biologic basis for this pediatric cancer disparity has not been previously determined. We hypothesized that unique molecular fingerprints might underlie the variable incidence and distinct disease characteristics of WT observed between race groups. To evaluate molecular disparities between WTs of different race groups, the Children's Oncology Group provided 80 favorable histology specimens divided evenly between black and white patients and matched for disease characteristics. As a surrogate of black sub-Saharan African patients, we also analyzed 18 Kenyan WT specimens. Tissues were probed for peptide profiles using matrix-assisted laser desorption ionization time of flight imaging mass spectrometry. To control for histologic variability within and between specimens, cellular regions were analyzed separately as triphasic (containing blastema, epithelia, and stroma), blastema only, and stroma only. Data were queried using ClinProTools and statistically analyzed. Peptide profiles, detected in triphasic WT regions, recognized race with good accuracy, which increased for blastema- or stroma-only regions. Peptide profiles from North American WTs differed between black and white race groups but were far more similar in composition than Kenyan specimens. Individual peptides were identified that also associated with WT patient and disease characteristics (eg, treatment failure and stage). Statistically significant peptide fragments were used to sequence proteins, revealing specific cellular signaling pathways and candidate drug targets. Wilms tumor specimens arising among different race groups show unique molecular fingerprints that could explain disparate incidences and biologic behavior and that could reveal novel therapeutic targets. Copyright © 2014 American College of Surgeons. Published

  18. Adult Wilms' tumor. Intraoperative cytology and ancillary studies performed in a case as an adjunct to the histologic diagnosis. (United States)

    Berkley, C; Stanley, M W; Wolpert, J; Rainwater, L M; Smith, C


    A case of blastema-predominant Wilms' tumor in a 64-year-old woman is reported. Intraoperative cytology of a renal mass was used to rule out malignant lymphoma and neuroendocrine carcinoma. Light and electron microscopy, immunocytochemical staining and flow cytometry (FCM) were also performed. Immunoperoxidase studies of smears showed positive staining for vimentin and negative staining for cytokeratins and epithelial membrane antigen. FCM DNA analysis of paraffin-embedded tissue showed no aneuploid peak. Frozen section interpretation of such tumors as seen in this case may be difficult, requiring distinction among several small-blue-cell neoplasms, including Wilms' tumor, neuroendocrine carcinoma and malignant lymphoma; intraoperative cytology can provide a valuable adjunct to frozen section diagnosis.

  19. Recurrent DGCR8, DROSHA, and SIX homeodomain mutations in favorable histology Wilms tumors | Office of Cancer Genomics (United States)

    We 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 that tumors with SIX1/2 and/or miRNAPG mutations show a pre-induction metanephric mesenchyme gene expression pattern and are significantly associated with both perilobar nephrogenic rests and 11p15 imprinting aberrations.

  20. Emotional Functioning and School Contentment in Adolescent Survivors of Acute Myeloid Leukemia, Infratentorial Astrocytoma, and Wilms Tumor


    Jóhannsdóttir, Inga M.; Moum, Torbjørn; Hjermstad, Marianne J.; Wesenberg, Finn; Hjorth, Lars; Schrøder, Henrik; Lähteenmäki, Päivi M.; Jónmundsson, Gudmundur; Loge, Jon H.


    Purpose: Cancer in childhood may disrupt normal developmental processes and cause psychosocial problems in adolescent survivors of childhood cancers (ACCSs). Previous studies report inconsistent findings. Study aims were to assess subjective well-being (SWB), psychological distress, and school contentment in survivors of three dissimilar childhood cancers. Patients and methods: Nordic patients treated for acute myeloid leukemia (AML), infratentorial astrocytoma (IA), and Wilms tumor (WT) in c...

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

    Directory of Open Access Journals (Sweden)

    Leila C.A. Cardoso


    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.

  2. Detection of Preoperative Wilms Tumor Rupture with CT: A Report from the Children’s Oncology Group (United States)

    Naranjo, Arlene; Hoffer, Fredric; Mullen, Elizabeth; Geller, James; Gratias, Eric J.; Ehrlich, Peter F.; Perlman, Elizabeth J.; Rosen, Nancy; Grundy, Paul; Dome, Jeffrey S.


    Purpose: To retrospectively determine the diagnostic performance of computed tomography (CT) in identifying the presence or absence of preoperative Wilms tumor rupture. Materials and Methods: The cohort was derived from the AREN03B2 study of the Children’s Oncology Group. The study was approved by the institutional review board and was compliant with HIPAA. Written informed consent was obtained before enrollment. The diagnosis of Wilms tumor rupture was established by central review of notes from surgery and/or pathologic examination. Seventy Wilms tumor cases with rupture were matched to 70 Wilms tumor controls without rupture according to age and tumor weight (within 6 months and 50 g, respectively). CT scans were independently reviewed by two radiologists, and the following CT findings were assessed: poorly circumscribed mass, perinephric fat stranding, peritumoral fat planes obscured, retroperitoneal fluid (subcapsular vs extracapsular), ascites beyond the cul-de-sac, peritoneal implants, ipsilateral pleural effusion, and intratumoral hemorrhage. All fluids were classified as hemorrhagic or nonhemorrhagic by using a cutoff of 30 HU. The relationship between CT findings and rupture was assessed with logistic regression models. Results: The sensitivity and specificity for detecting Wilms tumor rupture were 54% (36 of 67 cases) and 88% (61 of 69 cases), respectively, for reviewer 1 and 70% (47 of 67 cases) and 88% (61 of 69 cases), respectively, for reviewer 2. Interobserver agreement was substantial (ĸ = 0.76). All imaging signs tested, except peritoneal implants, intratumoral hemorrhage, and subcapsular fluid, showed a significant association with rupture (P ≤ .02). The attenuation of ascitic fluid did not have a significant correlation with rupture (P = .9990). Ascites beyond the cul-de-sac was the single best indicator of rupture for both reviewers, followed by perinephric fat stranding and retroperitoneal fluid for reviewers 1 and 2, respectively (P cul

  3. Expression of the Wilms' tumor gene WT1 in the murine urogenital system. (United States)

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


    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

  4. Nephron Progenitor But Not Stromal Progenitor Cells Give Rise to Wilms Tumors in Mouse Models with β-Catenin Activation or Wt1 Ablation and Igf2 Upregulation

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    Le Huang


    Full Text Available Wilms tumor, a common childhood tumor of the kidney, is thought to arise from undifferentiated renal mesenchyme. Variable tumor histology and the identification of tumor subsets displaying different gene expression profiles suggest that tumors may arise at different stages of mesenchyme differentiation and that this ontogenic variability impacts tumor pathology, biology, and clinical outcome. To test the tumorigenic potential of different cell types in the developing kidney, we used kidney progenitor-specific Cre recombinase alleles to introduce Wt1 and Ctnnb1 mutations, two alterations observed in Wilms tumor, into embryonic mouse kidney, with and without biallelic Igf2 expression, another alteration that is observed in a majority of tumors. Use of a Cre allele that targets nephron progenitors to introduce a Ctnnb1 mutation that stabilizes β-catenin resulted in the development of tumors with a predominant epithelial histology and a gene expression profile in which genes characteristic of early renal mesenchyme were not expressed. Nephron progenitors with Wt1 ablation and Igf2 biallelic expression were also tumorigenic but displayed a more triphasic histology and expressed early metanephric mesenchyme genes. In contrast, the targeting of these genetic alterations to stromal progenitors did not result in tumors. These data demonstrate that committed nephron progenitors can give rise to Wilms tumors and that committed stromal progenitors are less tumorigenic, suggesting that human Wilms tumors that display a predominantly stromal histology arise from mesenchyme before commitment to a stromal lineage.

  5. Prognostic importance of expression of the Wilms' tumor 1 gene in newly diagnosed acute promyelocytic leukemia. (United States)

    Hecht, Anna; Nolte, Florian; Nowak, Daniel; Nowak, Verena; Reinwald, Mark; Hanfstein, Benjamin; Faldum, Andreas; Büchner, Thomas; Spiekermann, Karsten; Sauerland, Cristina; Weiss, Christel; Hofmann, Wolf-Karsten; Lengfelder, Eva


    Wilms' tumor 1 gene (WT1) is known to be highly expressed in acute promyelocytic leukemia (APL) but information on its impact on prognosis is lacking. WT1 expression was analyzed in bone marrow samples of 79 patients with APL at initial diagnosis. Patients had a differing outcome according to their level of WT1 expression. In patients who achieved a complete remission (CR), low or high WT1 expression was significantly associated with inferior overall survival (OS) compared to intermediate WT1 expression (49% for WT1high vs. 63% for WT1low vs. 93% for WT1int; p=0.008). Moreover, there were significant differences in relapse-free survival (RFS) between the three expression groups (42% for WT1high vs. 63% for WT1low vs. 83% for WT1int; p=0.047). In multivariable analysis WT1 expression showed an independent prognostic impact on OS of responders to induction therapy. In conclusion, the level of WT1 expression can add prognostic information in APL risk stratification.

  6. Genomic characterization of Wilms' tumor suppressor 1 targets in nephron progenitor cells during kidney development. (United States)

    Hartwig, Sunny; Ho, Jacqueline; Pandey, Priyanka; Macisaac, Kenzie; Taglienti, Mary; Xiang, Michael; Alterovitz, Gil; Ramoni, Marco; Fraenkel, Ernest; Kreidberg, Jordan A


    The Wilms' tumor suppressor 1 (WT1) gene encodes a DNA- and RNA-binding protein that plays an essential role in nephron progenitor differentiation during renal development. To identify WT1 target genes that might regulate nephron progenitor differentiation in vivo, we performed chromatin immunoprecipitation (ChIP) coupled to mouse promoter microarray (ChIP-chip) using chromatin prepared from embryonic mouse kidney tissue. We identified 1663 genes bound by WT1, 86% of which contain a previously identified, conserved, high-affinity WT1 binding site. To investigate functional interactions between WT1 and candidate target genes in nephron progenitors, we used a novel, modified WT1 morpholino loss-of-function model in embryonic mouse kidney explants to knock down WT1 expression in nephron progenitors ex vivo. Low doses of WT1 morpholino resulted in reduced WT1 target gene expression specifically in nephron progenitors, whereas high doses of WT1 morpholino arrested kidney explant development and were associated with increased nephron progenitor cell apoptosis, reminiscent of the phenotype observed in Wt1(-/-) embryos. Collectively, our results provide a comprehensive description of endogenous WT1 target genes in nephron progenitor cells in vivo, as well as insights into the transcriptional signaling networks controlled by WT1 that might direct nephron progenitor fate during renal development.

  7. Wilms Tumor 1 Gene Mutations in Patients with Cytogenetically Normal Acute Myeloid Leukemia

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    Salah Aref


    Full Text Available OBJECTIVE: This study aimed to assess the prognostic impact of Wilms tumor 1 (WT1 mutations in cytogenetically normal acute myeloid leukemia (CN-AML among Egyptian patients. METHODS: Exons 1, 2, 3, 7, 8, and 9 of WT1 were screened for mutations in samples from 82 CNAML patients out of 203 newly diagnosed AML patients, of age ranging from 21 to 74 years, using high-resolution capillary electrophoresis. RESULTS: Eleven patients out of 82 (13.41% harbored WT1 mutations. Mutations were detected in exon 7 (n=7, exon 9 (n=2, exon 8 (n=1, and exon 3 (n=1, but not in exons 1 or 2. There was no statistically significant difference between the WT1 mutants and wild types as regards age, sex, French-American-British subtypes, and the prevalence of success of induction remission therapy (p=0.966; 28.6% vs. 29.3%. Patients with WT1 mutations had overall survival lower than patients with the wild type (HR=1.38; 95% CI 4.79-6.86; p=0.004. CONCLUSION: CN-AML patients with WT1 mutations have poor clinical outcome. We recommend molecular testing for WT1 mutations in patients with CN-AML at diagnosis in order to improve risk stratification of those patients.

  8. The Wilms' tumor gene Wt1 is required for normal development of the retina. (United States)

    Wagner, Kay-Dietrich; Wagner, Nicole; Vidal, Valerie P I; Schley, Gunnar; Wilhelm, Dagmar; Schedl, Andreas; Englert, Christoph; Scholz, Holger


    The Wilms' tumor gene Wt1 is known for its important functions during genitourinary and mesothelial formation. Here we show that Wt1 is necessary for neuronal development in the vertebrate retina. Mouse embryos with targeted disruption of Wt1 exhibit remarkably thinner retinas than age-matched wild-type animals. A large fraction of retinal ganglion cells is lost by apoptosis, and the growth of optic nerve fibers is severely disturbed. Strikingly, expression of the class IV POU-domain transcription factor Pou4f2 (formerly Brn-3b), which is critical for the survival of most retinal ganglion cells, is lost in Wt1(-/-) retinas. Forced expression of Wt1 in cultured cells causes an up-regulation of Pou4f2 mRNA. Moreover, the Wt1(-KTS) splice variant can activate a reporter construct carrying 5'-regulatory sequences of the human POU4F2. The lack of Pou4f2 and the ocular defects in Wt1(-/-) embryos are rescued by transgenic expression of a 280 kb yeast artificial chromosome carrying the human WT1 gene. Taken together, our findings demonstrate a continuous requirement for Wt1 in normal retina formation with a critical role in Pou4f2-dependent ganglion cell differentiation.

  9. Association between Long Interspersed Nuclear Element-1 Methylation and Relative Telomere Length in Wilms Tumor

    Institute of Scientific and Technical Information of China (English)

    Hui-Bo Chang; Ji-Zhen Zou; Cai He; Rui Zeng; Yuan-Yuan Li; Fei-Fei Ma; Zhuo Liu


    Background: DNA hypomethylation of long interspersed nuclear elements-1 (LINEs-1) occurs during carcinogenesis, whereas information addressing LINE-1 methylation in Wilms tumor (WT) is limited.The main purpose of our study was to quantify LINE-1 methylation levels and evaluate their relationship with relative telomere length (TL) in WT.Methods: We investigated LINE-1 methylation and relative TL using bisulfite-polymerase chain reaction (PCR) pyrosequencing and quantitative PCR, respectively, in 20 WT tissues, 10 normal kidney tissues and a WT cell line.Significant changes were analyzed by t-tests.Results: LINE-1 methylation levels were significantly lower (P < 0.05) and relative TLs were significantly shorter (P < 0.05) in WT compared with normal kidney.There was a significant positive relationship between LINE-1 methylation and relative TL in WT (r =0.671,P =0.001).LINE-1 Methylation levels were significantly associated with global DNA methylation (r =0.332, P < 0.01).In addition, relative TL was shortened and LINE-1 methylation was decreased in a WT cell line treated with the hypomethylating agent 5-aza-2'-deoxycytidine compared with untreated WT cell line.Conclusion: These results suggest that LINE-1 hypomethylation is common and may be linked to telomere shortening in WT.

  10. Senescence Process in Primary Wilms' Tumor Cell Culture Induced by p53 Independent p21 Expression. (United States)

    Theerakitthanakul, Korkiat; Khrueathong, Jeerasak; Kruatong, Jirasak; Graidist, Potchanapond; Raungrut, Pritsana; Kayasut, Kanita; Sangkhathat, Surasak


    Wilms tumor (WT) is an embryonal tumor occurring in developing kidney tissue. WT cells showing invasive cancer characteristics, also retain renal stem cell behaviours. In-vitro culture of WT is hampered by limited replicative potential. This study aimed to establish a longterm culture of WT cells to enable the study of molecular events to attempt to explain its cellular senescence. Primary cell cultures from fresh WT tumor specimen were established. Of 5 cultures tried, only 1 could be propagated for more than 7 passages. One culture, identified as PSU-SK-1, could be maintained > 35 passages and was then subjected to molecular characterization and evaluation for cancer characteristics. The cells consistently harbored concomitant mutations of CTNNB1 (Ser45Pro) and WT1 (Arg413Stop) thorough the cultivation. On Transwell invasion assays, the cells exhibited migration and invasion at 55% and 27% capability of the lung cancer cells, A549. On gelatin zymography, PSU-SK-1 showed high expression of the matrix metaloproteinase. The cells exhibited continuous proliferation with 24-hour doubling time until passages 28-30 when the growth slowed, showing increased cell size, retention of cells in G1/S proportion and positive β-galactosidase staining. As with those evidence of senescence in advanced cell passages, expression of p21 and cyclin D1 increased when the expression of β-catenin and its downstream protein, TCF, declined. There was also loss-of-expression of p53 in this cell line. In conclusion, cellular senescence was responsible for limited proliferation in the primary culture of WT, which was also associated with increased expression of p21 and was independent of p53 expression. Decreased activation of the Wnt signalling might explain the induction of p21 expression.

  11. Chest radiography versus chest CT in the evaluation for pulmonary metastases in patients with Wilms' tumor: a retrospective review

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    Wootton-Gorges, S.L.; Albano, E.A.; Riggs, J.M.; Ihrke, H.; Rumack, C.M.; Strain, J.D. [Colorado Univ., Denver, CO (United States). Dept. of Radiology


    Background. Determination of the presence of pulmonary metastases in children with Wilms' tumor is an important part of staging and treatment. We sought to compare the efficacy of chest radiography (CXR) and chest CT in the evaluation for pulmonary metastases in patients with Wilms' tumor. Materials and methods. This retrospective study included 83 children with Wilms' tumor diagnosed between 1980 and 1993. All patients with pulmonary nodules (n = 12) as well as 14 Wilms' tumor patients without pulmonary metastases (control group) had blinded review of the CXR and chest CTs by three pediatric radiologists. Presence, size, and certainty of metastatic diagnosis were recorded. Medical records were reviewed. The remaining 57 patients had review of their medical and imaging records to confirm the absence of pulmonary metastases. Results. Ten of the 12 with pulmonary masses had imaging available for review. Eight had both positive CXR and chest CT examinations. Two patients had pulmonary nodules seen by CT only: one had a right cardiophrenic angle mass and died as a result of liver metastases. The other had a solitary nodule, which proved to be a plasma-cell granuloma. Overall, the CXR and chest CT data concur in 79/81 (98 %). Conclusion. CXR alone appears adequate for the diagnosis or exclusion of pulmonary metastases in patients with Wilms' tumor. (orig.)

  12. Fibrocytes Regulate Wilms Tumor 1-Positive Cell Accumulation in Severe Fibrotic Lung Disease. (United States)

    Sontake, Vishwaraj; Shanmukhappa, Shiva K; DiPasquale, Betsy A; Reddy, Geereddy B; Medvedovic, Mario; Hardie, William D; White, Eric S; Madala, Satish K


    Collagen-producing myofibroblast transdifferentiation is considered a crucial determinant in the formation of scar tissue in the lungs of patients with idiopathic pulmonary fibrosis. Multiple resident pulmonary cell types and bone marrow-derived fibrocytes have been implicated as contributors to fibrotic lesions because of the transdifferentiation potential of these cells into myofibroblasts. In this study, we assessed the expression of Wilms tumor 1 (WT1), a known marker of mesothelial cells, in various cell types in normal and fibrotic lungs. We demonstrate that WT1 is expressed by both mesothelial and mesenchymal cells in idiopathic pulmonary fibrosis lungs but has limited or no expression in normal human lungs. We also demonstrate that WT1(+) cells accumulate in fibrotic lung lesions, using two different mouse models of pulmonary fibrosis and WT1 promoter-driven fluorescent reporter mice. Reconstitution of bone marrow cells into a TGF-α transgenic mouse model demonstrated that fibrocytes do not transform into WT1(+) mesenchymal cells, but they do augment accumulation of WT1(+) cells in severe fibrotic lung disease. Importantly, the number of WT1(+) cells in fibrotic lesions was correlated with severity of lung disease as assessed by changes in lung function, histology, and hydroxyproline levels in mice. Finally, inhibition of WT1 expression was sufficient to attenuate collagen and other extracellular matrix gene production by mesenchymal cells from both murine and human fibrotic lungs. Thus, the results of this study demonstrate a novel association between fibrocyte-driven WT1(+) cell accumulation and severe fibrotic lung disease. Copyright © 2015 by The American Association of Immunologists, Inc.

  13. Effect of abdominal irradiation on growth in boys treated for a Wilms' tumor

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    Wallace, W.H.; Shalet, S.M.; Morris-Jones, P.H.; Swindell, R.; Gattamaneni, H.R. (Christie Hospital, Manchester (England))


    To study the effect of abdominal irradiation on spinal growth in childhood we have measured final height, sitting height, and leg length in 30 male survivors of a Wilms' tumor. Twenty-one patients received whole abdominal irradiation by either megavoltage therapy (MV: n = 11) or orthovoltage therapy (OV: n = 10); the remainder received flank irradiation. To examine the effect of the adolescent growth spurt on the irradiated spine we have followed prospectively seven patients who received whole abdominal irradiation and nine patients who received flank irradiation through puberty. Compared to a normal population there is a modest reduction in median final standing height SDS (H.SDS: -1.15) accompanied by a marked reduction in median final sitting height SDS (S.HT SDS: -2.41) with no apparent effect on median subischial leg length SDS (SILL.SDS: 0.04). This reduction in spinal growth is reflected by a strongly positive disproportion score (DPS; (SILL SDS-S.HT SDS) + 2.81). The incidence of scoliosis after abdominal irradiation has been low (10%). During puberty there is a significant fall in median sitting height SDS after both whole abdominal (median fall: -0.9, P = 0.02) and flank irradiation (median fall: -1.85, P = 0.01), and this is reflected in a significant increase in disproportion (DPS: whole abdominal; median rise +1.4, P = 0.02: flank, median rise +1.34, P = 0.01). After MV irradiation there is a significant correlation between the degree of disproportion and the age at treatment (P less than 0.0005). The younger the patient is at treatment the more severe is the restriction on spinal growth and the shorter and more disproportionate they become as an adult. The estimated eventual loss in potential height from abdominal irradiation at the age of one is 10 cm and at five years is 7 cm.

  14. Integration of nutrition support into oncologic treatment protocols for high and low nutritional risk children with Wilms' tumor. A prospective randomized study. (United States)

    Rickard, K A; Godshall, B J; Loghmani, E S; Coates, T D; Grosfeld, J L; Weetman, R M; Lingard, C D; Foland, B B; Yu, P L; McGuire, W


    Benefits and risks of nutrition support were evaluated in 31 malnourished children with newly diagnosed Wilms' tumor managed according to the third National Wilms' Tumor Study protocol. Patients were classified at diagnosis as being at high nutritional risk (HNR, n = 19) or low nutritional risk (LNR, n = 12). Ten HNR patients were randomized to central parenteral nutrition (CPN) and nine HNR patients were randomized to peripheral parenteral nutrition (PPN) plus enteral nutrition (EN) for 4 weeks of initial intense treatment and EN (nutritional counseling, oral foods and supplements) thereafter. Thirteen HNR patients (seven CPN, six PPN) completed the protocol. Twelve LNR patients received EN; 11 Stage I malnourished patients were randomized to 10 or 26 weeks of chemotherapy. Dietary, anthropometric, and biochemical data were determined for HNR patients at weeks 0-4, 6, 13, 19, and 26 and for LNR patients at weeks 1, 2, 5, and 26. In HNR patients, adequate parenteral nutrition support reversed protein energy malnutrition (PEM), and prevented chemotherapy and radiotherapy delays due to granulocytopenia. CPN was superior to PPN in reversing PEM: energy intake, weight gain, and retinol binding protein were higher (P less than 0.05). LNR patients lost weight and fat reserves in the first 2 weeks of treatment; depletion persisted at week 5, and 25% had chemotherapy delays. Thereafter, EN reversed PEM in patients with both chemotherapy regimens. These data suggest that CPN is preferable during initial intense treatment for HNR patients, and that, although EN is ineffective in preventing depletion and treatment delays in the first 5 weeks of treatment for LNR patients, it is effective thereafter.

  15. Wilm's tumor-1 protein levels in urinary exosomes from diabetic patients with or without proteinuria.

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    Anuradha Kalani

    Full Text Available BACKGROUND: Podocyte injury is an early feature of diabetic nephropathy (DN. Recently, urinary exosomal Wilm's tumor-1 protein (WT1, shed by renal epithelial cells, has been proposed as a novel biomarker for podocyte injury. However, its usefulness as biomarker for early diabetic nephropathy has not been verified yet. We investigated urinary exosomal WT1 in type-1 diabetic patients to confirm its role as a non-invasive biomarker for predicting early renal function decline. METHODS: The expression of WT1 protein in urinary exosomes from spot urine samples of type-1 diabetes mellitus patients (n = 48 and healthy controls (n = 25 were analyzed. Patients were divided based on their urinary albumin excretion, ACR (mg/g creatinine into non- proteinuria group (ACR30 mg/g, n = 18. Regression analysis was used to assess the association between urinary exosomal levels of WT1 with parameters for renal function. Receiver Operating Characteristic (ROC curve analysis was used to determine the diagnostic performance of exosomal WT-1. RESULTS: WT1 protein was detected in 33 out of 48 diabetic patients and in only 1 healthy control. The levels of urinary exosomal WT1 protein is significantly higher (p = 0.001 in patients with proteinuria than in those without proteinuria. In addition, all the patients with proteinuria but only half of the patients without proteinuria were positive for exosomal WT1. We found that the level of exosomal WT1 were associated with a significant increase in urine protein-to-creatinine ratio, albumin-to-creatinine ratio, and serum creatinine as well as a decline in eGFR. Furthermore, patients exhibiting WT1-positive urinary exosomes had decreased renal function compared to WT1-negative patients. ROC analysis shows that WT-1 effectively predict GFR<60 ml. min-1/1.73 m(2. CONCLUSION: The predominant presence of WT1 protein in urinary exosomes of diabetic patients and increase in its expression level with decline in renal

  16. Overexpression of Wilms tumor 1 gene as a negative prognostic indicator in acute myeloid leukemia.

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    Xiaodong Lyu

    Full Text Available 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 (NPM1(mt/no FLT3(ITD, the unfavorable (FLT3(ITD 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.

  17. Avaliação do papel do cirurgião no tratamento do Tumor de Wilms: análise de um estudo cooperativo The role of surgery in the treatment of Wilms Tumor

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    S.T. Schettini


    , with the preset surgical approach protocol (in accordance with the National Wilms Tumor Study-NWTS.. MATERIAL AND METHOD: One hundred and sixty six operated on patients entered between October 1986 and December 1988, with the data updated until February 1992 were studied. The minimum follow-up period was 24 months for 147 patients (average 36 months. The remaining 19 patients were followed in the outpatient clinic for three to 18 months. RESULTS: After submitting these data to statistical analysis and the obtained results compared to those in the literature we observed that: -previous ligature of the renal vessels had no discernible effect on the ultimate outcome (relapses and mortality; -intra-operative tumoral rupture with contamination of the peritoneal cavity unfavorably interferes with the mortality rate; -even when the adrenal and the perirenal fat are normal from the surgeon's point of view, the histological findings showed tumor contamination in a number of cases; -there is a worse prognosis when the tumor weight exceeds 500 g; -according to the lymphonode evaluation, sur- geon's performance was not in accordance with the protocol recommendations, considering that the node evaluation was neglected in 56.6% of the patients. This led to a surgical staging error and has contributed to some unexpected results like: -lower relapses rate in the patients with affected lymphnodes; -higher incidence of lung metastases in patients without lymphnodes analysis; -stage II patients with better prognosis, compared to stage I patients and patients with stage I and III having similar relapse rates. CONCLUSION: From the above-referred results, we concluded that some omissions have happened jeopardizing accuracy of the surgical staging in a significant way. In this way, in our environment the oncologist in charge of the treatment of Wilms tumor bearers, must stablish a close integration with a surgical team, whose members have a precise understanding of the surgeon's importance

  18. A 4-gene expression score associated with high levels of Wilms Tumor-1 (WT1) expression is an adverse prognostic factor in acute myeloid leukaemia

    NARCIS (Netherlands)

    Niavarani, A. (Ahmadreza); Herold, T. (Tobias); Y. Reyal (Yasmin); M.C. Sauerland (Maria); T. Büchner (Thomas); W. Hiddemann (Wolfgang); S.K. Bohlander (Stefan); P.J.M. Valk (Peter); Bonnet, D. (Dominique)


    textabstractWilms Tumor-1 (WT1) expression level is implicated in the prognosis of acute myeloid leukaemia (AML). We hypothesized that a gene expression profile associated with WT1 expression levels might be a good surrogate marker. We identified high WT1 gene sets by comparing the gene expression p

  19. Dependence of Wilms tumor cells on signaling through insulin-like growth factor 1 in an orthotopic xenograft model targetable by specific receptor inhibition

    DEFF Research Database (Denmark)

    Bielen, Aleksandra; Box, Gary; Perryman, Lara


    pathway inactivation. By contrast, Wilms tumor cells established orthotopically within the kidney were histologically accurate and exhibited significantly elevated insulin-like growth factor-mediated signaling, and growth was significantly reduced on treatment with NVP-AEW541 in parallel with signaling...

  20. Mutations in the SIX1/2 pathway and the DROSHA/DGCR8 miRNA microprocessor complex underlie high-risk blastemal type Wilms tumors. (United States)

    Wegert, Jenny; Ishaque, Naveed; Vardapour, Romina; Geörg, Christina; Gu, Zuguang; Bieg, Matthias; Ziegler, Barbara; Bausenwein, Sabrina; Nourkami, Nasenien; Ludwig, Nicole; Keller, Andreas; Grimm, Clemens; Kneitz, Susanne; Williams, Richard D; Chagtai, Tas; Pritchard-Jones, Kathy; van Sluis, Peter; Volckmann, Richard; Koster, Jan; Versteeg, Rogier; Acha, Tomas; O'Sullivan, Maureen J; Bode, Peter K; Niggli, Felix; Tytgat, Godelieve A; van Tinteren, Harm; van den Heuvel-Eibrink, Marry M; Meese, Eckart; Vokuhl, Christian; Leuschner, Ivo; Graf, Norbert; Eils, Roland; Pfister, Stefan M; Kool, Marcel; Gessler, Manfred


    Blastemal histology in chemotherapy-treated pediatric Wilms tumors (nephroblastoma) is associated with adverse prognosis. To uncover the underlying tumor biology and find therapeutic leads for this subgroup, we analyzed 58 blastemal type Wilms tumors by exome and transcriptome sequencing and validated our findings in a large replication cohort. Recurrent mutations included a hotspot mutation (Q177R) in the homeo-domain of SIX1 and SIX2 in tumors with high proliferative potential (18.1% of blastemal cases); mutations in the DROSHA/DGCR8 microprocessor genes (18.2% of blastemal cases); mutations in DICER1 and DIS3L2; and alterations in IGF2, MYCN, and TP53, the latter being strongly associated with dismal outcome. DROSHA and DGCR8 mutations strongly altered miRNA expression patterns in tumors, which was functionally validated in cell lines expressing mutant DROSHA.

  1. 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


    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.

  2. 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

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    Marilia Fornaciari Grabois


    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

  3. Growth inhibition of breast cancer cell line MCF-7 by siRNA silencing of Wilms tumor 1 gene. (United States)

    Navakanit, Ruxapon; Graidist, Potchanapond; Leeanansaksiri, Wilairat; Dechsukum, Chavaboon


    RNA interference (RNAi) is sequence-specific inhibition of gene expression induced by double-stranded RNA. Define the role of Wilms tumor 1 gene (WT1) in breast cancer oncogenesis using RNAi. MCF-7 breast cancer cells, which express a high level of WT1, were transfected with synthetic small interfering RNA (siRNA) targeting WT1 (siRNA(WT1)) resulting in inhibition of WTI expression, as well as growth, in a dose- and time-dependent manner. The minimum concentration of siRNA(WT1) for growth inhibition and WT1 silencing was 25 nM and 50 nM respectively. WT1 expression was completely abolished at 200 nM siRNA,. These data suggest that WTI1is indispensable for the survival of breast cancer MCF-7 cell line.

  4. Factors possibly affecting prognosis in children with Wilms' tumor diagnosed before 24 months of age: A report from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) Wilms Tumor Working Group. (United States)

    D'Angelo, Paolo; Di Cataldo, Andrea; Terenziani, Monica; Bisogno, Gianni; Collini, Paola; Di Martino, Martina; Melchionda, Fraia; Mosa, Clara; Nantron, Marilina; Perotti, Daniela; Puccio, Giuseppe; Serra, Annalisa; Catania, Serena; Spreafico, Filippo


    Children with Wilms' tumor (WT) aged under 24 months (infants) have a better prognosis than older patients. Our aim was to study the epidemiology of this age group, with focus on the modality of diagnosis, tumor size, and association with malformations/syndromes, seeking to understand if any of these factors might be related to prognosis. Infants diagnosed with WT between 2003 and February 2010 were evaluated. A query form was used to collect data on the modality of WT diagnosis (symptomatic or incidental), tumor volume, maximum diameter, site, and stage. Data were collected for 117 of 124 WT infants registered. Twenty-four cases had an incidental diagnosis (ID) of renal mass, usually arising from an abdominal ultrasound performed for other reasons, and 93 had been diagnosed based on clinical signs/symptoms. The incidental cohort displayed unifocal disease, mean tumor diameter 5.52 cm, mean tumor volume 84.30 ml, and 14 patients showed associated malformations. Symptomatic patients had mean maximum tumor diameter of 10.18 cm, mean tumor volume of 451.18 ml, and six had associated malformations. Our study showed that 20% of the infants had an ID of WT; they had a relatively smaller nonmetastatic tumor and a higher rate of malformations than infants of the symptomatically diagnosed group, but we did not detect any difference in age at diagnosis between the two groups. Conversely, we found a significant difference in the 5-year event-free survival rate (P = 0.018) between infants under 1 year (96%), more frequently associated with congenital malformations, and infants 1-2 years (80%). © 2017 Wiley Periodicals, Inc.

  5. The Rare Association of Spina Bifida and Extrarenal Wilms Tumor: A Case Report and Review of the Literature. (United States)

    Igbaseimokumo, Usiakimi; Cartwright, Cathy; Lewing, Karen; Hutchison, Lisa; Habeebu, Sultan


    The diagnosis of nephroblastoma outside of the kidneys, in the absence of a renal primary tumor, is known as extrarenal Wilms tumor (ERWT). ERWT is an uncommon entity that typically involves the embryonic path of the developing kidneys and gonads. The occurrence of ERWT in a dysraphic spine is uncommon, with no reported cases of preoperative diagnosis, with all cases diagnosed at pathology. These tumors are malignant and ideally should be completely excised. Thus, preoperative diagnosis would be highly desirable. A newborn female was found to have a lumbar lipoma. Magnetic resonance imaging (MRI) was performed to rule out lipomyelomeningocele. The MRI showed a dorsal lipoma on the terminal spinal cord, as well as a 2 × 2 cm uniformly enhancing mass abutting the bifid posterior elements of L5. The lesion was completely excised, and the pathological diagnosis was ERWT. We report this case with a review of the literature to raise awareness of this association, illustrate the key imaging findings, and document the clinical outcome. The lack of pathognomonic radiologic features makes the preoperative diagnosis extremely difficult, but a diagnosis of ERWT should be considered in the context of a soft tissue mass without the typical imaging features of a hemangioma or teratoma. Copyright © 2017 Elsevier Inc. All rights reserved.

  6. In Vivo Assays for Assessing the Role of the Wilms' Tumor Suppressor 1 (Wt1) in Angiogenesis. (United States)

    McGregor, Richard J; Ogley, R; Hadoke, Pwf; Hastie, Nicholas


    The Wilms' tumor suppressor gene (WT1) is widely expressed during neovascularization, but it is almost entirely absent in quiescent adult vasculature. However, in vessels undergoing angiogenesis, WT1 is dramatically upregulated. Studies have shown Wt1 has a role in both tumor and ischemic angiogenesis, but the mechanism of Wt1 action in angiogenic tissue remains to be elucidated. Here, we describe two methods for induction of in vivo angiogenesis (subcutaneous sponge implantation, femoral artery ligation) that can be used to assess the influence of Wt1 on new blood vessel formation. Subcutaneously implanted sponges stimulate an inflammatory and fibrotic response including cell infiltration and angiogenesis. Femoral artery ligation creates ischemia in the distal hindlimb and produces an angiogenic response to reperfuse the limb which can be quantified in vivo by laser Doppler flowmetry. In both of these models, the role of Wt1 in the angiogenic process can be assessed using histological/immunohistochemical staining, molecular analysis (qPCR) and flow cytometry. Furthermore, combined with suitable genetic modifications, these models can be used to explore the causal relationship between Wt1 expression and angiogenesis and to trace the lineage of cells expressing Wt1. This approach will help to clarify the importance of Wt1 in regulating neovascularization in the adult, and its potential as a therapeutic target.

  7. Risk stratification and consecutive prognosis progresses in childhood Wilms tumors. Two cases report. (United States)

    Diaconescu, S; Olaru, C; Mihailă, D; Aprodu, S G; Miron, I


    Even if Wilms' tumour is the commonest primary malignant neoplasia in children, it maintained a continuous interest due to actual therapeutic successes contrasting with the customary delayed diagnosis, malignancy and specific risk factors. Two recent illustrative cases from our clinic are presented. The first one - a little girl of 22 months with repeated admissions for habitual constipation and psychomental disturbances revealed at abdominal ultrasonography a hypo-echoic mass at the superior pole of the right kidney. CT confirmed the presence of a solid homogeneous mass of 23/25 mm without node or distant metastases, suggestive for Wilms' tumour. Conforming to SIOP protocol she received chemotherapy and after 4 weeks a superior polar nephrectomy was performed. Pathology confirmed the diagnosis of triphasic nephroblastoma of intermediary risk. Postoperative chemotherapy according to the protocol SIOP assured the cure with a disease free period of 23 months. The second case - also a girl, of 3 years, is admitted for constipation, pain in the left flank and mental retard (QI=40). Ultrasonography determined a huge mass (Ø~6 cm) situated at the superior pole of the left kidney. CT attested a nonhomogeneous, encapsulated tumour image of 6.2/5.4/7.2 cm in large posterolateral contact with the renal parenchyma out of which it appears to be developed. The diagnosis of WT is strongly suggested and after chemotherapy a radical left nephrectomy with initial vessels ligature was performed. Pathology: stage IIb nephroblastoma with focal epithelial anaplasia. After surgery she continued the chemotherapy (HR regime), was cured and excepting a medullar aplasia is in a good health after 24 months. Our both cases were girls under 3 years, presenting nonspecific features: constipation and psychic troubles, the tumour being incidentally discovered by the abdominal ultrasonography. CT established the diagnosis. Conventional chemotherapy framing adapted to the tumour's stage and

  8. Influence of Pulmonary Nodules on Chest Computed Tomography and Risk of Recurrence in Stage IV Wilms Tumor

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    Kirkland, Robert S. [National Institutes of Health, Bethesda, Maryland (United States); Nanda, Ronica H., E-mail: [Department of Radiation Oncology, Emory University Winship Cancer Institute, Atlanta, Georgia (United States); Alazraki, Adina [Department of Radiology, Aflac Cancer Center at Children' s Healthcare of Atlanta, Atlanta, Georgia (United States); Esiashvili, Natia [Department of Radiation Oncology, Emory University Winship Cancer Institute, Atlanta, Georgia (United States)


    Purpose: Chest computed tomography (CT) is currently accepted as the main modality for initial disease staging and response assessment in Wilms tumor (WT). However, there is great variability in the number and size of lung metastases at the time of diagnosis and after induction chemotherapy. There is a lack of clinical evidence as to how this variability in tumor burden affects choice of therapy and disease outcome. This study sought to evaluate a previously proposed lung metastases risk stratification system based on CT findings and clinical outcomes in stage IV WT patients. Methods and Materials: Thirty-five pediatric patients with a diagnosis of stage IV WT with evaluable pre- and postdiagnosis CT scans between 1997 and 2012 were included in the analysis. Patients were divided into low-, intermediate-, and high-risk categories based on the size and number of pulmonary metastases before and after 6 weeks of chemotherapy. Association of the lung risk groups with lung recurrence-free survival and overall survival at each time point was analyzed with relevant covariates. Results: Risk group distribution both at diagnosis and after induction chemotherapy was not influenced by tumor histology. Initial risk grouping suggested an association with disease-free survival at 5 years (P=.074); however, the most significant correlation was with postinduction chemotherapy disease status (P=.027). In patients with an intermediate or high burden of disease after 6 weeks of chemotherapy, despite receiving whole-lung and boost irradiation, survival outcomes were poorer. Conclusions: Pulmonary tumor burden in stage IV WT on chest CT can predict disease outcome. Patients with intermediate- or low-risk disease, especially after induction therapy, have a higher risk for recurrence. After prospective validation, this method may become a valuable tool in adaptation of therapy to improve outcome.

  9. Construction of a protein-protein interaction network of Wilms' tumor and pathway prediction of molecular complexes. (United States)

    Teng, W J; Zhou, C; Liu, L J; Cao, X J; Zhuang, J; Liu, G X; Sun, C G


    Wilms' tumor (WT), or nephroblastoma, is the most common malignant renal cancer that affects the pediatric population. Great progress has been achieved in the treatment of WT, but it cannot be cured at present. Nonetheless, a protein-protein interaction network of WT should provide some new ideas and methods. The purpose of this study was to analyze the protein-protein interaction network of WT. We screened the confirmed disease-related genes using the Online Mendelian Inheritance in Man database, created a protein-protein interaction network based on biological function in the Cytoscape software, and detected molecular complexes and relevant pathways that may be included in the network. The results showed that the protein-protein interaction network of WT contains 654 nodes, 1544 edges, and 5 molecular complexes. Among them, complex 1 is predicted to be related to the Jak-STAT signaling pathway, regulation of hematopoiesis by cytokines, cytokine-cytokine receptor interaction, cytokine and inflammatory responses, and hematopoietic cell lineage pathways. Molecular complex 4 shows a correlation of WT with colorectal cancer and the ErbB signaling pathway. The proposed method can provide the bioinformatic foundation for further elucidation of the mechanisms of WT development.

  10. Frequency and timing of loss of imprinting at 11p13 and 11p15 in Wilms' tumor development. (United States)

    Brown, Keith W; Power, Frances; Moore, Beth; Charles, Adrian K; Malik, Karim T A


    Epigenetic changes occur frequently in Wilms' tumor (WT), especially loss of imprinting (LOI) of IGF2/H19 at 11p15. Our previous results have identified imprinted transcripts (WT1-AS and AWT1) from the WT1 locus at 11p13 and showed LOI of these in some WTs. In this article, we set out to test the relationship between LOI at 11p13 and 11p15 and their timing in WT progression relative to other genetic changes. We found a higher level (83%) of 11p13 LOI in WT than of 11p15 LOI (71%). There was no correlation between methylation levels at the 11p13 and 11p15 differentially methylated regions or between allelic expression of WT1-AS/AWT1 and IGF2. Interestingly, retention of normal imprinting at 11p13 was associated with a small group of relatively late-onset, high-stage WTs. An examination of genetic and epigenetic alterations in nephrogenic rests, which are premalignant WT precursors, showed that LOI at both 11p13 and 11p15 occurred before either 16q loss of heterozygosity (LOH) or 7p LOH. This suggests that these LOH events are very unlikely to be a cause of LOI but that LOH may act by potentiating the effects of overexpression of IGF2 and/or WT1-AS/AWT1 that result from LOI.

  11. Wilms' tumor gene 1 (WT1 silencing inhibits proliferation of malignant peripheral nerve sheath tumor sNF96.2 cell line.

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    Rosalba Parenti

    Full Text Available Wilms' tumor gene 1 (WT1 plays complex roles in tumorigenesis, acting as tumor suppressor gene or an oncogene depending on the cellular context. WT1 expression has been variably reported in both benign and malignant peripheral nerve sheath tumors (MPNSTs by means of immunohistochemistry. The aim of the present study was to characterize its potential pathogenetic role in these relatively uncommon malignant tumors. Firstly, immunohistochemical analyses in MPNST sNF96.2 cell line showed strong WT1 staining in nuclear and perinuclear areas of neoplastic cells. Thus, we investigated the effects of silencing WT1 by RNA interference. Through Western Blot analysis and proliferation assay we found that WT1 knockdown leads to the reduction of cell growth in a time- and dose-dependent manner. siWT1 inhibited proliferation of sNF96.2 cell lines likely by influencing cell cycle progression through a decrease in the protein levels of cyclin D1 and inhibition of Akt phosphorylation compared to the control cells. These results indicate that WT1 knockdown attenuates the biological behavior of MPNST cells by decreasing Akt activity, demonstrating that WT1 is involved in the development and progression of MPNSTs. Thus, WT1 is suggested to serve as a potential therapeutic target for MPNSTs.

  12. Expression of the Wilms' tumor gene WT1 in human malignant mesothelioma cell lines and relationship to platelet-derived growth factor A and insulin- like growth factor 2 expression

    NARCIS (Netherlands)

    A.W. Langerak (Anton); K.A. Williamson (K.); K. Miyagawa (K.); A. Hagemeijer (Anne); M.A. Versnel (Marjan); N. Hastie (Nick)


    textabstractMutations in the WT1 tumor suppressor gene are known to contribute to the development of Wilms' tumor (WT) and associated gonadal abnormalities. WT1 is expressed principally in the fetal kidney, developing gonads, and spleen and also in the mesothelium, which lines the coelomic cavities.

  13. Emotional Functioning and School Contentment in Adolescent Survivors of Acute Myeloid Leukemia, Infratentorial Astrocytoma, and Wilms Tumor. (United States)

    Jóhannsdóttir, Inga M; Moum, Torbjørn; Hjermstad, Marianne J; Wesenberg, Finn; Hjorth, Lars; Schrøder, Henrik; Lähteenmäki, Päivi M; Jónmundsson, Gudmundur; Loge, Jon H


    Purpose: Cancer in childhood may disrupt normal developmental processes and cause psychosocial problems in adolescent survivors of childhood cancers (ACCSs). Previous studies report inconsistent findings. Study aims were to assess subjective well-being (SWB), psychological distress, and school contentment in survivors of three dissimilar childhood cancers. Patients and methods: Nordic patients treated for acute myeloid leukemia (AML), infratentorial astrocytoma (IA), and Wilms tumor (WT) in childhood from 1985 to 2001, aged ≥1 year at diagnosis, and aged 13-18 years at the time of study were eligible for this questionnaire-based survey that included items on SWB, psychological distress, school contentment, self-esteem, and personality traits; 65% (151/231) responded. An age-equivalent group from a Norwegian health survey (n=7910) served as controls. Results: The median age of ACCSs was 16 years; 52% were males. ACCSs reported better SWB (p=0.004) and self-esteem (p<0.001). They had fewer social problems in school (p=0.004) and their school contentment tended to be higher than controls. SWB and school contentment were positively influenced by self-esteem. However, ACCSs reported higher levels of psychological distress (p=0.002), mostly attributable to general worrying. No significant differences in outcomes were found across diagnoses, and time since diagnosis did not significantly affect the results. Conclusion: The overall emotional functioning of ACCSs was good, possibly due to changes in their perception of well-being after having survived a life-threatening disease. However, they seemed more worried than their peers. This may cause an additional strain at a vulnerable period in life.

  14. Prognostic impact of Wilms tumor gene mutations in Egyptian patients with acute myeloid leukemia with normal karyotype. (United States)

    Zidan, Magda Abdel Aziz; Kamal Shaaban, Howyda M; Elghannam, Doaa M


    The Wilms' tumor (WT1) gene mutations were detected in patients with most forms of acute leukemia. However, the biological significance and the prognostic impact of WT1 mutation in Egyptian patients with acute myeloid leukemia with normal karyotype (AML-NK) are still uncertain. We aimed to evaluate the incidence and clinical relevance of WT1 gene mutations in acute myeloid leukemia with normal karyotype (AML-NK). Exons 7 and 9 of WT1 were screened in samples from 216 adult NK-AML using polymerase chain reaction single-strand conformation polymorphism techniques. Twenty-three patients (10.6%) harbored WT1 mutations. Younger ages and higher marrow blasts were significantly associated with WT1 mutations (P = 0.006 and 0.003 respectively). Complete remission rates were significantly lower in patients with WT1 mutations than those with WT1 wild-type (P = 0.015). Resistance, relapse, and mortality rates were significantly higher in patients with WT1 mutations than those without (P = 0.041, 0.016, and 0.008 respectively). WT1 mutations were inversely associated with NPM1 mutations (P = 0.007). Patients with WT1 mutations had worse disease-free survival (P mutations independently predicted worse DFS (P mutational status. In conclusion, WT1 mutations are a negative prognostic indicator in intensively treated patients with AML-NK, may be a part of molecularly based risk assessment and risk-adapted treatment stratification of patients with AML-NK.

  15. TARGET Researchers Identify Mutations in SIX1/2 and microRNA Processing Genes in Favorable Histology Wilms Tumor | Office of Cancer Genomics (United States)

    TARGET researchers molecularly characterized favorable histology Wilms tumor (FHWT), a pediatric renal cancer. Comprehensive genome and transcript analyses revealed single-nucleotide substitution/deletion mutations in microRNA processing genes (15% of FHWT patients) and Sine Oculis Homeobox Homolog 1/2 (SIX1/2) genes (7% of FHWT patients). SIX1/2 genes play a critical role in renal development and were not previously associated with FHWT, thus presenting a novel role for SIX1/2 pathway aberrations in this disease.

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

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    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.

  17. Ataxia-telangiectasia and wilms tumor: reduced treatment but early relapse. (United States)

    Pérez-Villena, Ana; Cormenzana, María; de Prada, Inmaculada; Pérez-Martínez, Antonio; Aleo, Esther


    Ataxia-telangiectasia (A-T) is an autosomal recessive disease characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, immunodeficiency, a high incidence of lymphoreticular tumors, and an increased sensitivity to chemoradiotherapy-induced DNA damage. The appropriate cancer therapy remains unknown because of high toxicity rates with full-dose conventional protocols. We present a patient with A-T and nephroblastoma, who received an adapted treatment regimen. To our knowledge this is the second report on nephroblastoma in a patient with A-T but the first with confirmed premortem studies. Although the patient tolerated the chemotherapy regimen well, the patient relapsed and died a year after initial diagnosis.

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

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    Tao Yan-Fang


    Full Text Available Abstract Background 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. Methods 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. Results 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 cm3; YM155 10 mg/kg: 0.95 ± 0.55 cm3 compared to DMSO group (DMSO: 3.70 ± 2.4 cm3 or PBS group cells (PBS: 3.78 ± 2.20 cm3, ANOVA P Conclusions The present study demonstrates that YM155 treatment resulted in apoptosis and inhibition of cell proliferation of SK-NEP-1cells. YM155 had significant role and little side effect in the treatment of SK-NEP-1 xenograft tumors. Real-time PCR array analysis firstly showed expression profile of genes dyes-regulated after YM155 treatment. IPA analysis also represents new molecule mechanism of YM155 treatment, such as NR3C1 and dexamethasone may be new target of YM155. And our results may provide new clues of molecular mechanism of apoptosis induced by YM155.

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

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    Roberto I. Lopes


    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.

  20. The Wilms' tumor antigen is a novel target for human CD4+ regulatory T cells: implications for immunotherapy. (United States)

    Lehe, Cynthia; Ghebeh, Hazem; Al-Sulaiman, Abdullah; Al Qudaihi, Ghofran; Al-Hussein, Khaled; Almohareb, Fahad; Chaudhri, Naeem; Alsharif, Fahad; Al-Zahrani, Hazza; Tbakhi, Abdelghani; Aljurf, Mahmoud; Dermime, Said


    Compelling evidences indicate a key role for regulatory T cells (T(reg)) on the host response to cancer. The Wilms' tumor antigen (WT1) is overexpressed in several human leukemias and thus considered as promising target for development of leukemia vaccine. However, recent studies indicated that the generation of effective WT1-specific cytotoxic T cells can be largely affected by the presence of T(regs). We have generated T-cell lines and clones that specifically recognized a WT1-84 (RYFKLSHLQMHSRKH) peptide in an HLA-DRB1*0402-restricted manner. Importantly, they recognized HLA-DRB1*04-matched fresh leukemic cells expressing the WT1 antigen. These clones exerted a T helper 2 cytokine profile, had a CD4(+)CD25(+)Foxp3(+)GITR(+)CD127(-) T(reg) phenotype, and significantly inhibited the proliferative activity of allogeneic T cells independently of cell contact. Priming of alloreactive T cells in the presence of T(regs) strongly inhibited the expansion of natural killer (NK), NK T, and CD8(+) T cells and had an inhibitory effect on NK/NK T cytotoxic activity but not on CD8(+) T cells. Furthermore, priming of T cells with the WT1-126 HLA-A0201-restricted peptide in the presence of T(regs) strongly inhibited the induction of anti-WT1-126 CD8(+) CTL responses as evidenced by both very low cytotoxic activity and IFN-gamma production. Moreover, these T(reg) clones specifically produced granzyme B and selectively induced apoptosis in WT1-84-pulsed autologous antigen-presenting cells but not in apoptotic-resistant DR4-matched leukemic cells. Importantly, we have also detected anti-WT1-84 interleukin-5(+)/granzyme B(+)/Foxp3(+) CD4(+) T(regs) in five of eight HLA-DR4(+) acute myeloid leukemia patients. Collectively, our in vitro and in vivo findings strongly suggest important implications for the clinical manipulation of T(regs) in cancer patients.

  1. Stages of Wilms Tumor (United States)

    ... abdomen. Blood in the urine. High blood pressure (headache, feeling very tired, chest pain, or trouble seeing ... Cancer Late Effects of Treatment for Childhood Cancer Adolescents and Young Adults with Cancer Children with Cancer: ...

  2. Multiple tumor types including leiomyoma and Wilms tumor in a patient with Gorlin syndrome due to 9q22.3 microdeletion encompassing the PTCH1 and FANC-C loci. (United States)

    Garavelli, Livia; Piemontese, Maria Rosaria; Cavazza, Alberto; Rosato, Simonetta; Wischmeijer, Anita; Gelmini, Chiara; Albertini, Enrico; Albertini, Giuseppe; Forzano, Francesca; Franchi, Fabrizia; Carella, Massimo; Zelante, Leopoldo; Superti-Furga, Andrea


    Gorlin syndrome or nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant condition mainly characterized by the development of mandibular keratocysts which often have their onset during the second decade of life and/or multiple basal cell carcinoma (BCC) normally arising during the third decade. Cardiac and ovarian fibromas can be found. Patients with NBCCS develop the childhood brain malignancy medulloblastoma (now often called primitive neuro-ectodermal tumor [PNET]) in 5% of cases. The risk of other malignant neoplasms is not clearly increased, although lymphoma and meningioma can occur in this condition. Wilms tumor has been mentioned in the literature four times. We describe a patient with a 10.9 Mb 9q22.3 deletion spanning 9q22.2 through 9q31.1 that includes the entire codifying sequence of the gene PTCH1, with Wilms tumor, multiple neoplasms (lung, liver, mesenteric, gastric and renal leiomyomas, lung typical carcinoid tumor, adenomatoid tumor of the pleura) and a severe clinical presentation. We propose including leiomyomas among minor criteria of the NBCCS.

  3. Wilms' tumour (nephroblastoma)

    African Journals Online (AJOL)

    surgeon who first described this type of tumour in 1899. Wilms' tumour .... Open biopsy should be avoided at all costs, as it. 'upstages' the tumour. Survival ... surgeon. No laparoscopic surgery should be done, as the whole abdomen has to be.

  4. Quantitative assessment of Wilms tumor 1 expression by real-time quantitative polymerase chain reaction in patients with acute myeloblastic leukemia

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    Hossein Ayatollahi


    Full Text Available Background: The Wilms tumor 1 (WT1 gene is originally defined as a tumor suppressor gene and a transcription factor that overexpressed in leukemic cells. It is highly expressed in more than 80% of acute myeloid leukemia (AML patients, both in bone marrow (BM and in peripheral blood (PB, and it is used as a powerful and independent marker of minimal residual disease (MRD; we have determined the expression levels of the WT1 by real-time quantitative polymerase chain reaction (RQ-PCR in PB and BM in 126 newly diagnosed AML patients. Materials and Methods: This study was done in molecular pathology and cancer research center from April 2014 to June 2015, RQ-PCR method was used to determine the WT1 gene expression in BM and/or PB samples from 126 patients of AML, we cloned both WT1 and ABL genes for creating a standard curve, and we calculate copy number of WT1 genes in patients. Results: A total of 126 AML patients consist of 70 males (55.6% and 56 females (44.4%, with a median age of 26 years; 104 (81% patients out of 126 show overexpression of WT1 gene. We also concomitant monitoring of fusion transcripts (PML RARa, AML1-ETO, MLL-MLL, CBFb-MYH11, or DEK-CAN in our patients, the AML1-ETO group showing remarkably low levels of WT1 compared with other fusion transcript and the CBFB-MYH11 showing high levels of WT1. Conclusion: We conclude that WT1 expression by RQ-PCR in AML patients may be employed as an independent tool to detect MRD in the majority of normal karyotype AML patients.

  5. Induction of complete and molecular remissions in acute myeloid leukemia by Wilms' tumor 1 antigen-targeted dendritic cell vaccination.

    NARCIS (Netherlands)

    Tendeloo, V.F. Van; Velde, A. van de; Driessche, A. Van; Cools, N.; Anguille, S.; Ladell, K.; Gostick, E.; Vermeulen, K.; Pieters, K.; Nijs, G.; Stein, B.; Smits, E.L.; Schroyens, W.A.; Gadisseur, A.P.; Vrelust, I.; Jorens, P.G.; Goossens, H.; Vries, I.J.M. de; Price, D.A.; Oji, Y.; Oka, Y.; Sugiyama, H.; Berneman, Z.N.


    Active immunization using tumor antigen-loaded dendritic cells holds promise for the adjuvant treatment of cancer to eradicate or control residual disease, but so far, most dendritic cell trials have been performed in end-stage cancer patients with high tumor loads. Here, in a phase I/II trial, we i

  6. 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


    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.

  7. The Wilms tumor gene, Wt1, is critical for mouse spermatogenesis via regulation of sertoli cell polarity and is associated with non-obstructive azoospermia in humans.

    Directory of Open Access Journals (Sweden)

    Xiao Na Wang

    Full Text Available Azoospermia is one of the major reproductive disorders which cause male infertility in humans; however, the etiology of this disease is largely unknown. In the present study, six missense mutations of WT1 gene were detected in 529 human patients with non-obstructive azoospermia (NOA, indicating a strong association between WT1 mutation and NOA. The Wilms tumor gene, Wt1, is specifically expressed in Sertoli cells (SCs which support spermatogenesis. To examine the functions of this gene in spermatogenesis, Wt1 was deleted in adult testis using Wt1(flox and Cre-ER(TM mice strains. We found that inactivation of Wt1 resulted in massive germ cell death and only SCs were present in most of the seminiferous tubules which was very similar to NOA in humans. In investigating the potential mechanism for this, histological studies revealed that the blood-testis barrier (BTB was disrupted in Wt1 deficient testes. In vitro studies demonstrated that Wt1 was essential for cell polarity maintenance in SCs. Further studies found that the expression of cell polarity associated genes (Par6b and E-cadherin and Wnt signaling genes (Wnt4, Wnt11 were downregulated in Wt1 deficient SCs, and that the expression of Par6b and E-cadherin was regulated by Wnt4. Our findings suggest that Wt1 is important in spermatogenesis by regulating the polarity of SCs via Wnt signaling pathway and that WT1 mutation is one of the genetic causes of NOA in humans.

  8. Wilms Tumor 1 Expression and Pre-emptive Immunotherapy in Patients with Acute Myeloid Leukemia Undergoing an Allogeneic Hemopoietic Stem Cell Transplantation. (United States)

    Di Grazia, Carmen; Pozzi, Sarah; Geroldi, Simona; Grasso, Raffaella; Miglino, Maurizio; Colombo, Nicoletta; Tedone, Elisabetta; Luchetti, Silvia; Lamparelli, Teresa; Gualandi, Francesca; Ibatici, Adalberto; Bregante, Stefania; Van Lint, Maria Teresa; Raiola, Anna Maria; Dominietto, Alida; Varaldo, Riccardo; Galaverna, Federica; Ghiso, Anna; Sica, Simona; Bacigalupo, Andrea


    Minimal residual disease (MRD) was monitored by Wilms tumor 1 (WT1) expression in 207 patients with acute myeloid leukemia (AML) after an allogeneic hemopoietic stem cell transplantation (HSCT) as a trigger to initiate pre-emptive immunotherapy (IT) with cyclosporin discontinuation and/or donor lymphocyte infusion. The trigger for IT was WT1 ≥ 180 copies/10(4) Abelson cells in marrow cells in the first group of 122 patients (WT1-180) and ≥ 100 copies in a subsequent group of 85 patients (WT1-100). Forty patients received IT. The cumulative incidence (CI) of relapse was 76% in WT1-180 (n = 17) versus 29% in WT1-100 patients (n = 23) receiving IT (P = .006); the leukemia-free survival from MRD positivity was 23% versus 74%, respectively (P = .003). We then looked at the entire AML patient population (n = 207). WT1-180 and WT1-100 patients were comparable for disease phase and age. The overall 4-year CI of transplantation-related mortality was 13% in both groups; the CI of leukemia relapse was 38% in the WT1-180 and 28% in the WT1-100 patients (P = .05) and leukemia-free survival was 56% versus 48%, respectively (P = .07). In conclusion, we suggests that WT1-based pre-emptive immunotherapy is feasible in patients with undergoing an allogeneic HSCT. The protective effect on relapse is greater when IT is triggered at lower levels of WT1.

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

    Directory of Open Access Journals (Sweden)

    Gowda KM Srinivasa


    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.

  10. Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors (United States)


    Childhood Renal Cell Carcinoma; Clear Cell Renal Cell Carcinoma; Clear Cell Sarcoma of the Kidney; Papillary Renal Cell Carcinoma; Rhabdoid Tumor of the Kidney; Stage I Renal Cell Cancer; Stage I Renal Wilms Tumor; Stage II Renal Cell Cancer; Stage II Renal Wilms Tumor; Stage III Renal Cell Cancer; Stage III Renal Wilms Tumor; Stage IV Renal Cell Cancer; Stage IV Renal Wilms Tumor

  11. Treatment Options for Wilms Tumor (United States)

    ... abdomen. Blood in the urine. High blood pressure (headache, feeling very tired, chest pain, or trouble seeing ... Cancer Late Effects of Treatment for Childhood Cancer Adolescents and Young Adults with Cancer Children with Cancer: ...

  12. A Study of CD45RA+ Depleted Haploidentical Stem Cell Transplantation in Children With Relapsed or Refractory Solid Tumors and Lymphomas (United States)


    Ewing Sarcoma; Gastrointestinal Tumor; Germ Cell Tumor; Hepatic Tumor; Lymphoma; Wilms Tumor; Rhabdoid Tumor; Clear Cell Carcinoma; Renal Cell Carcinoma; Melanoma; Neuroblastoma; Rhabdomyosarcoma; Non-rhabdomyosarcoma

  13. The Wilms' Tumor Gene WT1 −17AA/−KTS Splice Variant Increases Tumorigenic Activity Through Up-Regulation of Vascular Endothelial Growth Factor in an In Vivo Ovarian Cancer Model

    Directory of Open Access Journals (Sweden)

    Keiko Yamanouchi


    Full Text Available The Wilms' tumor 1 gene WT1 encodes a zinc transcription factor involved in a variety of cancer-related processes. In this study, we sought to investigate the effects of WT1 splice variants on tumorigenic activity and survival in an in vivo ovarian cancer model. To this end, we established stable ovarian cancer cell lines transduced with lentiviral constructs containing each of the four WT1 splice variants (−17AA/−KTS, +17AA/−KTS, −17AA/+KTS, and +17AA/+KTS. In mice inoculated intraperitoneally with SKOV3ip1 cells expressing WT1 −17AA/−KTS, disseminated tumor weights and production of ascites were significantly increased compared with those in mice inoculated with cells expressing the control vector. The overall survival in mice inoulated with WT1 −17AA/−KTS-expressing cells was significantly shorter than that in mice inoculated with control cells (P = .0115. Immunoblot analysis revealed that WT1 −17AA/−KTS significantly increased the expression of vascular endothelial growth factor (VEGF compared with the control. Greater numbers of CD31-immunopositive vessels were observed in tumors from mice injected with cells expressing WT1 −17AA/−KTS than in tumors from control mice. Finally, WT1 −17AA/−KTS significantly increased tumor microvessel density compared with that in the control (P < .05. Treatment with anti-VEGF antibody (bevacizumab inhibited tumor growth, dissemination, and ascites production in mice injected with cells expressing WT1 −17AA/−KTS. The overexpression of WT1 −17AA/−KTS induced a more aggressive phenotype in ovarian cancer cells through VEGF up-regulation in an in vivo ovarian cancer model. Our findings indicated that WT1 −17AA/−KTS enhanced tumorigenic activity and could decreased patient survival through up-regulation of VEGF expression in ovarian cancers.

  14. Wilms' tumor protein 1 (WT1) peptide vaccination in AML patients: predominant TCR CDR3β sequence associated with remission in one patient is detectable in other vaccinated patients. (United States)

    Ochsenreither, Sebastian; Fusi, Alberto; Geikowski, Anne; Stather, David; Busse, Antonia; Stroux, Andrea; Letsch, Anne; Keilholz, Ulrich


    Clinically effective T-cell responses can be elicited by single peptide vaccination with Wilms' tumor 1 (WT1) epitope 126-134 in patients with acute myeloid leukemia (AML). We recently showed that a predominant T-cell receptor (TCR) β chain was associated with vaccine-induced complete remission in an AML patient (patient 1). In this study, we address the question of whether this predominant clone or the accompanying Vβ11 restriction could be found in other AML patients vaccinated with the same WT1 peptide. For assessment of Vβ usage, cytotoxic T lymphocytes (CTLs) from four vaccinated patients were divided into specific and non-specific by epitope-specific enrichment. Vβ families were quantified in both fractions using reverse transcribed quantitative PCR. Vβ11-positive 'complementary determining region 3' (CDR3) sequences were amplified from these samples, from bone marrow samples of 17 other vaccination patients, and from peripheral blood of six healthy controls, cloned and sequenced. We observed a clear bias towards Vβ11 usage of the WT1-specific CTL populations in all four patients. The predominant CDR3β amino acid (AA) sequence of patient 1 was detected in two other patients. CDR3β loops with closely related AA sequences were only found in patient 1. There were no CDR3β AA sequences with side chains of identical chemical properties detected in any patient. We provide the first data addressing TCR Vβ chain usage in WT1-specific T-cell populations after HLA A*0201-restricted single peptide vaccination. We demonstrate both shared Vβ restriction and the sharing of a TCR β transcript with proven clinical impact in one patient.

  15. "Wilms Tumor Protein 1" (WT1) peptide vaccination-induced complete remission in a patient with acute myeloid leukemia is accompanied by the emergence of a predominant T-cell clone both in blood and bone marrow. (United States)

    Ochsenreither, Sebastian; Fusi, Alberto; Busse, Antonia; Bauer, Sandra; Scheibenbogen, Carmen; Stather, David; Thiel, Eckhard; Keilholz, Ulrich; Letsch, Anne


    Within the last few years, the first peptide vaccination trials for treatment of acute myeloid leukemia (AML) have been initiated. Athough the presence of epitope-specific T cells could be seen both in bone marrow (BM) and peripheral blood (PB), nothing is known about their clonal composition. In this study, we analyzed material from a patient with recurrent AML vaccinated with "Wilms Tumor Protein 1" (WT1) peptide, who achieved a complete remission (CR) lasting for 12 months. For identification of expanded WT1-specific T-cell clones, enrichment by tetramer and IFNγ secretion were followed by comparative quantitative reverse transcribed PCR (qRT PCR) quantification of all TCR Vβ-families. Vβ-families with increase in the enriched fraction were cloned and sequenced. A predominant clone was quantified by clonotypic qRT PCR from PB and BM. Quantity and functionality of WT1-specific cells were assessed by tetramer analyses and intracellular IFNγ staining. A specific predominant clone was identified during clinical remission. Clone-specific qRT PCR showed an increase both in PB and BM after 8 vaccinations. Six months after achieving CR, the transcript levels in BM decreased. Relapse was accompanied by secondary rise of the WT1-specific clone in PB but not in BM. In parallel, a lack of vaccine-induced WT1 specific IFNγ production was observed at that timepoint. In conclusion, we provide first data regarding evolution and compartmentalization of a peptide vaccine-induced T-cell clone in PB and BM of an AML patient. At the time of relapse, the same clone reappeared spontaneously in PB but not in BM showing impaired functionality.

  16. 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)


    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.

  17. What Happens After Treatment for Wilms Tumor? (United States)

    ... and tests depends on the initial stage and histology (favorable or unfavorable) of the cancer, the type ... Our Volunteers More ACS Sites Bookstore Shop Cancer Atlas Press Room Cancer Statistics Center Volunteer Learning Center ...

  18. Clinical significance of Wilm tumor gene(WT1) expression in breast cancer%WT1基因在乳腺癌组织中表达的研究

    Institute of Scientific and Technical Information of China (English)

    贺白; 顾伟英; 朱江; 罗光华; 张磊; 赵春英


    目的 探讨乳腺癌组织中WT1基因表达水平及其临床意义.方法 采用实时定量RT-PCR方法,检测110例乳腺肿瘤及其邻近正常乳腺组织中的WT1基因及内参照GAPDH的表达水平,以WT1_N=(WT1拷贝数/GAPDH拷贝数)×10~4计算WT1表达水平,并计算同一患者肿瘤组织与正常组织中WT1_N比值,定义为T/N_(WT1)比值,分析WT1基因表达(T/N_(WT1))水平与临床病理参数的关系.结果 102例乳腺癌组织中WT1_N明显高于其周围邻近正常乳腺组织中的表达(P0.05).乳腺癌组织中WT1_N及其T/N_(WT1)比值明显高于乳腺良性肿瘤组织(P<0.01),且T/N_(WT1)比值与有无淋巴结转移、乳腺癌病理类型、雌激素受体(ER)状态及孕激素受体(PR)状态无相关性.WT1基因表达水平与IL-8基因表达水平具有很好的相关性(r=0.723,P<0.01).结论 乳腺癌组织高表达WT1,乳腺癌组织中WT1表达水平可作为评价疾病进展及判断预后的指标.%Objective To investigate the clinical significance of Wilm tumor gene (WT1) expression in breast cancer. Methods Real-time quantitative reverse transcriptase polymerase chain reaction (RQ-RT-PCR) method was established for detecting WT1 and GAPDH mRNA expression levels in 110 cases of various breast tumor and the corresponding adjacent normal breast tissue. Normalized WT1 expression level (WT1_N)was determined as a ratio between WT1 and GAPDH for each case. The tumor tissue WT1_N over the normaltissue WT1_N of the same case was calculated as T/N_(WT1) ratio, and T/N_(WT1) value was analyzed with the clinicalpathological parameters. Results The WT1_N expression levels of the 102 breast cancer tissues were significantly higher than those of the adjacent normal breast tissues, with the median WT1_N of 2.38 (ranged from 0.12 to 112.3) and 0.81 (ranged from 0.03 to 11.65) for each (P <0.01), but there were no statistical differences between the WT1_N of 8 benign breast tumors and the nearing normal tissues, with the

  19. General Information about Wilms Tumor and Other Childhood Kidney Tumors (United States)

    ... abdomen. Blood in the urine. High blood pressure (headache, feeling very tired, chest pain, or trouble seeing ... Cancer Late Effects of Treatment for Childhood Cancer Adolescents and Young Adults with Cancer Children with Cancer: ...

  20. Treatment Option Overview (Wilms Tumor and Other Childhood Kidney Tumors) (United States)

    ... abdomen. Blood in the urine. High blood pressure (headache, feeling very tired, chest pain, or trouble seeing ... Cancer Late Effects of Treatment for Childhood Cancer Adolescents and Young Adults with Cancer Children with Cancer: ...

  1. Kidney Tumors | Office of Cancer Genomics (United States)

    Pediatric kidney tumors fall into four primary categories: Wilms tumors (~85% of all cases), clear cell sarcomas of the kidney (~5%), congenital mesoblastic nephromas (~4%), and rhabdoid tumors of the kidney (~3%). The TARGET initiative is investigating three of these tumor types.

  2. Wilms' tumour gene 1 (WT1) in human neoplasia.

    NARCIS (Netherlands)

    Keilholz, U.; Menssen, H.D.; Gaiger, A.; Menke, A.; Oji, Y.; Oka, Y.; Scheibenbogen, C.; Stauss, H.; Thiel, E. van; Sugiyama, H.


    The transcription factor Wilms' tumour gene 1 (WT1) is important as a prognostic marker as well as in the detection and monitoring of minimal residual disease in leukaemia and myelodysplastic syndromes. Evidence has accumulated over the past decade to show that WT1 is a key molecule for tumour proli

  3. Lin28 sustains early renal progenitors and induces Wilms tumor

    National Research Council Canada - National Science Library

    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


    .... 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...

  4. Radiofrequency ablation in the treatment of liver tumors in children

    NARCIS (Netherlands)

    van Laarhoven, Stijn; van Baren, Robertine; Tamminga, Rienk Yde Johan; de Jong, Koert Pieter

    Hepatoblastoma and liver metastasis of Wilms' tumors are rare hepatic tumors in children. Treatment of both tumors consists of a combination of chemotherapy and liver surgery. Radiofrequency ablation (RFA) is frequently used for the treatment of adult liver tumors but is rarely mentioned as a

  5. MLLT1 YEATS domain mutations in clinically distinctive Favourable Histology Wilms tumours | Office of Cancer Genomics (United States)

    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.

  6. Tumorer

    DEFF Research Database (Denmark)

    Prause, J.U.; Heegaard, S.


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

  7. 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)


    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

  8. Cixutumumab in Treating Patients With Relapsed or Refractory Solid Tumors (United States)


    Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Childhood Hepatoblastoma; Childhood Synovial Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Recurrent Adrenocortical Carcinoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Liver Cancer; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive; Neuroectodermal Tumor; Recurrent Neuroblastoma; Recurrent Osteosarcoma; Recurrent Retinoblastoma; Recurrent Wilms Tumor and Other Childhood Kidney Tumors

  9. 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)


    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

  10. Malnourished Malawian patients presenting with large Wilms tumours have a decreased vincristine clearance rate

    NARCIS (Netherlands)

    T. Israels; C.W.N. Damen; M. Cole; N. van Geloven; A.V. Boddy; H.N. Caron; J.H. Beijnen; E.M. Molyneux; G.J. Veal


    Introduction: In developing countries, patients with a Wilms' tumour often present late with a high degree of malnutrition and large tumours. We investigated whether this affects vincristine pharmacokinetics. Methods: Patients newly diagnosed with Wilms' tumour in Malawi and the UK were included. We

  11. 乳腺黏液癌神经内分泌分化和肾母细胞瘤蛋白1表达特征及其意义%Neuroendocrine differentiation and Wilmstumor protein-1 expression in breast mucinous carcinoma and their significance

    Institute of Scientific and Technical Information of China (English)

    王教辰; 翁寿向; 金晓芬; 余文杰; 周涛; 甘梅富


    Objective: To investigate neuroendocrine differentiation and Wilmstumor protein-1 ( WT-1 ) expression in breast mucinous carcinoma and their clinicopathological significance.Methods: The clinicopathological data of 65 patients with breast mucinous carcinoma, including 31 cases of mixed mucinous carcinoma, 23 cases of hypocellular pure mucinous carcinoma and 11 cases of hypercellular pure mucinous carcinoma, admitted in Taizhou Hospital from January 2010 to June 2015 were retrospectively reviewed.The expression of neuroendocrine markers and WT-1 was detected by immunohistochemistry staining in all cases. Re sults: The mixed mucinous carcinomas and hypercelluar pure mucinous carcinomas had higher incidence of axillary lymph node metastasis and human epidermal recepter 2 ( HER-2 ) positive than hypocellular pure mucinous carcinoma (all P0.05).The expression of neuroendocrine marker was stronger in hypercellular mucinous carcinoma than that in mixed mucinous carcinoma and hypocellular mucinous carcinoma(all P0.05 ) .The expression of WT-1 was weaker in mixed mucinous carcinoma than that in hypercellular and hypocellular pure mucinous carcinoma( all P0.05).The mucinous carcinomas with lymph node metastasis had lower expression of neuroendocrine markers than those without lymph node metastasis ( P0.05 ) . Conclus ion:Hypercellular pure mucinous breast carcinoma has higher rates of lymph node metastasis and HER-2 amplification than hypocellular pure mucinous carcinoma, the sub-classification of breast pure mucinous carcinoma should be considered . Neuroendocrine differentiation and WT-1 expression may be helpful in distinguishing the subtypes of breast mucinous carcinoma.Breast mucinous carcinoma with neuroendocrine differentiation trends to have less lymph node metastasis.%目的:分析乳腺黏液癌的临床病理特征并探讨其神经内分泌分化及肾母细胞瘤蛋白1( WT-1)表达的意义。方法:收集并分析浙江省台州医院2010年1

  12. National Academy of Clinical Biochemistry Laboratory Medicine Practice Guidelines for use of tumor markers in clinical practice

    DEFF Research Database (Denmark)

    Sturgeon, Catharine M; Hoffman, Barry R; Chan, Daniel W


    BACKGROUND: This report presents updated National Academy of Clinical Biochemistry Laboratory Medicine Practice Guidelines summarizing quality requirements for the use of tumor markers. METHODS: One subcommittee developed guidelines for analytical quality relevant to serum and tissue-based tumor...

  13. Prognostic Markers in Nephroblastoma Markers in Nephroblastoma (Wilms’ Tumor)

    NARCIS (Netherlands)

    M.A.I. Ghanem (Mazen)


    markdownabstract__Abstract__ Rance, in 1814, apparently was the first to recognize renal tumors of infancy, called nephroblastoma, as a separate entity; Max Wilms, in 1899, further characterized this tumor composed of primitive cells and structures that has become associated with his name.

  14. MLLT1 YEATS domain mutations in clinically distinctive Favourable Histology Wilms tumours

    KAUST Repository

    Perlman, Elizabeth J.


    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.

  15. [Primary Spinal Tumor Registry at the National Centre for Spinal Disorders]. (United States)

    Szövérfi, Zsolt; Lazáry, Aron; Varga, Péter Pál


    Primary spinal tumors are rare diseases. Primary spinal tumor registry would be useful to help decision making in this complex field of spine surgery. In this article the authors present the latest findings from the Primary Spinal Tumor Registry at the National Centre for Spinal Disorders, Hungary. The registry is based on a novel database management software, the REDCap electronic data capture system. It contains data of 323 patients treated surgically during an 18-year period. Among the 126 malignant tumors, the most frequent was chordoma (61 cases). In the case of benign tumors schwannoma showed the largest prevalence (45 cases). The authors conclude that due to the rarity of the disease and the complexity of the management, multicenter, prospective registries are required to provide high level of evidence. The structure of the Primary Spinal Tumor Registry in the National Centre for Spinal Disorders in Hungary is optimal for user-friendly, fast and secure data collection providing a prospective database for scientific researches and clinical follow-up.

  16. Evidence for susceptibility genes to familial Wilms tumour in addition to WT1, FWT1 and FWT2 (United States)

    Rapley, E A; Barfoot, R; Bonaïti-Pellié, C; Chompret, A; Foulkes, W; Perusinghe, N; Reeve, A; Royer-Pokora, B; Schumacher, V; Shelling, A; Skeen, J; Tourreil, S de; Weirich, A; Pritchard-Jones, K; Stratton, M R; Rahman, N


    Three loci have been implicated in familial Wilms tumour: WT1 located on chromosome 11p13, FWT1 on 17q12-q21, and FWT2 on 19q13. Two out of 19 Wilms tumour families evaluated showed strong evidence against linkage at all three loci. Both of these families contained at least three cases of Wilms tumour indicating that they were highly likely to be due to genetic susceptibility and therefore that one or more additional familial Wilms tumour susceptibility genes remain to be found. © 2000 Cancer Research Campaign PMID:10901367

  17. Pediatric liver tumors--a pictorial review. (United States)

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


    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.

  18. Pediatric liver tumors - a pictorial review

    Energy Technology Data Exchange (ETDEWEB)

    Jha, Priyanka; Tavri, Sidhartha; Patel, Chirag; Gooding, Charles; Daldrup-Link, Heike [University of California, San Francisco, Department of Radiology, San Francisco, CA (United States); Chawla, Soni C. [Department of Radiological Sciences 2D115, Olive View-UCLA Medical Center, Sylmar, CA (United States)


    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.)

  19. Tumor induction following intraoperative radiotherapy: Late results of the National Cancer Institute canine trials

    Energy Technology Data Exchange (ETDEWEB)

    Barnes, M.; Duray, P.; DeLuca, A.; Anderson, W.; Sindelar, W.; Kinsella, T. (Fox Chase Cancer Center, Philadelphia, PA (USA))


    Intraoperative radiotherapy has been employed in human cancer research for over a decade. Since 1979, trials to assess the acute and late toxicity of IORT have been carried out at the National Cancer Institute in an adult dog model in an attempt to establish dose tolerance guidelines for a variety of organs. Of the 170 animals entered on 12 studies with a minimum follow-up of 2 years, 148 dogs received IORT; 22 control animals received only surgery. Animals were sacrificed at designated intervals following IORT, usually at 1, 6, 12, 24, and 60 month intervals. 102 of 148 irradiated dogs were sacrificed less than 24 months; 46 dogs were followed greater than or equal to 24 months after IORT. To date, 34 of the 46 animals have been sacrificed; the 12 remaining animals are to be followed to 5 years. These 12 animals have minimum follow-up of 30 months. In the irradiated group followed for greater than or equal to 24 months, 10 tumors have arisen in 9 animals. One animal developed an incidental spontaneous breast carcinoma outside the IORT port, discovered only at scheduled post-mortem exam. The remaining nine tumors arose within IORT ports. Two tumors were benign neural tumors--a neuroma and a neurofibroma. One animal had a collision tumor comprised of grade I chondrosarcoma adjacent to grade III osteosarcoma arising in lumbar vertebrae. Two other grade III osteosarcomas, one grade III fibrosarcoma, and one grade III malignant fibrous histiocytoma arose in retroperitoneal/paravertebral sites. An embryonal rhabdomyosarcoma (sarcoma botryoides) arose within the irradiated urinary bladder of one animal. No sham irradiated controls nor IORT animals sacrificed less than 24 months have developed any spontaneous or radiation-induced tumors. The time range of diagnoses of tumors was 24-58 months. The IORT dose range associated with tumor development was 20-35 Gy.

  20. Tumor

    Institute of Scientific and Technical Information of China (English)


    2008479 Preliminary study of MR elastography in brain tumors. XU Lei(徐磊), et al.Neurosci Imaging Center, Beijing Tiantan Hosp, Capital Med Univ, Beijing 100050.Chin J Radiol 2008;42(6):605-608. Objective To investigate the potential values of magnetic resonance elastography (MRE) for evaluating the brain tumor consistency in vivo. Methods Fourteen patients with known solid brain tumor (5 male, 9 female; age range: 16-63 years)

  1. Biallelic DICER1 mutations occur in Wilms tumours. (United States)

    Wu, M K; Sabbaghian, N; Xu, B; Addidou-Kalucki, S; Bernard, C; Zou, D; Reeve, A E; Eccles, M R; Cole, C; Choong, C S; Charles, A; Tan, T Y; Iglesias, D M; Goodyer, P R; Foulkes, W D


    DICER1 is an endoribonuclease central to the generation of microRNAs (miRNAs) and short interfering RNAs (siRNAs). Germline mutations in DICER1 have been associated with a pleiotropic tumour predisposition syndrome and Wilms tumour (WT) is a rare manifestation of this syndrome. Three WTs, each in a child with a deleterious germline DICER1 mutation, were screened for somatic DICER1 mutations and were found to bear specific mutations in either the RNase IIIa (n = 1) or the RNase IIIb domain (n = 2). In the two latter cases, we demonstrate that the germline and somatic DICER1 mutations were in trans, suggesting that the two-hit hypothesis of tumour formation applies for these examples of WT. Among 191 apparently sporadic WTs, we identified five different missense or deletion somatic DICER1 mutations (2.6%) in four individual WTs; one tumour had two very likely deleterious somatic mutations in trans in the RNase IIIb domain (c.5438A>G and c.5452G>A). In vitro studies of two somatic single-base substitutions (c.5429A>G and c.5438A>G) demonstrated exon 25 skipping from the transcript, a phenomenon not previously reported in DICER1. Further we show that DICER1 transcripts lacking exon 25 can be translated in vitro. This study has demonstrated that a subset of WTs exhibits two 'hits' in DICER1, suggesting that these mutations could be key events in the pathogenesis of these tumours.

  2. A Survey of Mesenchyme-related Tumors of the Rat Kidney in the National Toxicology Program Archives, with Particular Reference to Renal Mesenchymal Tumor. (United States)

    Hard, Gordon C; Seely, John Curtis; Betz, Laura J


    In order to harmonize diagnostic terminology, confirm diagnostic criteria, and describe aspects of tumor biology characteristic of different tumor types, a total of 165 cases of mesenchyme-related tumors and nephroblastomas of the rat kidney were reexamined from the National Toxicology Program (NTP) Archives. This survey demonstrated that renal mesenchymal tumor (RMT) was the most common spontaneous nonepithelial tumor in the rat kidney, also occurring more frequently in the NTP studies than nephroblastoma. Renal sarcoma was a distinct but very rare tumor entity, representing a malignant, monomorphous population of densely crowded, fibroblast-like cells, in which, unlike RMT, preexisting tubules did not persist. Nephroblastoma was characterized by early death of affected animals, suggesting an embryonal origin for this tumor type. Male and female rats were equally disposed to developing RMT, but most of the cases of nephroblastoma occurred in female rats and liposarcoma occurred mostly in male rats. This survey confirmed discrete histopathological and biological differences between the mesenchyme-related renal tumor types and between RMT and nephroblastoma. Statistical analysis also demonstrated a lack of any relationship of these renal tumor types to test article administration in the NTP data bank.

  3. The spiritual quest in “Los tres cantos” by Teresa Wilms Montt

    Directory of Open Access Journals (Sweden)

    Gabriela Jerez Garcés


    Full Text Available Analysis of the text “Los tres cantos” (1917 of the chilean writer Teresa Wilms Montt, understanding it as an exercise of spiritual search from the interior of the language, in which the poetry recovers her possibilities of extension of the spiritual conscience. The text is framed at the beginning of the vanguard will, which starts germinating in Chile about 1910, year in which there appears a trend named spiritualism of vanguard, aesthetic current based on the idea of that the spiritual life and experience of the soul constitute the axis of the human transcendence.Key words: Chilean Poetry, Teresa Wilms, spiritualism, vanguard.

  4. Treatments and outcomes of peritoneal surface tumors through a centralized national service (United kingdom). (United States)

    Rout, S; Renehan, A G; Parkinson, M F; Saunders, M P; Fulford, P E; Wilson, M S; O'Dwyer, S T


    Treatment of peritoneal surface malignancies with combined cytoreductive surgery and heated intraperitoneal chemotherapy may improve oncologic outcome. To better define treatment pathways, five-year results in patients referred to one of two centralized national treatment centers in the United Kingdom were analyzed. A prospective database of patients referred to the Manchester Peritoneal Tumor Service, established in 2002, was analyzed. Outcomes were evaluated using Kaplan-Meier life tables and Cox models. Two hundred seventy-eight patients (median age, 56.9 (range, 16-86) years) were considered by a dedicated multidisciplinary team and tracked on seven clinical pathways. Among the 118 surgically treated, the most common diagnosis was pseudomyxoma peritonei (101 patients, 86%). Major complications occurred in 11 patients (9%); there was no 30-day mortality. Where complete cytoreduction was achieved, three-year and five-year tumor-related survival rates were 94% and 86%, respectively. In the Cox model, incompleteness of cytoreduction (P = 0.001) and high-grade tumor (P < 0.0001) were independent prognosticators of poor outcome. The establishment of a national treatment center has allowed refinement of techniques to achieve internationally recognized results. Having achieved low levels of morbidity and mortality in the treatment of mainly pseudomyxoma peritonei of appendiceal origin, the technique of cytoreductive surgery and heated intraperitoneal chemotherapy may be considered for peritoneal carcinomatosis of colorectal origin.

  5. Malignant renal tumors in children

    Directory of Open Access Journals (Sweden)

    Justin Scott Lee


    Full Text Available Renal malignancies are common in children. While the majority of malignant renal masses are secondary to Wilms tumor, it can be challenging to distinguish from more aggressive renal masses. For suspicious renal lesions, it is crucial to ensure prompt diagnosis in order to select the appropriate surgical procedure and treatment. This review article will discuss the common differential diagnosis that can be encountered when evaluating a suspicious renal mass in the pediatric population. This includes clear cell sarcoma of the kidney, malignant rhabdoid tumor, renal medullary carcinoma and lymphoma. 

  6. Final height and IGF1 in adult survivors of Wilms tumour

    NARCIS (Netherlands)

    K. Blijdorp (Karin); M.M. van den Heuvel-Eibrink (Marry); R. Pieters (Rob); S. Pluijm (Saskia); A. Wagner (Anja); H. Segers (Heidi); A-J. van der Lely (Aart-Jan); S.J.C.M.M. Neggers (Bas)


    textabstractObjective: One-sided nephrectomy is followed by increased levels of IGF1, associated with linear growth during childhood. The aim was to evaluate final height and IGF1 levels in nephrectomized Wilms tumour survivors when compared with healthy Dutch references and survivors of other cance

  7. Renal function after unilateral nephrectomy for Wilms' tumour : The influence of radiation therapy

    NARCIS (Netherlands)

    deGraaf, SSN; vanGent, H; ReitsmaBierens, WCC; vanLuyk, WHJ; Dolsma, WV; Postma, A


    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 I-125-iothalamate clearance and

  8. Clinical significance of serum biomarkers in pediatric solid mediastinal and abdominal tumors. (United States)

    Sandoval, John A; Malkas, Linda H; Hickey, Robert J


    Childhood cancer is the leading cause of death by disease among U.S. children between infancy and age 15. Despite successes in treating solid tumors such as Wilms tumor, disappointments in the outcomes of high-risk solid tumors like neuroblastoma have precipitated efforts towards the early and accurate detection of these malignancies. This review summarizes available solid tumor serum biomarkers with a special focus on mediastinal and abdominal cancers in children.

  9. Clinical Significance of Serum Biomarkers in Pediatric Solid Mediastinal and Abdominal Tumors

    Directory of Open Access Journals (Sweden)

    Robert J. Hickey


    Full Text Available Childhood cancer is the leading cause of death by disease among U.S. children between infancy and age 15. Despite successes in treating solid tumors such as Wilms tumor, disappointments in the outcomes of high-risk solid tumors like neuroblastoma have precipitated efforts towards the early and accurate detection of these malignancies. This review summarizes available solid tumor serum biomarkers with a special focus on mediastinal and abdominal cancers in children.

  10. Tumor size predicts long-term survival in colon cancer: an analysis of the National Cancer Data Base. (United States)

    Saha, Sukamal; Shaik, Mohammed; Johnston, Gregory; Saha, Supriya Kumar; Berbiglia, Lindsay; Hicks, Micheal; Gernand, Jill; Grewal, Sandeep; Arora, Madan; Wiese, David


    American Joint Committee on Cancer uses tumor size for "T" staging of many solid tumors for its effect on prognosis. However, tumor size has not been incorporated in tumor (T), nodal status (N), metastasis (M) staging for colon cancer. Hence, the National Cancer Data Base was used to determine whether tumor size correlates with TNM staging and survival. For the 300,386 patients, tumor size was divided into S1 (0 to 2 cm), S2 (>2 to 4 cm), S3 (>4 to 6 cm), and S4 (>6 cm). Statistical comparison was done for TNM stage, grade, and nodal status with tumor size. Kaplan-Meier survival analysis was done for each "S" stage. Of the 300,386 patients, 13% were classified as S1, 39% S2, 30% S3 and 18% as S4. Right colon was the most common site (48%). Tumor size positively correlated with grade, T stage, and nodal stage. Tumor size was inversely associated with survival. Tumor size is positively correlated with important prognostic factors and negatively impacted survival. Copyright © 2015 Elsevier Inc. All rights reserved.

  11. Pituitary Tumors (United States)

    ... institutes of the National Institutes of Health (NIH) conduct research related to brain tumors, including pituitary tumors, in their laboratories at ... institutes of the National Institutes of Health (NIH) conduct research related to brain tumors, including pituitary tumors, in their laboratories at ...

  12. WNT5A Is Regulated by PAX2 and May Be Involved in Blastemal Predominant Wilms Tumorigenesis

    Directory of Open Access Journals (Sweden)

    Yahya Tamimi


    Full Text Available The PAX2 gene encodes a transcription factor expressed during development. In humans, PAX2 mutations cause the renal-coloboma syndrome, whereas homozygous mutations are lethal, causing severe organ malformation, notably in the brain and kidney. Wilms tumor (WT of the kidney results from a failure in the mesenchymal-epithelial transition, a crucial step partly controlled by PAX2. Downstream target genes regulated by PAX2 are still undefined. We therefore hypothesized that identification and characterization of the genes regulated by PAX2 may improve our understanding of developmentally related malignancies including WT. We used nickel agarose chromatin enrichment, chromatin immunoprecipitation, and the human embryonic kidney-derived cell line HEK293 to identify regulatory elements responding to PAX2. Among others, we identified WNT5A as a gene potentially regulated by PAX2. Here, we demonstrate that WNT5A is a direct target of PAX2 in HEK293 cells, using both transactivation and electrophoretic mobility shift assays. We were unable to find any WNT5A disease-associated mutations after screening a panel of 99 WT samples. However, quantitative reverse transcription-polymerase chain reaction in human favorable-histology WT revealed that ∼66% of the cases expressed significantly less WNT5A than human fetal kidney. Moreover, the WiT9 WT cell line revealed a weak expression of the WNT5A gene. A correlation of decreased WNT5A expression with predominant blastemal histology tumors suggests a possible inhibitory role in WT pathogenesis. This study underlines the importance of PAX2 in the regulation of WNT5A. Further in vivo study is necessary to determine whether the PAX2 and WNT5A are truly involved in WT pathogenesis.

  13. [Leather dust and systematic research on occupational tumors: the national and regional registry TUNS]. (United States)

    Mensi, Carolina; Sieno, Claudia; Consonni, Dario; Riboldi, Luciano


    The sinonasal cancer (SNC) are a rare tumors characterized by high occupational etiologic fraction. For this reason their incidence and etiology can be actively monitored by a dedicated cancer registry. The National Registry of these tumours is situated at the Italian Institute for Occupational Safety and Prevention (ISPESL) and is based on Regional Operating Centres (ROCs). In Lombardy Region the ROC has been established at the end of 2007 with the purpose to make a systematic surveillance and therefore to support in the most suitable way the scientific research and the prevention actions in the high risk working sectors. The aims of this surveillance are: to estimate the regional incidence of SNC, to define different sources of occupational and environmental exposure both known (wood, leather, nickel, chromium) and unknown. The registry collects all the new incident cases of epithelial SNC occurring in residents in Lombardy Region since 01.01.2008. The regional Registry is managed according to National Guidelines. Until January 2010 we received 596 cases of suspected SNC; only 91 (15%) of these were actually incident cases according to the inclusion criteria of the Registry, and they were preferentially adenocarcinoma and squamous carcinoma. In 2008 the regional age-standardized incidence rate of SNC for males and females, respectively, is 0.8 and 0.5 per 100,000. Occupational or environmental exposure to wood or leather dust is ascertained in over the 50% of cases. The occupational exposure to leather dust was duo to work in shoe factories. Our preliminary findings confirm that occupational exposure to wood and leather dusts are the more relevant risk factors for SNC. The study of occupational sectors and job activity in cases without such exposure could suggest new etiologic hypothesis.

  14. Flavopiridol in Treating Children With Relapsed or Refractory Solid Tumors or Lymphomas (United States)


    Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Liver Cancer; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Malignant Germ Cell Tumor; Recurrent Childhood Medulloblastoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Childhood Visual Pathway and Hypothalamic Glioma; Recurrent Childhood Visual Pathway Glioma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Neuroblastoma; Recurrent Osteosarcoma; Recurrent Retinoblastoma; Recurrent Wilms Tumor and Other Childhood Kidney Tumors; Recurrent/Refractory Childhood Hodgkin Lymphoma; Unspecified Childhood Solid Tumor, Protocol Specific

  15. Isolation of Breast Tumor Suppressor Genes from Chromosome 11p (United States)


    at chromosome crucial role in urogenital development (Pelletier et al., llp15 has also been described in Wilms tumors but thus 1991). However...Atkins L and Riccardi VM. (1979). Nowak NJ, Evans G, Stanbridge EJ, de Jong P, Shows TB , Cytogenet. Cell Genet., 24, 185-192. Weissman BE and Higgins MJ...Singh-Kahlon P, Weksberg R, Squire J, Grundy P, Coppes MJ and Haber D. (1995). Hematology/ Shows TB and Higgins MJ. (1994). Genes Chromosomes Oncology

  16. The Impact of Ethnicity on Wilms Tumor: Characteristics and Outcome of a South African Cohort

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    D. K. Stones


    Full Text Available Background. Nephroblastoma is the commonest renal tumour seen in children. It has a good prognosis in developed countries with survival rates estimated to be between 80% and 90%, while in Africa it remains low. Method. Retrospective study of patients diagnosed with nephroblastoma who are seen at 4 paediatric oncology units, representing 58.5% of all South African children with nephroblastoma and treated following SIOP protocol between January 2000 and December 2010. Results. A total of 416 patients were seen at the 4 units. Over 80% of our patients were African and almost 10% of mixed ethnicity. The most common stage was stage 4. The median survival was 28 months after diagnosis with the mixed ethnicity patients recording the longest duration (39 months and the white patients had the shortest median survival. The overall 5-year survival rate was estimated to be 66%. Stage 2 patients did significantly better (85%. Conclusions. Our patients are similar with regard to gender ratio, median age, and age distribution as described in the literature, but in South Africa the more advanced stage disease seen than in other developed countries is translated into low overall survival rate.

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

    DEFF Research Database (Denmark)

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


    BASP1 expression augments transcriptional activation by WT1. BASP1 is present in the developing nephron structures of the embryonic kidney and, coincident with that of WT1, its expression is restricted to the highly specialized podocyte cells of the adult kidney. Taken together, our results show...

  18. Busulfan, Melphalan, Topotecan Hydrochloride, and a Stem Cell Transplant in Treating Patients With Newly Diagnosed or Relapsed Solid Tumor (United States)


    Solid Tumor; Adult Central Nervous System Germ Cell Tumor; Adult Rhabdomyosarcoma; Childhood Central Nervous System Germ Cell Tumor; Childhood Soft Tissue Sarcoma; Ewing Sarcoma; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Ovarian Mixed Germ Cell Tumor; Previously Untreated Childhood Rhabdomyosarcoma; Recurrent Adult Brain Tumor; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Malignant Germ Cell Tumor; Recurrent Childhood Medulloblastoma; Recurrent Childhood Pineoblastoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Childhood Visual Pathway and Hypothalamic Glioma; Recurrent Childhood Visual Pathway Glioma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Extragonadal Germ Cell Tumor; Recurrent Extragonadal Non-seminomatous Germ Cell Tumor; Recurrent Malignant Testicular Germ Cell Tumor; Recurrent Neuroblastoma; Recurrent Ovarian Germ Cell Tumor; Recurrent Wilms Tumor and Other Childhood Kidney Tumors; Unspecified Adult Solid Tumor, Protocol Specific; Unspecified Childhood Solid Tumor, Protocol Specific

  19. Evaluation of nephron sparing surgery for renal tumors: a single institution experience%肾脏肿瘤保留肾单位手术的评价:单一机构的经验

    Institute of Scientific and Technical Information of China (English)

    Hytham Abd-elkareem Ahmed; Abdelhamid Hussein Ezzat; Ismael Mourad


    Objective: The aim of the study was to report the experience of National Cancer Institute (NCI), Cairo University, Egypt, in managing various benign and malignant renal tumors with nephron sparing surgery (NSS), and to assess its safety and feasibility. Methods: Reviewing the literature for NSS, and records of patients who underwent NSS in the period from January 2000 to December 2009 at National Cancer Institute, Cairo University regarding the patient and tumor related characteristics, the indication for NSS, operative technique, postoperative complications, full histopathological data, and follow up results. Results: The total number of patients was 17. Mean age at surgery was 30.7 years (range 1.5-65 years). Five patients had bilateral tumors during surgery. The mean tumor size was 4.5 cm (range 1-9 cm). All patients had normal preoperative kidney functions. Seven patients had an absolute indication for NSS, 6 patients had a relative indication, and 4 patients had an elective indication. All the 5 patients with bilateral tumors underwent bilateral simultaneous surgery. Cold ischemia was used in 8 patients, 1 patient was exposed to warm ischemia, manual compression was used in 2 patients, and no vascular control was applied in 6 patients. Complications were encountered in 2 patients, one of them had urinary leakage which needed reoperation, and the other had subcutaneous hematoma which was treated conservatively. Histopathological analysis revealed Wilm's tumor (8 patients), angiomyolipoma (4 patients), renal cell carcinoma (4 patients), and hydatid cyst (1 patient). All patients had negative surgical margin. For patients with Wilm's tumor, the mean follow up was 21.4 months (range 0-94 months), 2 patients had local recurrence, and 1 patient had distant metastasis. For patients with RCC, the mean follow up was 15.3 months (5-33 months), no patients had local recurrence or distant metastasis. All patients had normal kidney functions during postoperative and follow

  20. The National Tumor Association Foundation (ANT: A 30 year old model of home palliative care

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    Bonazzi Valeria


    Full Text Available Abstract Background Models of palliative care delivery develop within a social, cultural, and political context. This paper describes the 30-year history of the National Tumor Association (ANT, a palliative care organization founded in the Italian province of Bologna, focusing on this model of home care for palliative cancer patients and on its evaluation. Methods Data were collected from the 1986-2008 ANT archives and documents from the Emilia-Romagna Region Health Department, Italy. Outcomes of interest were changed in: number of patients served, performance status at admission (Karnofsky Performance Status score [KPS], length of participation in the program (days of care provided, place of death (home vs. hospital/hospice, and satisfaction with care. Statistical methods included linear and quadratic regressions. A linear and a quadratic regressions were generated; the independent variable was the year, while the dependent one was the number of patients from 1986 to 2008. Two linear regressions were generated for patients died at home and in the hospital, respectively. For each regression, the R square, the unstandardized and standardized coefficients and related P-values were estimated. Results The number of patients served by ANT has increased continuously from 131 (1986 to a cumulative total of 69,336 patients (2008, at a steady rate of approximately 121 additional patients per year and with no significant gender difference. The annual number of home visits increased from 6,357 (1985 to 904,782 (2008. More ANT patients died at home than in hospice or hospital; this proportion increased from 60% (1987 to 80% (2007. The rate of growth in the number of patients dying in hospital/hospice was approximately 40 patients/year (p 40 increased. Mean days of care for patients with KPS > 40 exceeded mean days for patients with KPS Conclusions The ANT home care model of palliative care delivery has been well-received, with progressively growing numbers

  1. 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)


    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.)

  2. National Academy of Clinical Biochemistry laboratory medicine practice guidelines for use of tumor markers in testicular, prostate, colorectal, breast, and ovarian cancers

    DEFF Research Database (Denmark)

    Sturgeon, Catharine M; Duffy, Michael J; Stenman, Ulf-Håkan


    BACKGROUND: Updated National Academy of Clinical Biochemistry (NACB) Laboratory Medicine Practice Guidelines for the use of tumor markers in the clinic have been developed. METHODS: Published reports relevant to use of tumor markers for 5 cancer sites--testicular, prostate, colorectal, breast...

  3. Epidemiological study on giant cell tumor recurrence at the Brazilian National Institute of Traumatology and Orthopedics

    Directory of Open Access Journals (Sweden)

    Roberto Clayton Lima Oliveira Júnior


    Full Text Available ABSTRACT OBJECTIVE: Giant cell tumors are benign bone neoplasms that are relatively rare in adults and their biological behavior is still unpredictable. The incidence of local recurrence has presented variation between 0% and 65% in studies conducted worldwide, but few data are available on this complication in the Brazilian population. METHODS: Information on 155 patients with confirmed histological diagnoses of giant cell tumor who were treated in our institution's orthopedic oncology service between January 2000 and July 2014 was gathered. Demographic characteristics were evaluated and compared between patients who presented local recurrence during the clinical follow-up. RESULTS: Local recurrence was observed in 26 patients (16.7%, of whom 22 were female (84.6%. The most common site of local recurrence was the distal femur (38.4%. Eleven patients presented early recurrence, while 15 cases were diagnosed after 15 months, representing 42.3% and 57.7%, respectively. Metastases were identified in five patients (3.2%. CONCLUSION: Tumor-related factors did not show any increased incidence of local recurrence of giant cell tumors. Surgical treatment with an intralesional margin is a valid option for treating local recurrences and does not show any difference in disease-free survival in relation to other types of procedures. Clinical treatment is reserved for cases of unresectable tumors or when surgical treatment is impossible.

  4. Thirty-day readmission and reoperation after surgery for spinal tumors: a National Surgical Quality Improvement Program analysis. (United States)

    Karhade, Aditya V; Vasudeva, Viren S; Dasenbrock, Hormuzdiyar H; Lu, Yi; Gormley, William B; Groff, Michael W; Chi, John H; Smith, Timothy R


    OBJECTIVE The goal of this study was to use a large national registry to evaluate the 30-day cumulative incidence and predictors of adverse events, readmissions, and reoperations after surgery for primary and secondary spinal tumors. METHODS Data from adult patients who underwent surgery for spinal tumors (2011-2014) were extracted from the prospective National Surgical Quality Improvement Program (NSQIP) registry. Multivariable logistic regression was used to evaluate predictors of reoperation, readmission, and major complications (death, neurological, cardiopulmonary, venous thromboembolism [VTE], surgical site infection [SSI], and sepsis). Variables screened included patient age, sex, tumor location, American Society of Anesthesiologists (ASA) physical classification, preoperative functional status, comorbidities, preoperative laboratory values, case urgency, and operative time. Additional variables that were evaluated when analyzing readmission included complications during the surgical hospitalization, hospital length of stay (LOS), and discharge disposition. RESULTS Among the 2207 patients evaluated, 51.4% had extradural tumors, 36.4% had intradural extramedullary tumors, and 12.3% had intramedullary tumors. By spinal level, 20.7% were cervical lesions, 47.4% were thoracic lesions, 29.1% were lumbar lesions, and 2.8% were sacral lesions. Readmission occurred in 10.2% of patients at a median of 18 days (interquartile range [IQR] 12-23 days); the most common reasons for readmission were SSIs (23.7%), systemic infections (17.8%), VTE (12.7%), and CNS complications (11.9%). Predictors of readmission were comorbidities (dyspnea, hypertension, and anemia), disseminated cancer, preoperative steroid use, and an extended hospitalization. Reoperation occurred in 5.3% of patients at a median of 13 days (IQR 8-20 days) postoperatively and was associated with preoperative steroid use and ASA Class 4-5 designation. Major complications occurred in 14.4% of patients: the

  5. Deducing the stage of origin of Wilms' tumours from a developmental series of Wt1-mutant mice

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    Rachel L. Berry


    Full Text Available Wilms' tumours, paediatric kidney cancers, are the archetypal example of tumours caused through the disruption of normal development. The genetically best-defined subgroup of Wilms' tumours is the group caused by biallelic loss of the WT1 tumour suppressor gene. Here, we describe a developmental series of mouse models with conditional loss of Wt1 in different stages of nephron development before and after the mesenchymal-to-epithelial transition (MET. We demonstrate that Wt1 is essential for normal development at all kidney developmental stages under study. Comparison of genome-wide expression data from the mutant mouse models with human tumour material of mutant or wild-type WT1 datasets identified the stage of origin of human WT1-mutant tumours, and emphasizes fundamental differences between the two human tumour groups due to different developmental stages of origin.

  6. Organization of a tumor bank: the experience of the National Cancer Institute of Mexico. (United States)

    Ruíz-Godoy, L; Meneses-García, A; Suárez-Roa, L; Enriquez, V; Lechuga-Rojas, R; Reyes-Lira, E


    A tumor bank (TB) is an ordered collection of neoplastic samples, normal tissue, and/or fluids preserved under optimal conditions, as well as storing patients' clinical information. The objective of this article is to outline the planning and logistics necessary for the functioning of the Instituto Nacional de Cancerología (INCan) TB in Mexico City. For the planning and logistics of a TB, several technical, legal, medical, structural, and physical aspects were considered, which can be grouped under four headings: (1) design and structure, (2) equipping the area and informatics, (3) ethical-legal aspects, and (4) sample collection, preservation, and quality control. One crucial element of interinstitutional interest will be the transfer of these concepts to the different oncological centers, integrating in this manner a network that enables the exploration of the different pathologies from therapeutic, epidemiological, and molecular points of view. 2010 S. Karger AG, Basel.

  7. Future directions in the treatment of neuroendocrine tumors: consensus report of the National Cancer Institute Neuroendocrine Tumor clinical trials planning meeting. (United States)

    Kulke, Matthew H; Siu, Lillian L; Tepper, Joel E; Fisher, George; Jaffe, Deborah; Haller, Daniel G; Ellis, Lee M; Benedetti, Jacqueline K; Bergsland, Emily K; Hobday, Timothy J; Van Cutsem, Eric; Pingpank, James; Oberg, Kjell; Cohen, Steven J; Posner, Mitchell C; Yao, James C


    Neuroendocrine tumors (NETs) arise from a variety of anatomic sites and share the capacity for production of hormones and vasoactive peptides. Because of their perceived rarity, NETs have not historically been a focus of rigorous clinical research. However, the diagnosed incidence of NETs has been increasing, and the estimated prevalence in the United States exceeds 100,000 individuals. The recent completion of several phase III studies, including those evaluating octreotide, sunitinib, and everolimus, has demonstrated that rigorous evaluation of novel agents in this disease is both feasible and can lead to practice-changing outcomes. The NET Task Force of the National Cancer Institute GI Steering Committee convened a clinical trials planning meeting to identify key unmet needs, develop appropriate study end points, standardize clinical trial inclusion criteria, and formulate priorities for future NET studies for the US cooperative group program. Emphasis was placed on the development of well-designed clinical trials with clearly defined efficacy criteria. Key recommendations include the evaluation of pancreatic NET separately from NETs of other sites and the exclusion of patients with poorly differentiated histologies from trials focused on low-grade histologies. Studies evaluating novel agents for the control of hormonal syndromes should avoid somatostatin analog washout periods when possible and should include quality-of-life end points. Because of the observed long survival after progression of many patients, progression-free survival is recommended as a feasible and relevant primary end point for both phase III studies and phase II studies where a delay in progression is expected in the absence of radiologic responses.

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

    Directory of Open Access Journals (Sweden)

    Manjula Jain


    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.

  9. Cytomorphology and immunohistochemistry of extrarenal rhabdoid tumor: a case report with review of literature. (United States)

    Jain, Manjula; Harbhajanka, Aparna; Choudhary, S Roy


    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 eosinophilic 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 enolase NSE, 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.

  10. Differential splicing of exon 5 of the Wilms tumour (WTI) gene. (United States)

    Renshaw, J; King-Underwood, L; Pritchard-Jones, K


    The WTI gene encodes a developmentally regulated transcription factor whose function is altered by alternative splicing at two sites: the 17 amino acids of exon 5, whose functional effects are ill-defined, and the 3 amino acids (KTS) between exons 9 and 10, which determine sequence-specific DNA binding and nuclear localisation. Germline mutations, which prevent normal KTS splicing, can underlie the Denys-Drash syndrome, and disruptions of splicing of exon 5 may occur in Wilms tumours. We analysed by reverse transcriptase polymerase chain reaction (RT-PCR) amplification the relative ratios of the four splice variants of WTI mRNA in normal and tumour tissues and found tissue-specific, developmental stage-specific, and species-specific differences in the splicing of exon 5 but not of KTS. We found no evidence for disrupted splicing in acute leukaemias or gonadal tumours. The significance of these findings is discussed, and the possibility is raised that WTI may orchestrate the appropriate response to growth and differentiation factor signalling, mediated by alterations in the relative levels of exon 5 containing WTI isoforms.

  11. Studies on tumor induced angiogenesis. (United States)

    Ambrus, J L; Ambrus, C M; Forgach, P; Stadler, S; Halpern, J; Sayyid, S; Niswander, P; Toumbis, C


    Methods were developed to test angiogenic response to human tumor implants and various biologic agents in the cornea of rabbits and non-human primates (Macaca arctoides). Crude PDGF preparations were found to have significant angiogenic effect. Purified, recombinant PDGF preparations were also effective inhibitors (e.g. pentoxifylline (Px) (which also were found to release PgI2 and t-PA) inhibited human tumor implant induced angiogenesis and reduced spontaneous metastases in 3 transplantable murine tumors (Furth-Columbia Wilms' tumor in Furth-Wistar rats, C-1300 neuroblastoma in A/J mice and HM-Kim mammary carcinoma in Wistar rats) but not in the NIH adenocarcinoma in Balb/c mice. Sodium diethyldithiocarbamate (DDTC), a metal complexing agent with special affinity to copper and anti-thyroid as well as, immune stimulating activity was shown to be anti-angiogenic and to potentiate the effect of Px. The anti-fibrinolytic agents epsilon amino caproic acid (EACA) and tranaxamic acid (t-AMCHA) were anti-angiogenic. DDTC and Px were synergistic from this point of view.

  12. Oxaliplatin and Irinotecan in Treating Young Patients With Refractory Solid Tumors or Lymphomas (United States)


    Childhood Burkitt Lymphoma; Childhood Central Nervous System Germ Cell Tumor; Childhood Diffuse Large Cell Lymphoma; Childhood Grade III Lymphomatoid Granulomatosis; Childhood Immunoblastic Large Cell Lymphoma; Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Liver Cancer; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Malignant Germ Cell Tumor; Recurrent Childhood Medulloblastoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Childhood Visual Pathway Glioma; Recurrent Colon Cancer; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Melanoma; Recurrent Nasopharyngeal Cancer; Recurrent Neuroblastoma; Recurrent Osteosarcoma; Recurrent Wilms Tumor and Other Childhood Kidney Tumors; Recurrent/Refractory Childhood Hodgkin Lymphoma; Unspecified Childhood Solid Tumor, Protocol Specific

  13. Single nucleotide polymorphisms in the Wilms' tumour gene 1 in clear cell renal cell carcinoma.

    Directory of Open Access Journals (Sweden)

    Xingru Li

    Full Text Available The Wilms' tumour gene 1 (WT1 single nucleotide polymorphism (SNP rs16754 has recently been described as an independent prognostic factor in acute myeloid leukaemia (AML patients. It is of great interest to test whether WT1 SNPs can be used as a molecular marker in other cancer types in order to improve risk and treatment stratification. We performed sequencing analysis on all 10 exons of the WT1 gene in a total of 182 patients with clear cell renal cell carcinoma (ccRCC. Six different SNPs were identified, in descending order for minor allele frequency: rs2234582, rs16754, rs1799925, rs5030315, rs2234583, and rs2234581. At least one minor allele for WT1 SNP was identified in 61% of ccRCC patients. In the entire study population, only 6% carried two copies of the minor allele. The genotypes of WT1 SNPs in 78 tumour-free kidney tissue specimens were found to be in 95% concordance with corresponding tumour samples. No correlation was observed between WT1 SNP genotypes and RNA expression level. WT1 SNP genotypes did not associate with clinical and pathological characteristics. We found favourable outcomes associated with the homozygous minor allele for WT1 SNP. However, SNP genotypes did not show to be of prognostic significance when comparing wild-type versus homozygous or heterozygous for the minor allele in the entire cohort. None of the previously reported WT1 mutations in AML was found in the present study. A novel WT1 missense mutation was identified in only one patient. Our data suggest that common WT1 mutations are not involved in ccRCC. Due to too few cases harbouring the homozygous minor allele, the prognostic impact needs to be verified in larger study populations.

  14. Why should survivors of childhood renal tumor and others with only one kidney be denied the chance to play contact sports? (United States)

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


    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.

  15. A proteomic investigation of glomerular podocytes from a Denys-Drash syndrome patient with a mutation in the Wilms tumour suppressor gene WT1.

    NARCIS (Netherlands)

    Viney, R.L.; Morrison, A.A.; Heuvel, L.P.W.J. van den; Ni, L.; Mathieson, P.W.; Saleem, M.A.; Ladomery, M.R.


    Glomerular podocytes are essential for blood filtration in the kidney underpinned by their unique cytoskeletal morphology. An increasing number of kidney diseases are being associated with key podocyte abnormalities. The Wilms tumour suppressor gene (WT1) encodes a zinc finger protein with a crucial

  16. Wilms' tumor suppressor gene (WT1) suppresses apoptosis by transcriptionally downregulating BAX expression in immature rat granulosa cells

    National Research Council Canada - National Science Library

    Park, Minji; Choi, Yuri; Choi, Hyeonhae; Roh, Jaesook


    The important role of WT1 in early folliculogenesis was evident from its restricted expression pattern in immature follicles and from its involvement in transcriptional control of inhibin-α and FSH receptor...

  17. Risk of solid tumors and hematological malignancy in persons with Turner and Klinefelter syndromes: A national cohort study. (United States)

    Ji, Jianguang; Zöller, Bengt; Sundquist, Jan; Sundquist, Kristina


    The risk of solid and hematological malignancy in patients with Turner syndrome, characterized by X chromosome monosomy in women, and Klinefelter syndrome, characterized with two and more X chromosomes in men, is not well established, but such evidence may have etiological implications on cancer development. We identified a total of 1,409 women with Turner syndrome and 1,085 men with Klinefelter syndrome from the Swedish Hospital Discharge and Outpatient Register. These individuals were further linked to the Swedish Cancer Register to examine the standardized incidence ratios (SIRs) of cancer using the general population without Turner and Klinefelter syndromes as reference. The overall risk of cancer was 1.34 for women with Turner syndrome; it was increased only for solid tumors. For a specific type of tumor, the risk of melanoma and central nervous system tumor was significantly increased. For persons with Klinefelter syndrome, the risk of solid tumors was decreased (SIR = 0.66), whereas the risk of hematological malignancy was increased (SIR = 2.72). Non-Hodgkin lymphoma and leukemia showed an increased SIR of 3.02 and 3.62, respectively. Our study supported the hypothesis that X chromosome plays an important role in the etiology of solid tumors. The underlying mechanisms for the increased incidence of non-Hodgkin lymphoma and leukemia in persons with Klinefelter syndrome need to be investigated further.

  18. Nation

    DEFF Research Database (Denmark)

    Østergaard, Uffe


    Nation er et gammelt begreb, som kommer af det latinske ord for fødsel, natio. Nationalisme bygger på forestillingen om, at mennesker har én og kun én national identitet og har ret til deres egen nationalstat. Ordet og forestillingen er kun godt 200 år gammel, og i 1900-tallet har ideologien bredt...... sig over hele verden. Nationalisme er blevet global....

  19. Tumor prevalence in brown bullheads (Ameiurus nebulosus) from Darby Creek, John Heinz National Wildlife Refuge at Tinicum, Philadelphia, PA (United States)

    US Fish and Wildlife Service, Department of the Interior — The John Heinz National Wildlife Refuge at Tinicum (JHNWRT) is located along Darby Creek near its confluence with the Delaware River, south of Philadelphia,...

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

    Directory of Open Access Journals (Sweden)

    Samin Alavi


    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.

  1. Bone tumor (United States)

    Tumor - bone; Bone cancer; Primary bone tumor; Secondary bone tumor; Bone tumor - benign ... The cause of bone tumors is unknown. They often occur in areas of the bone that grow rapidly. Possible causes include: Genetic defects ...

  2. WT1 immunoprofiling and comparison of malignant Mullerian mixed tumors of the female genital tract. (United States)

    Franko, Angela; Magliocco, Anthony M; Duan, Quili; Duggan, Máire A


    A malignant Mullerian mixed tumor (MMMT) is a biphasic homologous or heterologous malignancy of the female genital tract. WT1 (Wilms tumor 1) is both a tumor suppressor gene and oncogene overexpressed in the nuclei of some gynecologic carcinomas. Expression in MMMT is incompletely described. Whole sections from 16 MMMTs were stained with WT1 (N terminus) using a standard immunoperoxidase technique. There were 7 heterologous and 9 homologous tumors and 10 were endometrial, 5 were ovarian, and 1 was of peritoneal origin. The tissue and cell staining pattern and score (intensity by amount) were evaluated and correlated with the tumor subtype and anatomic location. Among the 16 tumors, 81.3% showed mostly stromal and cytoplasmic staining and a score of 3 or 6. Staining was positive in 80% of the endometrial and ovarian tumors and the 1 peritoneal tumor and in all heterologous and 66.7% of the homologous tumors. The immunoprofile correlated with tumor subtype but not with anatomic location. Stromal and epithelial staining was more frequent (83.3%) in homologous tumors and differed significantly (P=0.009) from the heterologous types where stromal staining prevailed (85.7%). MMMT is another genital tract malignancy which can over express WT1 and the immunoprofile may assist in tumor subtyping.

  3. Newcastle disease virus selectively kills human tumor cells. (United States)

    Reichard, K W; Lorence, R M; Cascino, C J; Peeples, M E; Walter, R J; Fernando, M B; Reyes, H M; Greager, J A


    Newcastle disease virus (NDV), strain 73-T, has previously been shown to be cytolytic to mouse tumor cells. In this study, we have evaluated the ability of NDV to replicate in and kill human tumor cells in culture and in athymic mice. Plaque assays were used to determine the cytolytic activity of NDV on six human tumor cell lines, fibrosarcoma (HT1080), osteosarcoma (KHOS), cervical carcinoma (KB8-5-11), bladder carcinoma (HCV29T), neuroblastoma (IMR32), and Wilm's tumor (G104), and on nine different normal human fibroblast lines. NDV formed plaques on all tumor cells tested as well as on chick embryo cells (CEC), the native host for NDV. Plaques did not form on any of the normal fibroblast lines. To detect NDV replication, virus yield assays were performed which measured virus particles in infected cell culture supernatants. Virus yield increased 10,000-fold within 24 hr in tumor and CEC supernatants. Titers remained near zero in normal fibroblast supernatants. In vivo tumoricidal activity was evaluated in athymic nude Balb-c mice by subcutaneous injection of 9 x 10(6) tumor cells followed by intralesional injection of either live or heat-killed NDV (1.0 x 10(6) plaque forming units [PFU]), or medium. After live NDV treatment, tumor regression occurred in 10 out of 11 mice bearing KB8-5-11 tumors, 8 out of 8 with HT-1080 tumors, and 6 out of 7 with IMR-32 tumors. After treatment with heat-killed NDV no regression occurred (P less than 0.01, Fisher's exact test). Nontumor-bearing mice injected with 1.0 x 10(8) PFU of NDV remained healthy. These results indicate that NDV efficiently and selectively replicates in and kills tumor cells, but not normal cells, and that intralesional NDV causes complete tumor regression in athymic mice with a high therapeutic index.

  4. Occurrence of nephroblastomatosis with dup(18)(q11.2-q23) implicates trisomy 18 tumor screening protocol in select patients with 18q duplication. (United States)

    Starr, Lois J; Sanmann, Jennifer N; Olney, Ann Haskins; Wandoloski, Melissa; Sanger, Warren G; Coulter, Donald W


    Duplications of the long arm of chromosome 18 have been previously reported in patients with phenotypic findings similar to full trisomy 18. Trisomy 18 increases the risk for Wilms tumor and it is currently recommended that these patients undergo abdominal ultrasonography screening every 6 months. We report on nephroblastomatosis in a 27-month-old male with a 55 Mb duplication of chromosome 18q11.2-q23 (chr18:22693370-77982126, hg 19) and propose that the trisomy 18 tumor screening protocol could also benefit patients with large 18q duplications.

  5. Tumor immunoscintigraphy by means of radiolabeled monoclonal antibodies: Multicenter studies of the Italian National Research Council--Special Project Biomedical Engineering

    Energy Technology Data Exchange (ETDEWEB)

    Siccardi, A.G. (Universita di Milano (Italy))


    Four radioimmunopharmaceuticals ({sup 99m}Tc- and 111In-labeled anti-melanoma and {sup 111}In- and {sup 131}I-labeled anti-carcinoembryonic antigen F(ab')2 fragments derived from monoclonal antibodies 225.28S and F023C5) were developed by means of a collaborative effort coordinated by the Italian National Research Council, Special Project Biomedical Engineering. After appropriate pilot studies, the radioimmunopharmaceuticals, prepared by Sorin Biomedica (Saluggia, Italy), were distributed to 31 Nuclear Medicine departments in Italy and in 10 other European countries within the framework of three immunoscintigraphy multicenter studies. A total of 1245 patients were studied, 898 of whom carried 1725 documented tumor lesions; 1596 of 2193 tumor lesions (468 of which were previously unknown) were imaged by immunoscintigraphy in 785 of 990 lesion-bearing patients. Among the occult lesions, 173 were imaged in 92 patients admitted to the study as lesion-free patients. The results have been analyzed in terms of the reliability, reproducibility, and diagnostic usefulness of the method and of each immunoradiopharmaceutical.

  6. Brain metastasis in pediatric extracranial solid tumors: survey and literature review. (United States)

    Kebudi, Rejin; Ayan, Inci; Görgün, Omer; Ağaoğlu, Fulya Yaman; Vural, Sema; Darendeliler, Emin


    Brain is a rare site of metastasis in most extracranial pediatric solid tumors. The aim of this study is to investigate the incidence, treatment, prognosis of brain metastasis in extracranial pediatric malignant tumors in a single institution and to review the literature. From September 1989 to December 2002, 1100 children Pediatric Oncology, Oncology Institute, Istanbul University. Patients with parenchymal metastases in the brain were assessed. Sixteen (10 female, 6 male) of 1100 patients (1.45%) with extracranial solid tumors developed brain metastases. The median age of the patients was 10.5 (1-16) years. The diagnosis was sarcomas in 12 patients: 5 osteosarcomas, 4 Ewing's sarcoma family tumors, 1 rhabdomyosarcoma, 1 clear cell sarcoma of the soft tissue, 1 alveolar soft part sarcoma. Two patients had Wilms' tumor and two had germ cell tumors. Four patients (25%) had brain metastasis at diagnosis. Twelve (75%) developed brain metastasis during therapy or relapse at a median duration of 16 (1-70) months from initial diagnosis. All patients had metastases to various sites, mostly lung, at the time the brain metastases were detected. Treatment included surgery, followed by postoperative radiotherapy (RT) and chemotherapy (CT) in 1, S and RT in 1, S in 1, RT and CT in 6, RT in 1, CT in 1 and no treatment in 5. Only one patient with alveolar soft part sarcoma is alive with disease 20 months from diagnosis of brain metastasis. All other patients died at a median time of 2 months (2 days-6 months) from the time of brain metastasis. Children with metastatic cancer who develop headaches or any other neurologic symptom should be investigated for possible brain metastasis. Although, the outcome for these patients is dismal in this series and in the literature; reports of long term survival in a few cases with Wilms' tumor, osteosarcoma and alveolar soft part sarcoma who had isolated brain metastasis, suggest that a subset of patients may benefit from therapy.

  7. Gastrointestinal stromal tumor: analysis of 146 cases of the center of reference of the National Cancer Institute--INCA. (United States)

    Linhares, Eduardo; Gonçalves, Rinaldo; Valadão, Marcus; Vilhena, Bruno; Herchenhorn, Daniel; Romano, Sergio; Ferreira, Maria Aparecida; Ferreira, Carlos Gil; Ramos, Cintia de Araujo; de Jesus, José Paulo


    To evaluate the treatment of GIST in INCA. We conducted a retrospective analysis of all cases of GIST treated at INCA in the period from 1997 to 2009. We analyzed 146 patients with a mean age of 44.5 years and female predominance. The main symptom was abdominal pain. We observed the occurrence of a second primary tumor in 22% of cases and 92% of the immunohistochemistry exams were positive for CD117. The most frequent location was in the stomach and the high-risk group was predominant. Surgery was considered R0 (extensive) in 70% of the cases and the main sites of metastases were liver and peritoneum. Overall survival in two and five years was, respectively, 86% and 59%. There was a significant difference between overall survival (p = 0.29) of the high-risk group versus the other. Our patients presented mainly in the form of high-risk disease, with obvious impact on survival. The use of imatinib improved survival of patients with recurrent and metastatic disease. We should study its use in the setting of adjuvant and neoadjuvant therapy to improve results of the high risk group. The creation of reference centers is a need for the study of rare diseases.

  8. Renal tumor and trauma: a pitfall for conversative management

    Directory of Open Access Journals (Sweden)

    Simone de Campos Vieira Abib


    Full Text Available PURPOSE: Conservative management has been largely used for renal trauma. Although this approach is safe and highly recommended, it can hide a pre-existing unknown condition, such as tumors or urinary malformations. A high index of suspicion is needed for early recognition of these conditions. We present four cases treated at the Pediatric Oncology Institute - Federal University of São Paulo, which have been initially treated conservatively for renal trauma. MATERIALS AND METHODS: We reviewed all 218 renal cases of renal tumors treated at our institution in a 22-year period, searching for associated trauma events. RESULTS: Four cases of renal tumors were initially treated conservatively for blunt renal trauma of low energy mechanism. Patients' ages ranged from 7 to 12 years old. Two patients had no previous symptoms, one patient had hematuria and another had an abdominal mass. Computerized Axial Tomography (CT of the abdomen revealed disparate magnitude of the renal bleeding to the low energy mechanism of trauma. All patients underwent surgical treatment. Kidney specimens showed Wilms tumor in three cases and renal carcinoma in one. CONCLUSIONS: The association between renal tumors and trauma should be suspected when renal trauma hemorrhage on abdominal CT scan does not match the low energy mechanism of blunt abdominal trauma. The key for a successful diagnosis of renal tumor or congenital malformations is the high index of suspicion for these conditions.

  9. Review of phase I and II trials for Wilms' tumour - Can we optimise the search for novel agents? (United States)

    Brok, Jesper; Pritchard-Jones, Kathy; Geller, James I; Spreafico, Filippo


    Survival rates for patients with Wilms' tumour (WT) approximate 90% with refined use of currently available interventions. However, a subgroup of patients, with initial high-risk histopathology or relapsing disease, have a poor prognosis, and it is a challenge to identify and prioritise the development of new innovative approaches for these subgroups. We conducted a systematic literature search for published phase I and II clinical trials that registered patients with WTs and characterised the early phase trial activity, quantified response rates and highlighted avenues for further development. We identified 63 trials (48 phase I, three phase I/II, and 12 phase II trials) enrolling 214 patients with WTs, alongside other malignancies. The number of annually recruited WTs did not change significantly and was less than 20% of the potential candidates. The vast majority of the trials were conducted in North America, and 56 different interventions were investigated, including conventional chemotherapy and biologically targeted therapies. Overall, 33 WTs revealed some degree of tumour control. Of these, five patients demonstrated complete remission (2%), 15 patients partial response (7%) and 13 patients stable disease (6%). None of the included novel biologically targeted therapies emerged as promising interventions, and only conventional chemotherapy was able to induce a complete and partial response. We conclude that early phase trial recruitment of WTs is below expected levels, and the clinical outcome of the included patients is dismal. Improvement of the availability and recruitment to early phase trials for WTs, especially in Europe, is needed. Copyright © 2017 Elsevier Ltd. All rights reserved.

  10. Transcriptional Repression of Catalase in Mouse Skin Tumor Progression

    Directory of Open Access Journals (Sweden)

    Kevin A. Kwei


    Full Text Available Previous studies in our laboratory have shown that the elevation of reactive oxygen species levels and the repression of the antioxidant enzyme, catalase, played a critical role in the in vitro progression of benign papilloma cells to malignant carcinoma cells. Catalase message, protein levels, and activity levels were found to be downregulated in the malignantly progressed cells. The goal of this study is to further characterize the repression of catalase in malignant progression of mouse skin tumors. To validate the in vitro observations, we examined catalase expression in tumor samples generated by the multistep chemical carcinogenesis protocol. Higher levels of catalase mRNA and protein were observed in benign papillomas versus malignant carcinomas. Nuclear run-on analysis showed that catalase repression in the cultured malignant cells was transcription-dependent. Results from luciferase reporter assays indicated that malignant cells have lower catalase promoter activities than benign papilloma cells, in part through the Wilm's tumor suppressor 1 (WT1 binding site within the proximal promoter region. The WTi protein levels were found to be inversely correlated with the observed catalase promoter activities, with higher levels observed in the malignant cells versus the benign cells. These results led us to conclude that WTi is acting as a transcription repressor in catalase gene regulation during tumor progression.

  11. Rheumatoid arthritis patients fulfilling Korean National Health Insurance reimbursement guidelines for anti-tumor necrosis factor-α treatment and comparison to other guidelines. (United States)

    Hur, Jin-Wuk; Choe, Jung-Yoon; Kim, Dong-Wook; Kim, Hyun Ah; Kim, Sang-Hyon; Kim, Wan-Uk; Kim, Yun Sung; Lee, Hye-Soon; Lee, Sang-Heon; Park, Sung-Hwan; Park, Won; Park, Yong-Beom; Suh, Chang-Hee; Shim, Seung-Cheol; Song, Yeong-Wook; Yoon, Bo Young; Yu, Dae Young; Yoo, Dae Hyun


    The aim of this study was to compare anti-tumor necrosis factor-α (TNFα) treatment status in rheumatoid arthritis (RA) patients with the Korean National Health Insurance (KNHI) reimbursement eligibility criteria and with American College of Rheumatology (ACR) recommendations, Japan College of Rheumatology (JCR) guidelines and British Society for Rheumatology (BSR) guidelines. Between December 2011 and August 2012, outpatients from 17 South Korean general hospitals diagnosed with RA according to the 1987 ACR criteria were enrolled into a noninterventional, cross-sectional, observational study. Of 1700 patients (1414 female (83.2 %), mean age of 56.6 ± 12.0, mean disease duration 97.9 ± 91.8 months), 306 (18.0 %) had used anti-TNFα agents, and 224 (13.2 %) were currently using an anti-TNFα agent. Of 1394 anti-TNFα-naive patients, 32 (2.3 %) met KNHI reimbursement guidelines, 148 (10.6 %) met ACR recommendations, and 127 (9.1 %) and 126 (9.0 %) were considered eligible for anti-TNFα agents according to JCR and BSR guidelines, respectively. The main discrepancy was the higher active joint count required by the KNHI eligibility criteria. In the opinion of treating rheumatologists, the KNHI reimbursement criteria ineligibility accounted for 15.3 % (n = 213) of the reasons for not initiating anti-TNFα agents in anti-TNFα-naive group. The anti-TNFα user group showed significantly higher disease activity than the anti-TNFα-naive group based on DAS28 score. In comparison with the ACR recommendations and JCR and BSR guidelines, fewer patients met KNHI reimbursement eligibility criteria for anti-TNFα agents. The current amendment of the KNHI criteria based on DAS28 score will improve an access to biologic agents including anti-TNFα treatment for South Korean patients with active RA.

  12. Canine mammary gland tumors. (United States)

    Sorenmo, Karin


    The National Consensus Group recommends that all women with tumors larger than 1 cm be offered chemotherapy regardless of tumor histology of lymph node status. This recommendation is to ensure that everyone at risk for failing, even though the risk may be low in women with relatively small tumors and favorable histology, has a choice and receives the benefit of adjuvant chemotherapy. This type of treatment recommendation may also be made in dogs based on recognized, well-accepted prognostic factors such as tumor size, stage, type, and histologic differentiation. Based on the limited clinical information available in veterinary medicine, the drugs that are effective in human breast cancer, such as cyclophosphamide, 5-fluorouracil, and doxorubicin, may also have a role in the treatment of malignant mammary gland tumors in dogs. Randomized prospective studies are needed, however, to evaluate the efficacy of chemotherapy in dogs with high-risk mammary gland tumors and to determine which drugs and protocols are the most efficacious. Until such studies are performed, the treatment of canine mammary gland tumors will be based on the individual oncologist's understanding of tumor biology, experience, interpretation of the available studies, and a little bit of gut-feeling. Table 2 is a proposal for treatment guidelines for malignant canine mammary gland tumors according to established prognostic factors, results from published veterinary studies, and current recommendations for breast cancer treatment in women.

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

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


    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.

  14. Genome-wide paternal uniparental disomy as a cause of Beckwith-Wiedemann syndrome associated with recurrent virilizing adrenocortical tumors. (United States)

    Bertoin, F; Letouzé, E; Grignani, P; Patey, M; Rossignol, S; Libé, R; Pasqual, C; Lardière-Deguelte, S; Hoeffel-Fornes, C; Gaillard, D; Previderè, C; Delemer, B; Lalli, E


    Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome characterized by fetal macrosomia, macroglossia, and abdominal wall defects. BWS patients are at risk to develop Wilms tumor, neuroblastoma, hepatoblastoma, and adrenal tumors. A young woman with BWS features, but with inconclusive genetic evidence for the disease, came to clinical observation for signs of virilization at the age of 16 years. An adrenocortical tumor was diagnosed and surgically resected. The tumor underwent 2 local relapses that were also surgically treated. The patient was also operated to remove a breast fibroadenoma. SNP arrays were used to analyze chromosome abnormalities in normal and tumor samples from the patient and her parents. The patient presented genome-wide mosaic paternal uniparental disomy (patUPD) both in the adrenocortical and the breast tumors, with different degrees of loss of heterozygosity (LOH). The more recent relapses of the adrenocortical tumor showed a loss of part of chromosome 17p that was absent in the first tumor. Analysis of a skin biopsy sample also showed mosaic patUPD with partial LOH, while no LOH was detected in leukocyte DNA. This case shows that virilizing adrenocortical tumors may be a clinical feature of patients with BWS. The SNP array technology is useful to diagnose genome-wide patUPD mosaicism in BWS patients with an inconclusive molecular diagnosis and underlines the tumorigenic potential of the absence of the maternal genome combined with an excess of the paternal genome.

  15. Skeletal sequelae of radiation therapy for malignant childhood tumors

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    Butler, M.S.; Robertson, W.W. Jr.; Rate, W.; D' Angio, G.J.; Drummond, D.S. (UMDNJ Robert Wood Johnson Medical School, New Brunswick (USA))


    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.

  16. Hypothalamic tumor (United States)

    Complications of brain surgery may include: Bleeding Brain damage Death (rarely) Infection Seizures can result from the tumor or from any surgical procedure on the brain. Hydrocephalus can occur with some tumors and ...

  17. Urogenital tumors

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    Weller, R.E.


    An overview is provided for veterinary care of urogenital tumors in companion animals, especially the dog. Neoplasms discussed include tumors of the kidney, urinary bladder, prostate, testis, ovary, vagina, vulva and the canine transmissible venereal tumor. Topics addressed include description, diagnosis and treatment.

  18. Brain Tumors (United States)

    A brain tumor is a growth of abnormal cells in the tissues of the brain. Brain tumors can be benign, with no cancer cells, ... cancer cells that grow quickly. Some are primary brain tumors, which start in the brain. Others are ...

  19. Odontogenic Tumors




    DefinitionThe neoplasms that consist of the cells considered specialized for odontogenesis, and their product (dentin, enamel, cementum) are called odontogenic tumors.ClassificationTo initiate odontogenesis, epithelium is a must. Same rule holds for the odontogenic tumors: without odontogenic epithelium, odontogenic tumors cannot be, without the induction of odontogenic epithelium odontogenic mesenchyme cannot develop.

  20. Predictive role of minimal residual disease and log clearance in acute myeloid leukemia: a comparison between multiparameter flow cytometry and Wilm's tumor 1 levels. (United States)

    Rossi, Giovanni; Minervini, Maria Marta; Melillo, Lorella; di Nardo, Francesco; de Waure, Chiara; Scalzulli, Potito Rosario; Perla, Gianni; Valente, Daniela; Sinisi, Nicola; Cascavilla, Nicola


    In acute myeloid leukemia (AML), the detection of minimal residual disease (MRD) as well as the degree of log clearance similarly identifies patients with poor prognosis. No comparison was provided between the two approaches in order to identify the best one to monitor follow-up patients. In this study, MRD and clearance were assessed by both multiparameter flow cytometry (MFC) and WT1 expression at different time points on 45 AML patients achieving complete remission. Our results by WT1 expression showed that log clearance lower than 1.96 after induction predicted the recurrence better than MRD higher than 77.0 copies WT1/10(4) ABL. Conversely, on MFC, MRD higher than 0.2 % after consolidation was more predictive than log clearance below 2.64. At univariate and multivariate analysis, positive MRD values and log clearance below the optimal cutoffs were associated with a shorter disease-free survival (DFS). At the univariate analysis, positive MRD values were also associated with overall survival (OS). Therefore, post-induction log clearance by WT1 and post-consolidation MRD by MFC represented the most informative approaches to identify the relapse. At the optimal timing of assessment, positive MRD and log-clearance values lower than calculated thresholds similarly predicted an adverse prognosis in AML.

  1. Corrigendum: Janković et al., "Wilms tumor (WT1 gene expression in children with acute leukemia in Serbia" published in Genetika 48(1:409-421

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    Full Text Available The authors of the above-referenced article have requested a corrigendum to it. The article should include the following correction in affiliation of authors: 2Institute of Molecular Genetics and Genetic Engineering, University of Belgrade, Belgrade, Serbia. Link to the corrected article 10.2298/GENSR1601409J

  2. Clinico pathologic session: case 4/2000 - heart failure in a 9-year-old child after treatment with anthracycline for Wilm's tumor

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    Mansur, Alfredo Jose [Sao Paulo Univ., SP (Brazil). Faculdade de Medicina. Instituto do Coracao] (ed.)


    The evolution of the case is studied. The patient was diagnosed with state III nephroblastoma and underwent nephrectomy, followed by chemotherapy and radiotherapy. Diagnostic examinations (electrocardiogram, chest X-ray and radiocardiography with technetium-99m labelled red blood cell) are discussed. Several differential diagnoses are reported as well as urologist's comments. Autopsy results are presented.

  3. Mutation or loss of Wilms' tumor gene 1 (WT1 are not major reasons for immune escape in patients with AML receiving WT1 peptide vaccination

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    Ochsenreither Sebastian


    Full Text Available Abstract Background Efficacy of cancer vaccines may be limited due to immune escape mechanisms like loss or mutation of target antigens. Here, we analyzed 10 HLA-A2 positive patients with acute myeloid leukemia (AML for loss or mutations of the WT1 epitope or epitope flanking sequences that may abolish proper T cell recognition or epitope presentation. Methods All patients had been enrolled in a WT1 peptide phase II vaccination trial (NCT00153582 and ultimately progressed despite induction of a WT1 specific T cell response. Blood and bone marrow samples prior to vaccination and during progression were analyzed for mRNA expression level of WT1. Base exchanges within the epitope sequence or flanking regions (10 amino acids N- and C-terminal of the epitope were assessed with melting point analysis and sequencing. HLA class I expression and WT1 protein expression was analyzed by flow cytometry. Results Only in one patient, downregulation of WT1 mRNA by 1 log and loss of WT1 detection on protein level at time of disease progression was observed. No mutation leading to a base exchange within the epitope sequence or epitope flanking sequences could be detected in any patient. Further, no loss of HLA class I expression on leukemic blasts was observed. Conclusion Defects in antigen presentation caused by loss or mutation of WT1 or downregulation of HLA molecules are not the major basis for escape from the immune response induced by WT1 peptide vaccination.

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

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


    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.

  5. Mobile phones, cordless phones and rates of brain tumors in different age groups in the Swedish National Inpatient Register and the Swedish Cancer Register during 1998-2015. (United States)

    Hardell, Lennart; Carlberg, Michael


    We used the Swedish Inpatient Register (IPR) to analyze rates of brain tumors of unknown type (D43) during 1998-2015. Average Annual Percentage Change (AAPC) per 100,000 increased with +2.06%, 95% confidence interval (CI) +1.27, +2.86% in both genders combined. A joinpoint was found in 2007 with Annual Percentage Change (APC) 1998-2007 of +0.16%, 95% CI -0.94, +1.28%, and 2007-2015 of +4.24%, 95% CI +2.87, +5.63%. Highest AAPC was found in the age group 20-39 years. In the Swedish Cancer Register the age-standardized incidence rate per 100,000 increased for brain tumors, ICD-code 193.0, during 1998-2015 with AAPC in men +0.49%, 95% CI +0.05, +0.94%, and in women +0.33%, 95% CI -0.29, +0.45%. The cases with brain tumor of unknown type lack morphological examination. Brain tumor diagnosis was based on cytology/histopathology in 83% for men and in 87% for women in 1980. This frequency increased to 90% in men and 88% in women in 2015. During the same time period CT and MRI imaging techniques were introduced and morphology is not always necessary for diagnosis. If all brain tumors based on clinical diagnosis with CT or MRI had been reported to the Cancer Register the frequency of diagnoses based on cytology/histology would have decreased in the register. The results indicate underreporting of brain tumor cases to the Cancer Register. The real incidence would be higher. Thus, incidence trends based on the Cancer Register should be used with caution. Use of wireless phones should be considered in relation to the change of incidence rates.

  6. Current Trends and Healthcare Resource Usage in the Hospital Treatment of Primary Malignant Brain Tumor in Japan: A National Survey Using the Diagnostic Procedure Combination Database (J-ASPECT Study-Brain Tumor) (United States)

    YOSHIMOTO, Koji; KADA, Akiko; KUGA, Daisuke; HATAE, Ryusuke; MURATA, Hideki; AKAGI, Yojiro; NISHIMURA, Kunihiro; KUROGI, Ryota; NISHIMURA, Ataru; HATA, Nobuhiro; MIZOGUCHI, Masahiro; SAYAMA, Tetsuro; IIHARA, Koji


    We conducted this study to clarify the current trends and healthcare resource usage in the treatment of inpatients with primary malignant brain tumors. The Diagnostic Procedure Combination (DPC) data of all inpatients treated between 2013 and 2014 in the 370 core and branch hospitals enrolled in the Japanese Neurosurgical Society training program were collected. DPC is a discharge abstract and administrative claims database of inpatients. We assessed 6,142 primary, malignant brain tumor patients. Patient information, diagnostic information, treatment procedure, and healthcare resource usage were analyzed. Chemotherapy was the most frequent treatment (27% of cases), followed by surgery (13%) and surgery + chemo-radiotherapy (11%). Temozolomide (TMZ), the most frequently used chemotherapeutic drug, was administered to 1,236 patients. Concomitant TMZ and radiotherapy was administered to 816 patients, and was performed according to the Stupp regimen in many cases. The mean length of hospital stay (LOS) was 16 days, and the mean medical cost was 1,077,690 yen. The average medical cost of TMZ-only treatment was 1,138,620 yen whilst it was 4,424,300 yen in concomitant TMZ patients. The LOS was significantly shorter in high-volume than in low-volume hospitals, and the medical cost was higher in hospitals treating 21–50 patients compared to those treating 1–10 patients. However, the direct medical cost of TMZ treatment was the same across different volume hospitals. This is the first report of current trends and healthcare resource usage in the treatment of primary malignant brain tumor inpatients in the TMZ era in Japan. PMID:27680329

  7. Current Trends and Healthcare Resource Usage in the Hospital Treatment of Primary Malignant Brain Tumor in Japan: A National Survey Using the Diagnostic Procedure Combination Database (J-ASPECT Study-Brain Tumor). (United States)

    Yoshimoto, Koji; Kada, Akiko; Kuga, Daisuke; Hatae, Ryusuke; Murata, Hideki; Akagi, Yojiro; Nishimura, Kunihiro; Kurogi, Ryota; Nishimura, Ataru; Hata, Nobuhiro; Mizoguchi, Masahiro; Sayama, Tetsuro; Iihara, Koji


    We conducted this study to clarify the current trends and healthcare resource usage in the treatment of inpatients with primary malignant brain tumors. The Diagnostic Procedure Combination (DPC) data of all inpatients treated between 2013 and 2014 in the 370 core and branch hospitals enrolled in the Japanese Neurosurgical Society training program were collected. DPC is a discharge abstract and administrative claims database of inpatients. We assessed 6,142 primary, malignant brain tumor patients. Patient information, diagnostic information, treatment procedure, and healthcare resource usage were analyzed. Chemotherapy was the most frequent treatment (27% of cases), followed by surgery (13%) and surgery + chemo-radiotherapy (11%). Temozolomide (TMZ), the most frequently used chemotherapeutic drug, was administered to 1,236 patients. Concomitant TMZ and radiotherapy was administered to 816 patients, and was performed according to the Stupp regimen in many cases. The mean length of hospital stay (LOS) was 16 days, and the mean medical cost was 1,077,690 yen. The average medical cost of TMZ-only treatment was 1,138,620 yen whilst it was 4,424,300 yen in concomitant TMZ patients. The LOS was significantly shorter in high-volume than in low-volume hospitals, and the medical cost was higher in hospitals treating 21-50 patients compared to those treating 1-10 patients. However, the direct medical cost of TMZ treatment was the same across different volume hospitals. This is the first report of current trends and healthcare resource usage in the treatment of primary malignant brain tumor inpatients in the TMZ era in Japan.

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

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    Wyttenbach, R.; Vock, P.; Tschaeppeler, H. [Department of Diagnostic Radiology, Division of Children`s Radiology, University Hospital, Bern (Switzerland)


    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.) With 5 figs., 3 tabs., 20 refs.

  9. Pindborg tumor (United States)

    Caliaperoumal, Santhosh Kumar; Gowri, S.; Dinakar, J.


    Calcifying epithelial odontogenic tumor (CEOT), also known as Pindborg tumor, is a rare odontogenic epithelial neoplasm. So far, nearly 200 cases have been reported in the literature. We are reporting a case of CEOT in a 42-year-old male patient with painless bony swelling in the mandible. The clinical, radiographic, and histopathologic features are discussed with relevant references. PMID:27041911

  10. Pindborg tumor

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    Santhosh Kumar Caliaperoumal


    Full Text Available Calcifying epithelial odontogenic tumor (CEOT, also known as Pindborg tumor, is a rare odontogenic epithelial neoplasm. So far, nearly 200 cases have been reported in the literature. We are reporting a case of CEOT in a 42-year-old male patient with painless bony swelling in the mandible. The clinical, radiographic, and histopathologic features are discussed with relevant references.

  11. Wilms' Tumour 1 (WT1) peptide vaccination in patients with acute myeloid leukaemia induces short-lived WT1-specific immune responses. (United States)

    Uttenthal, Benjamin; Martinez-Davila, Irma; Ivey, Adam; Craddock, Charles; Chen, Frederick; Virchis, Andras; Kottaridis, Panagiotis; Grimwade, David; Khwaja, Asim; Stauss, Hans; Morris, Emma C


    Wilms' Tumour 1 (WT1) is a zinc finger transcription factor that is over-expressed in acute myeloid leukaemia (AML). Its restricted expression in normal tissues makes it a promising target for novel immunotherapies aiming to accentuate the cytotoxic T lymphocyte (CTL) response against AML. Here we report a phase I/II clinical trial of subcutaneous peptide vaccination with two separate HLA-A2-binding peptide epitopes derived from WT1, together with a pan-DR binding peptide epitope (PADRE), in Montanide adjuvant. Eight HLA-A2-positive patients with poor risk AML received five vaccination cycles at 3-weekly intervals. The three cohorts received 0·3, 0·6 and 1 mg of each peptide, respectively. In six patients, WT1-specific CTL responses were detected using enzyme-linked immunosorbent spot assays and pWT126/HLA-A*0201 tetramer staining, after ex vivo stimulation with the relevant WT1 peptides. However, re-stimulation of these WT1-specific T cells failed to elicit secondary expansion in all four patients tested, suggesting that the WT1-specific CD8(+) T cells generated following vaccination may be functionally impaired. No correlation was observed between peptide dose, cellular immune response, reduction in WT1 mRNA expression and clinical response. Larger studies are indicated to confirm these findings. © 2013 The Authors. British Journal of Haematology published by John Wiley & Sons Ltd.

  12. Tumor carcinoide apendicular Appendiceal carcinoid tumor

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    Julio Vázquez Palanco


    Full Text Available El objetivo de este trabajo fue dar a conocer un interesante caso de tumor carcinoide que se presentó con cuadro clínico de apendicitis aguda. El paciente fue un varón de 8 años de edad, al cual se realizó apendicectomía a causa de una apendicitis aguda. El resultado anatomopatológico confirmó un tumor de células endocrinas (argentafinoma, tumor carcinoide en el tercio distal del órgano, que infiltraba hasta la serosa, y apendicitis aguda supurada. El paciente fue enviado a un servicio de oncohematología para tratamiento oncoespecífico. Por lo inusual de estos tumores en edades tempranas y por lo que puede representar para el niño una conducta no consecuente, decidimos presentar este caso a la comunidad científica nacional e internacional. Es extremadamente importante el seguimiento de los pacientes con apendicitis aguda y de las conclusiones del examen histológico, por lo que puede representar para el niño una conducta inadecuada en una situación como esta.The objective of this paper was to make known an interesting case of carcinoid tumor that presented a clinical picture of acute appendicitis.The patient was an eight-year-old boy that underwent appendectomy due to an acute appendicitis. The anatomopathological report confirmed an endocrine cell tumor (argentaffinoma, carcinoid tumor in the distal third of the organ that infiltrated up to the serosa, and acute suppurative appendicitis. The patient was referred to an oncohematology service for oncospecific treatment. As it is a rare tumor at early ages, and taking into account what a inconsequent behavior may represent for the child, it was decided to present this case to the national and international scientific community. The follow-up of the patients with acute appendicitis and of the conclusions of the histological examination is extremely important considering what an inadequate conduct may represent for the child in a situation like this.

  13. Ear Tumors (United States)

    ... Japanese Espaniol Find information on medical topics, symptoms, drugs, procedures, news and more, written in everyday language. * This is ... the Ears, Nose, and Throat Additional Content Medical News Ear Tumors ... NOTE: This is the Consumer Version. DOCTORS: Click ...

  14. Pituitary tumor (United States)

    ... enough of its hormones. This condition is called hypopituitarism . The causes of pituitary tumors are unknown. Some ... Cyst Endocrine glands Gigantism Growth hormone test Hyperthyroidism Hypopituitarism Multiple endocrine neoplasia (MEN) I Prolactin blood test ...

  15. Tumor Grade (United States)

    ... Other Funding Find NCI funding for small business innovation, technology transfer, and contracts Training Cancer Training at ... much of the tumor tissue has normal breast (milk) duct structures Nuclear grade : an evaluation of the ...

  16. Pituitary Tumors (United States)

    ... impairment, and behavioral changes. Tumors can also either produce excessive amounts of hormone or limit how much hormone is produced. The hormones most commonly affected include: growth hormone (regulates body height and structure), prolactin (controls ...

  17. Ependymal tumors. (United States)

    Moynihan, Timothy J


    Ependymal tumors are rare malignancies that arise from the cells that line the ventricles and central canal of the spinal cord. Although they are more common in children, adults may also be effected by ependymal tumors. Prognosis is dependent on tumor location, histology, especially for myxopapillary tumors that tend to occur in the lumbar spine, extent of surgical resection, and stage of disease. Standard therapy consists of complete resection when feasible. The exact role of adjuvant radiotherapy in patients with radiographically confirmed complete resection is poorly defined. Patients with known residual disease may benefit from local radiation therapy, but the extent of radiation field and total dose are controversial. Even in patients treated with involved field radiotherapy, most relapses occur within the original tumor bed, thus local control remains the biggest obstacle to effective therapy. Chemotherapy has little impact against this tumor and has no role in the adjuvant setting, outside of a well designed clinical trial, with the possible exception of children younger than 5 years in an effort to delay radiation. A minority of patients may respond to one of several chemotherapy regimens at the time of recurrence, but the impact of this therapy is limited. Newer treatment strategies are needed.

  18. Tumor Types: Understanding Brain Tumors (United States)

    ... Classification of Tumors of the Central Nervous System Purchase WHO Blue Book NBTS Official Statement Questions and ... Privacy Copyright Site Search Search term Submit Submit Facebook Twitter YouTube Flickr

  19. Rhabdoid tumors of the kidney in childhood - a retrospective radiomorphologic analysis of 22 patients as part of the nephroblastoma study SIOP 93/01-GPOH registered cases; Rhabdoidtumoren der Niere im Kindesalter. Eine retrospektive radiomorphologische Analyse von 22 im Rahmen der Nephroblastomstudie SIOP 93/01-GPOH registrierten Faellen

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    Schenk, J.P.; Engelmann, D.; Rohrschneider, W.; Zieger, B.; Troeger, J. [Abt. Paediatrische Radiologie, Radiologische Universitaetsklinik Heidelberg (Germany); Semler, O.; Graf, N. [Abt. Paediatrische Onkologie und Haematologie, Universitaetskinderklinik Homburg/Saar (Germany)


    Purpose: The primary diagnosis of renal masses in children is made by imaging studies. This retrospective analysis describes the imaging features of rhabdoid tumors (RT) with US, CT and MRI, to point out characteristics and to evaluate the possibility of differentiation between RT and Wilms tumor. Materials and Methods: We reviewed 10 MRI (6 STIR, 9 T1w, 8T2w, 10 T1 post KM), 15 CT (9 Nativ-CT, 14 KM-CT) and 14 US images of 22 patients (age 2-57 months) with histopathologically confirmed RT. The following characteristics were evaluated: subcapsular fluid collection, multiple tumor lobules, presence of calcification, primary tumor size, visibility of tumor margin, tumor necrosis and metastases. Results: The mean total tumor volume was 238 ml. 19 RT were located in the perihilar/medullary region with invasion of the renal hilum, and 5/22 tumors showed multiple tumor lobules. Subcapsular fluid collection was found in 6/22 cases. Calcifications were present in 6/19. Eleven tumors were well defined from the renal parenchyma, 9 poorly defined, 2 could not be assessed. In 19/22 cases tumor necrosis was found. Distant metastases were seen in 8 cases in the lung, in 3 cases in the CNS. Metastases of regional lymph nodes were seen in 9 cases. Conclusion: The evaluated characteristics frequently found in RT are not indicative of these tumors. RT cannot clearly be differentiated from Wilms tumor by imaging studies. Because of frequent involvement of the CNS and lung, a MRI of the CNS and CT of the lung is indicated after histopathologic diagnosis of RT is made. (orig.) [German] Ziel: Die Erstdiagnose von Nierentumoren im Kindesalter erfolgt bildgebend. Ziel dieser Studie ist es, den seltenen Rhabdoidtumor (RT) zu beschreiben und die Moeglichkeit der Differenzierung zum Wilms-Tumor zu beurteilen. Material und Methodik: Bildgebende Untersuchungen von 22 Patienten mit RT (Alter: 2-57 Monate) wurden retrospektiv beurteilt. Ausgewertet wurden 10 MRT (6 STIR, 9 T1 w, 8 T2 w, 10 T1

  20. Is the NBN gene mutation I171V a potential risk factor for malignant solid tumors in children? (United States)

    Nowak, Jerzy; Mosor, Maria; Nowicka, Karina; Rembowska, Jolanta; Januszkiewicz, Danuta


    NBN gene is considered as one of the low-to-moderate cancer susceptibility gene. At least 4 germline NBN mutations have been found in several malignancies in adults. In our studies, we observed the high incidence of germline mutation I171V of NBN gene in breast, colorectal, larynx cancer, and in multiple primary tumors. In this study, we would like to answer the question whether I171V germline mutation of NBN gene may constitute risk factor for solid tumors in children. The frequency of this mutation has been analyzed in patients with neuroblastoma (n=66), Wilms tumor (n=54), medulloblastoma (n=57), and rhabdomyosarcoma (n=82) hospitalized in Pediatric Oncology, Hematology and Bone Marrow Transplantation Department in the years between 1987 and 2010. About 2947 anonymous blood samples collected on Guthrie cards drawn from the newborn screening program of the Wielkopolska region have been used as controls. All the patients and controls came from the same geographical region. I171V mutation of the NBN gene has been observed in 5 controls. Among children with solid tumors only in 1 child with medulloblastoma I171V variant has been found. In conclusion, I171V germline mutation in contrary to adults cannot be considered as a risk factor for children malignancies. However, owing to low number of patients with solid tumors the possibility of a Type II error may exist.

  1. Testicular tumors

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    Giovanni Rosti


    Full Text Available Germ cell tumors of the testes represent a unique paradigm of diseases which can be cured even in extremely advanced phase. Unfortunately, this makes them unique among adult solid tumors. Seminoma and non seminoma are relatively rare with approximatively 25,000 patients in Europe per year, but numbers are increasing world wide. Different strategies are needed depending on stage and prognostic scores. Seminoma is extremely sensitive to radiation therapy and chemotherapy, while all germ cell tumors show a very good response to chemotherapy. Clinical stage I seminoma is currently treated with radiation, single course carboplatin or surveillance policy. Clinical stage I non seminoma can also be approached with different strategies such as retroperitoneal lymph node dissection, observation or one-two courses of standard chemotherapy. Stage II seminoma may be treated with either radiation or chemotherapy, while for all advanced stages chemotherapy is mandatory. Since the mid-eighties PEB (Cisplatin, Etoposide and Bleomycin is the regimen of choice and no other schedule has proved superior in terms of efficacy. Surgery on the residual disease is crucial to the whole strategy and should be performed or attempted in all cases. Consequently, the correct treatment strategy for these tumors does not depend only on the ability of a single physician, but on a skilled team specialized in this particular tumor. Second line therapies (VeIP, PEI, TIP can cure 25%–40% of patients, but improved strategies for resistant tumors are desperately needed. High-dose chemotherapy has shown very good results in some studies while being less impressive in others. In any case, it should remain an option for relapsing patients and could be used in some cases of upfront chemotherapy in patients with slow marker decline, but this should only be considered in referring centers.

  2. Thermoradiotherapy of malignant tumors

    Energy Technology Data Exchange (ETDEWEB)

    Hatano, Kazuo; Itami, Jun; Arimizu, Noboru (Chiba Univ. (Japan). School of Medicine); Uno, Takashi; Toita, Takafumi; Shiina, Takeki; Mikuriya, Shuuichi; Yamada, Tsunehisa


    From October 1986 to June 1989, 79 patients with malignant tumors were treated by radiation therapy combined with hyperthermia at National Medical Center Hospital. Seventy two patients (male: 48, female: 23) were evaluable. Average age was 62.4 years old (ranged 20-81 years old). Irradiation was delivered twice to fifth weekly in tumor doses of 50 to 60 Gy (TDF 82-122). We used 3 types of hyperthermic equipment, Thermotron RF8, BSD 1000 System and Endoradiotherm 100A. Hyperthermia was initiated within 30 minutes following irradiation, most of the patients being treated with adequate equipment, 41-44degC, for 60-70 minutes every 72 hours. Fifteen of 71 patients (20.8%) showed a complete response and 36 patients (50%) showed a partial response, so effective rate was 70.8% of all. In superficial tumors, 4 of 21 patients (21.5%) showed a complete response and 8 of 21 patients showed a partial response, effective rate was 63.2% of all. In deep seated tumor, 10 of 46 patients (21.7%) showed a complete response and 25 of all patients showed a partial response, so effective rate was 76.1% of all. Five patients were heated following only intra-tumor injection of OK-432 and its effective rate was 75%. We think that this modality of therapy will be effective in cases which heating area had been irradiated over tolerable doses. CR rate of superficial tumors according to intra-tumor center temperature tends to higher in the cases of higher tumor temperature. In deep seated tumor, 11 patients (23.9%) had reached over 43degC, 29 patients (63.0%) heated 41-43degC, 6 patients (13.0%) heated under 41degC and CR rate of each group were 36.4%, 17.2%, 16.7%, respectively. We think that thermotherapy with irradiation is an effective therapy in the treatment of malignancies but the improvement of heating equipment will be expected. (author).

  3. Tumor thrombus

    DEFF Research Database (Denmark)

    Ravina, Mudalsha; Hess, Søren; Chauhan, Mahesh Singh


    PURPOSE: Thrombosis in cancer may manifest itself as venous thromboembolic disease or tumor thrombosis (TT). We present our experience with incidentally detected TT on FDG PET/CT in 21 oncologic patients. PATIENTS AND METHODS: We retrospectively reviewed all FDG PET/CT examinations during a 5-year......), but most other major branches of the venous vasculature was represented, and some patients had thrombi in several vessels. FDG uptake was linear in 7 patients, linear with a dilated vessel in 6 patients, and focal in 7 patients. The mean SUVmax of the primary tumors was 10.3 (range, 2.6-31.2; median, 6...

  4. Morphometric evaluation and clinical correlations in pediatric malignant small round cell tumors

    Directory of Open Access Journals (Sweden)

    Cherry Bansal


    Full Text Available Aims: Nuclear size increases in malignant tumors and reflects DNA content, ploidy and proliferation index. Present study investigated if the nuclear morphometry could differentiate histomorphologically similar paediatric malignant small round cell tumors on hematoxylin and eosin stained sections for diagnostics in a resource poor setting. Settings and Design: All the consecutive malignant pediatric tumors received in Pathology Department from other faculties of King George′s Medical University and also those referred directly to Pathology Department from other hospitals of city/other cities during 3 years period were recorded. Materials and Methods: Morphometric analysis was done in 22 confirmed (by higher ancillary techniques but histomorphologically difficult to differentiate round cell tumors. All sections were analyzed by cell images from six different areas, using Leica Q win 500 images software. Results: Nuclear measurements were obtained for retinoblastoma (RB (nine cases, neuroblastoma (five cases, Wilms tumor (WT (three cases, rhabdomyosarcoma (three cases, malignant hemangiopericytoma (one case and non-Hodgkin lymphoma (one case. Among the RBs, maximum mean nuclear area percent (24.93 was seen in a case with nerve involvement and metastasis, followed by cases with only nerve involvement (21.60 and smallest area (16.57 was in non-nerve involving, nonmetastatic cases. All five cases of neuroblastoma had almost similar mean nuclear area percent (18.05-18.29. WT case with metastasis had higher nuclear area (21.25 than nonmetastatic (19.47. Amongst all the tumors, minimum value (14.93 was seen in malignant hemangiopericytoma. Conclusion: Morphometric evaluation in paediatric malignant round cell tumors have generated useful data, and needs further multicentric confirmation for implementation.

  5. [Third National Ovarian Consensus. 2011. Grupo de Investigación en Cáncer de Ovario y Tumores Ginecológicos de México "GICOM"]. (United States)

    Gallardo-Rincón, Dolores; Cantú-de-León, David; Alanís-López, Patricia; Alvarez-Avitia, Miguel Angel; Bañuelos-Flores, Joel; Herbert-Núñez, Guillermo Sidney; Oñate-Ocaña, Luis Fernando; Pérez-Montiel, María Delia; Rodríguez-Trejo, Amelia; Ruvalcaba-Limón, Eva; Serrano-Olvera, Alberto; Ortega-Rojo, Andrea; Cortés-Esteban, Patricia; Erazo-Valle, Aura; Gerson-Cwilich, Raquel; De-la-Garza-Salazar, Jaime; Green-Renner, Dan; León-Rodríguez, Eucario; Morales-Vásquez, Flavia; Poveda-Velasco, Andrés; Aguilar-Ponce, José Luis; Alva-López, Luis Felipe; Alvarado-Aguilar, Salvador; Alvarado-Cabrero, Isabel; Aquino-Mendoza, Cinthia Alejandra; Aranda-Flores, Carlos Eduardo; Bandera-Delgado, Artfy; Barragán-Curiel, Eduardo; Barrón-Rodríguez, Patricia; Brom-Valladares, Rocío; Cabrera-Galeana, Paula Anel; Calderillo-Ruiz, Germán; Camacho-Gutiérrez, Salvador; Capdeville-García, Daniel; Cárdenas-Sánchez, Jesús; Carlón-Zárate, Elisa; Carrillo-Garibaldi, Oscar; Castorena-Roji, Gerardo; Cervantes-Sánchez, Guadalupe; Coronel-Martínez, Jaime Alberto; Chanona-Vilchis, José Gregorio; Díaz-Hernández, Verónica; Escudero-de-los Ríos, Pedro; Garibay-Cerdenares, Olga; Gómez-García, Eva; Herrera-Montalvo, Luis Alonso; Hinojosa-García, Luz María; Isla-Ortiz, David; Jiménez-López, Josefina; Lavín-Lozano, Arturo Javier; Limón-Rodriguez, Jesús Alberto; López-Basave, Horacio Noé; López-García, Sergio César; Maffuz-Aziz, Antonio; Martínez-Cedillo, Jorge; Martínez-López, Dulce María; Medina-Castro, Juan Manuel; Melo-Martínez, Carlos; Méndez-Herrera, Carmen; Montalvo-Esquivel, Gonzalo; Morales-Palomares, Miguel Angel; Morán-Mendoza, Andrés; Morgan-Villela, Gilberto; Mota-García, Aída; Muñoz-González, David Eduardo; Ochoa-Carrillo, Francisco J; Pérez-Amador, Maricruz; Recinos-Money, Edgar; Rivera-Rivera, Samuel; Robles Flores, Juan U; Rojas-Castillo, Edith; Rojas-Marín, Carlos; Salas-Gonzáles, Efraín; Sámano-Nateras, Liliana; Santibañez-Andrade, Miguel; Santillán-Gómez, Antonio; Silva-García, Araceli; Silva, Juan Alejandro; Solorza-Luna, Gilberto; Tabarez-Ortiz, Adán Raúl; Talamás-Rohana, Patricia; Tirado-Gómez, Laura Leticia; Torres-Lobatón, Alfonso; Quijano-Castro, Félix


    Ovarian cancer (OC) is the third most common gynecologic malignancy worldwide. Most of cases it is of epithelial origin. At the present time there is not a standardized screening method, which makes difficult the early diagnosis. The 5-year survival is 90% for early stages, however most cases present at advanced stages, which have a 5-year survival of only 5-20%. GICOM collaborative group, under the auspice of different institutions, have made the following consensus in order to make recommendations for the diagnosis and management regarding to this neoplasia. The following recommendations were made by independent professionals in the field of Gynecologic Oncology, questions and statements were based on a comprehensive and systematic review of literature. It took place in the context of a meeting of two days in which a debate was held. These statements are the conclusions reached by agreement of the participant members. No screening method is recommended at the time for the detection of early lesions of ovarian cancer in general population. Staging is surgical, according to FIGO. In regards to the pre-surgery evaluation of the patient, it is recommended to perform chest radiography and CT scan of abdomen and pelvis with IV contrast. According to the histopathology of the tumor, in order to consider it as borderline, the minimum percentage of proliferative component must be 10% of tumor's surface. The recommended standardized treatment includes primary surgery for diagnosis, staging and cytoreduction, followed by adjuvant chemotherapy Surgery must be performed by an Oncologist Gynecologist or an Oncologist Surgeon because inadequate surgery performed by another specialist has been reported in 75% of cases. In regards to surgery it is recommended to perform total omentectomy since subclinic metastasis have been documented in 10-30% of all cases, and systematic limphadenectomy, necessary to be able to obtain an adequate surgical staging. Fertility-sparing surgery will

  6. Superior sulcus tumors (Pancoast tumors). (United States)

    Marulli, Giuseppe; Battistella, Lucia; Mammana, Marco; Calabrese, Francesca; Rea, Federico


    Superior Sulcus Tumors, frequently termed as Pancoast tumors, are a wide range of tumors invading the apical chest wall. Due to its localization in the apex of the lung, with the potential invasion of the lower part of the brachial plexus, first ribs, vertebrae, subclavian vessels or stellate ganglion, the superior sulcus tumors cause characteristic symptoms, like arm or shoulder pain or Horner's syndrome. The management of superior sulcus tumors has dramatically evolved over the past 50 years. Originally deemed universally fatal, in 1956, Shaw and Paulson introduced a new treatment paradigm with combined radiotherapy and surgery ensuring 5-year survival of approximately 30%. During the 1990s, following the need to improve systemic as well as local control, a trimodality approach including induction concurrent chemoradiotherapy followed by surgical resection was introduced, reaching 5-year survival rates up to 44% and becoming the standard of care. Many efforts have been persecuted, also, to obtain higher complete resection rates using appropriate surgical approaches and involving multidisciplinary team including spine surgeon or vascular surgeon. Other potential treatment options are under consideration like prophylactic cranial irradiation or the addition of other chemotherapy agents or biologic agents to the trimodality approach.

  7. Tumor Biology and Immunology | Center for Cancer Research (United States)

    Tumor Biology and Immunology The Comparative Brain Tumor Consortium is collaborating with National Center for Advanced Translational Sciences to complete whole exome sequencing on canine meningioma samples. Results will be published and made publicly available.

  8. Learning about WAGR Syndrome (United States)

    ... used are: WAGR Complex Wilms' Tumor-Aniridia-Genitourinary Anomalies-Mental Retardation Syndrome Wilms' Tumor-Aniridia-Gonadoblastoma-Mental Retardation Syndrome Chromosome 11p deletion syndrome 11p deletion syndrome The cause ...

  9. Understanding Brain Tumors (United States)

    ... to Know About Brain Tumors . What is a Brain Tumor? A brain tumor is an abnormal growth
 ... Tumors” from Frankly Speaking Frankly Speaking About Cancer: Brain Tumors Download the full book Questions to ask ...

  10. Brain tumor - primary - adults (United States)

    ... Vestibular schwannoma (acoustic neuroma) - adults; Meningioma - adults; Cancer - brain tumor (adults) ... Primary brain tumors include any tumor that starts in the brain. Primary brain tumors can start from brain cells, ...

  11. Brain tumor - children (United States)

    ... children; Neuroglioma - children; Oligodendroglioma - children; Meningioma - children; Cancer - brain tumor (children) ... The cause of primary brain tumors is unknown. Primary brain tumors may ... (spread to nearby areas) Cancerous (malignant) Brain tumors ...

  12. Adrenal Gland Tumors: Statistics (United States)

    ... Gland Tumor: Statistics Request Permissions Adrenal Gland Tumor: Statistics Approved by the Cancer.Net Editorial Board , 03/ ... primary adrenal gland tumor is very uncommon. Exact statistics are not available for this type of tumor ...

  13. Granular cell tumors of the tracheobronchial tree.

    NARCIS (Netherlands)

    Maten, van der J; Blaauwgeers, JL; Sutedja, G.; Kwa, HB; Postmus, P.E.; Wagenaar, SS


    OBJECTIVE: To describe the population-based incidence and clinical characteristics of granular cell tumors of the tracheobronchial tree. METHODS: All newly registered tracheobronchial granular cell tumors in the Dutch Network and National Database for Pathology for 10 consecutive years (1990-1999) w

  14. Pediatric brain tumors

    Energy Technology Data Exchange (ETDEWEB)

    Poussaint, Tina Y. [Department of Radiology, Boston, MA (United States); Panigrahy, Ashok [Children' s Hospital of Pittsburgh of University of Pittsburgh Medical Center, Department of Radiology, Pittsburgh, PA (United States); Huisman, Thierry A.G.M. [Charlotte R. Bloomberg Children' s Center, Johns Hopkins Hospital, Division of Pediatric Radiology and Pediatric Neuroradiology, Baltimore, MD (United States)


    Among all causes of death in children from solid tumors, pediatric brain tumors are the most common. This article includes an overview of a subset of infratentorial and supratentorial tumors with a focus on tumor imaging features and molecular advances and treatments of these tumors. Key to understanding the imaging features of brain tumors is a firm grasp of other disease processes that can mimic tumor on imaging. We also review imaging features of a common subset of tumor mimics. (orig.)

  15. Teratoid Wilms′ tumor - A rare renal tumor

    Directory of Open Access Journals (Sweden)

    Biswanath Mukhopadhyay


    Full Text Available Teratoid Wilms′ tumor is an extremely rare renal tumor. We report a case of unilateral teratoid Wilms′ tumor in a 4-year-old girl. The patient was admitted with a right-sided abdominal mass. The mass was arising from the right kidney. Radical nephrectomy was done and the patient had an uneventful recovery. Histopathology report showed teratoid Wilms′ tumor.

  16. 78 FR 19275 - National Cancer Institute; Notice of Closed Meeting (United States)


    ... Committee: National Cancer Institute Special Emphasis Panel Tumor Immunology. Date: June 26-27, 2013. Time... HUMAN SERVICES National Institutes of Health National Cancer Institute; Notice of Closed Meeting... Training Review Branch, Division of Extramural Activities, National Cancer Institute, 6116...

  17. Cholecystokinin expression in tumors

    DEFF Research Database (Denmark)

    Rehfeld, Jens F


    in different neuroendocrine tumors; cerebral gliomas and astrocytomas and specific pediatric tumors. Tumor hypersecretion of CCK was recently reported in a patient with a metastatic islet cell tumor and hypercholecystokininemia resulting in a novel tumor syndrome, the cholecystokininoma syndrome. This review...... presents an overview of the cell-specific biogenesis of CCK peptides, and a description of the CCK expression in tumors and of the cholecystokininoma syndrome. Finally, assays for the diagnosis of CCK-producing tumors are reviewed....

  18. Bronchial carcinoid tumors: A rare malignant tumor

    African Journals Online (AJOL)


    Feb 3, 2015 ... Key words: Bronchial carcinoid tumor, diagnosis, outcome, treatment, West .... They can present with carcinoid syndrome, Cushing's ... 2012 reported BCT as a rare differential of pulmonary ... Management of carcinoid tumors.

  19. DCB - Tumor Metastasis Research (United States)

    Tumor metastasis research examines the mechanisms that allow cancer cells to leave the primary tumor and spread to another part of the body. Learn about recent tumor metastasis research studies supported by the Division of Cancer Biology.

  20. Brain Tumors (For Parents) (United States)

    ... Old Feeding Your 1- to 2-Year-Old Brain Tumors KidsHealth > For Parents > Brain Tumors Print A ... radiation therapy or chemotherapy, or both. Types of Brain Tumors There are many different types of brain ...

  1. Childhood Brain Tumors (United States)

    Brain tumors are abnormal growths inside the skull. They are among the most common types of childhood ... still be serious. Malignant tumors are cancerous. Childhood brain and spinal cord tumors can cause headaches and ...

  2. Tumors and Pregnancy (United States)

    Tumors during pregnancy are rare, but they can happen. Tumors can be either benign or malignant. Benign tumors aren't cancer. Malignant ones are. The most common cancers in pregnancy are breast cancer, cervical cancer, lymphoma, and melanoma. ...

  3. Neuroendocrine Tumor: Statistics (United States)

    ... Tumor > Neuroendocrine Tumor: Statistics Request Permissions Neuroendocrine Tumor: Statistics Approved by the Cancer.Net Editorial Board , 11/ ... the United States are diagnosed with Merkel cell skin cancer each year. Almost all people diagnosed with the ...

  4. Nation/non-nation

    DEFF Research Database (Denmark)

    Sonnichsen, André; Gad, Ulrik Pram


    Is nationality the only way of organizing political community? Given the ubiquity of the national principle, one might think so. But, in practice, the national principle is constantly challenged by what can be termed non-national identities. This article looks at manners in which such deviating...

  5. Nationalism in Stateless Nations

    DEFF Research Database (Denmark)

    Thomsen, Robert Chr.

    previously independent countries, are excellent examples of this. Building on theories of national identity-formation and nationalism, it traces the development of cultural and political nationalism, and changing images of the national self. With a focus on important fomenting factors and actors...... - intellectuals, political parties and the media - the book combines historical, sociological, political and media studies analyses in an interdisciplinary investigation, providing a comprehensive account of the waxing and waning of nationalism....

  6. Updates in Orbital Tumors

    Institute of Scientific and Technical Information of China (English)

    Nila; F.Moeloek


    Orbital anatomy, the clinical features of orbital tumors, the recent development of the diagnosis and management of orbital tumors were described. The incidence of orbital tumors in Dr. Cipto Mangunkusumo Hospital in the past years were introduced. The principle of management of orbital tumors and their prognosis were discussed.

  7. Endocrine Tumor: Overview (United States)

    ... are here Home > Types of Cancer > Endocrine Tumor Endocrine Tumor This is Cancer.Net’s Guide to Endocrine Tumor. Use the menu below to choose the ... social workers, and patient advocates. Cancer.Net Guide Endocrine Tumor Introduction Statistics Risk Factors Symptoms and Signs ...

  8. Supratentorial tumors; Supratentorielle Tumoren

    Energy Technology Data Exchange (ETDEWEB)

    Grunwald, I.; Dillmann, K.; Roth, C.; Backens, M.; Reith, W. [Universitaetsklinikum Saarland, Homburg (Germany). Klinik fuer Diagnostische und Interventionelle Neuroradiologie


    Magnetic resonance imaging is a routine diagnostic measure for a suspected intracerebral mass. Computed tomography is usually also indicated. Further diagnostic procedures as well as the interpretation of the findings vary depending on the tumor location. This contribution discusses the symptoms and diagnostics for supratentorial tumors separated in relation to their intra- or extracranial location. Supratentorial tumors include astrocytoma, differentiated by their circumscribed and diffuse growth, ganglioglioma, ependyoma, neurocytoma, primitive neuroectodermal tumors (PNET), oligodendroglioma, dysembryoplastic neuroepithelial tumors (DNET), meningoangiomatosis, pineal tumors, hamartoma, lymphoma, craniopharyngeoma and metastases. The supratentorial extracranial tumors include the choroid plexus, colloid cysts, meningeoma, infantile myofibromatosis and lipoma. The most common subforms, especially of astrocytoma, will also be presented. (orig.)

  9. Epidemiology of Brain Tumors. (United States)

    McNeill, Katharine A


    Brain tumors are the commonest solid tumor in children, leading to significant cancer-related mortality. Several hereditary syndromes associated with brain tumors are nonfamilial. Ionizing radiation is a well-recognized risk factor for brain tumors. Several industrial exposures have been evaluated for a causal association with brain tumor formation but the results are inconclusive. A casual association between the common mutagens of tobacco, alcohol, or dietary factors has not yet been established. There is no clear evidence that the incidence of brain tumors has changed over time. This article presents the descriptive epidemiology of the commonest brain tumors of children and adults.

  10. Cardiac Tumors; Tumeurs cardiaques

    Energy Technology Data Exchange (ETDEWEB)

    Laissy, J.P.; Fernandez, P. [Centre Hospitalier Universitaire Bichat Claude Bernard, Service d' Imagerie, 76 - Rouen (France); Mousseaux, E. [Hopital Europeen Georges Pompidou (HEGP), Service de Radiologie Cardio Vasculaire et Interventionnelle, 75 - Paris (France); Dacher, J.N. [Centre Hospitalier Universitaire Charles Nicolle, 75 - Rouen (France); Crochet, D. [Centre Hospitalier Universitaire, Hopital Laennec, Centre Hemodynamique, Radiologie Thoracique et Vasculaire, 44 - Nantes (France)


    Metastases are the most frequent tumors of the heart even though they seldom are recognized. Most primary cardiac tumors are benign. The main role of imaging is to differentiate a cardiac tumor from thrombus and rare pseudo-tumors: tuberculoma, hydatid cyst. Echocardiography is the fist line imaging technique to detect cardiac tumors, but CT and MRl arc useful for further characterization and differential diagnosis. Myxoma of the left atrium is the most frequent benign cardiac tumor. It usually is pedunculated and sometimes calcified. Sarcoma is the most frequent primary malignant tumor and usually presents as a sessile infiltrative tumor. Lymphoma and metastases are usually recognized by the presence of known tumor elsewhere of by characteristic direct contiguous involvement. Diagnosing primary and secondary pericardial tumors often is difficult. Imaging is valuable for diagnosis, characterization, pre-surgical evaluation and follow-up. (author)

  11. Gastrointestinal Neuroendocrine Tumors: Pancreatic Endocrine Tumors



    Pancreatic endocrine tumors (PETs) have long fascinated clinicians and investigators despite their relative rarity. Their clinical presentation varies depending upon whether the tumor is functional or not and also according to the specific hormonal syndrome produced. Tumors may be sporadic or inherited but little is known about their molecular pathology, especially the sporadic forms. Chromogranin A appears to be the most useful serum marker for diagnosis, staging and monitoring. Initially, t...

  12. Continuous Hyperthermic Peritoneal Perfusion (CHPP) With Cisplatin for Children With Peritoneal Cancer (United States)


    Peritoneal Neoplasms; Retroperitoneal Neoplasms; Gastrointestinal Neoplasms; Adenocarcinoma; Neuroblastoma; Ovarian Neoplasms; Sarcoma; Adrenocortical Carcinoma; Wilms Tumor; Rhabdomyosarcoma; Desmoplastic Small Round Cell Tumor

  13. Prognosis after Ipsilateral Breast Tumor Recurrence and Locoregional Recurrences in Patients Treated by Breast-Conserving Therapy in Five National surgical Adjuvant Breast and Bowel Project Protocols of Node-Negative Breast Cancer%淋巴结阴性乳腺癌保乳术后局部复发后的预后分析:来自NSABP 5个临床研究的结果

    Institute of Scientific and Technical Information of China (English)

    陈佳艺; 李学瑞


    @@ 1 文献来源 Anderson SJ,Wapnir I,Dignam JJ,et al.Prognosis after ipsilateral breast tumor recurrence and locoregional recurrences in patients treated by breast-conserving therapy in five National Surgical Adjuvant Breast and Bowel Project protocols of nodenegative breast cancer[J].J Clin Oncol,2009,27(15):2466-2473.

  14. Embolização pré operatória de tumores renais com microparticulas esféricas de tecnologia nacional. (Spherus®-First line Brasil Pre operative embolization of renal tumors with microspherical particles of national technology (Spherus®-First line Brasil

    Directory of Open Access Journals (Sweden)

    Gaudencio Espinosa


    Full Text Available Os autores relatam pela primeira vez utilização de uma nova partícula para embolização, constituída de um núcleo de acetato de polivinil revestido por polivinil-álcool, de forma esférica (Spherus®-First Line Brasil, como preparo pré-operatório em três pacientes portadores de neoplasia renal, na intenção de diminuir o tamanho do tumor e prevenir complicações hemorrágicas durante o ato operatório. Estas novas partículas foram projetadas e elaboradas nos laboratórios da COPPE/UFRJ. A embolização intra-arterial pré-operatória com estas novas partículas ocasionou acentuada isquemia em todo o tecido tumoral facilitando o procedimento cirúrgico.The authors report a brand new component for embolization composed by a polivinil acetate core and a polivinil-alcohol coat in a microspherical form ( Spherus®-First Line Brasil used as preoperative management in three patients with renal tumors in an attempt to reduce the size of the tumors and to avoid hemorrhagic complications during the operations. This new component was developed in COPPE/UFRJ laboratories. The preoperative arterial embolization with this new component caused strong ischemia in the tumor tissue , facilitating the operative procedure.

  15. Metaphyseal giant cell tumor

    Energy Technology Data Exchange (ETDEWEB)

    Pereira, L.F.; Hemais, P.M.P.G.; Aymore, I.L.; Carmo, M.C.R. do; Cunha, M.E.P.R. da; Resende, C.M.C.

    Three cases of metaphyseal giant cell tumor are presented. A review of the literature is done, demostrating the lesion is rare and that there are few articles about it. Age incidence and characteristics of the tumor are discussed.

  16. Tumor suppressor ARF (United States)

    Través, Paqui G.; Luque, Alfonso; Hortelano, Sonsoles


    ARF (alternative reading frame) is one of the most important tumor regulator playing critical roles in controlling tumor initiation and progression. Recently, we have demonstrated a novel and unexpected role for ARF as modulator of inflammatory responses. PMID:23162766

  17. Pediatric Brain Tumor Foundation (United States)

    ... you insights into your child's treatment. LEARN MORE Brain tumors and their treatment can be deadly so ... Cancer Foundation joins the PBTF Read more >> Pediatric Brain Tumor Foundation 302 Ridgefield Court, Asheville, NC 28806 ...

  18. Renal primitive neuroectodermal tumors. (United States)

    Bartholow, Tanner; Parwani, Anil


    Primitive neuroectodermal tumors exist as a part of the Ewing sarcoma/primitive neuroectodermal tumor family. These tumors most commonly arise in the chest wall and paraspinal regions; cases with a renal origin are rare entities, but have become increasingly reported in recent years. Although such cases occur across a wide age distribution, the average age for a patient with a renal primitive neuroectodermal tumor is the mid- to late 20s, with both males and females susceptible. Histologically, these tumors are characterized by pseudorosettes. Immunohistochemically, CD99 is an important diagnostic marker. Clinically, these are aggressive tumors, with an average 5-year disease-free survival rate of only 45% to 55%. Given that renal primitive neuroectodermal tumor bears many similarities to other renal tumors, it is important to review the histologic features, immunostaining profile, and genetic abnormalities that can be used for its correct diagnosis.

  19. Pituitary Tumors: Condition Information (United States)

    ... and metabolism. Thyroid-stimulating hormone is involved in growth, body temperature, and heart rate. Nonfunctioning pituitary tumors (also called nonsecretory tumors) do not produce hormones. They can press on or damage the pituitary ...

  20. Labial salivary gland tumors. (United States)

    Neville, B W; Damm, D D; Weir, J C; Fantasia, J E


    A study was conducted on labial salivary gland tumors from four oral pathology laboratories. Of the 103 identified tumors, 87 (84.5%) were from the upper lip, whereas 16 (15.5%) were from the lower lip. Of the 87 upper lip tumors, 80 (92.0%) were benign. Forty-three of these were monomorphic adenomas and 37 were pleomorphic adenomas. Seven malignant tumors of the upper lip were as follows: four adenoid cystic carcinomas, two acinic cell carcinomas, and one adenocarcinoma. Of the 16 lower lip tumors, 15 (93.8%) were malignant. Thirteen of these were mucoepidermoid carcinomas and two were acinic cell carcinomas. The only benign lower lip tumor was an intraductal papilloma. These results confirm the findings of previous investigations, showing that minor salivary gland tumors are much more common in the upper lip than the lower lip, but that lower lip tumors are more likely to be malignant.

  1. Aggressive malignant phyllodes tumor


    Nathan Roberts; Dianne M. Runk


    Introduction: Originally described in 1838 by Muller, phyllodes tumor is a rare fibroepithelial neoplasm which represents roughly 0.3–0.9% of all breast cancers. Phyllodes tumor are divided into benign, borderline and malignant histologic categories. Malignant phyllodes tumor represent anywhere from 10–30% of all phyllodes tumors. This group has both the potential to recur locally and metastasize, however not all malignant phyllodes behave this way. The challenge lays in predicting which tumo...

  2. Rhabdoid Tumor Mimicking Hemangioma

    NARCIS (Netherlands)

    Assen, Yvette J.; Madern, Gerard C.; de Laat, Peter C. J.; den Hollander, Jan C.; Oranje, Arnold P.


    We report a young boy with a malignant tumor, which remained unrecognized for 8 months because it was assumed to be a hemangioma. The presentation of a rhabdoid tumor mimicking hemangioma is very rare. It was reported only on two earlier occasions. Rhabdoid tumors are one of the most aggressive type

  3. Liver Tumors (For Parents) (United States)

    ... Teaching Kids to Be Smart About Social Media Liver Tumors KidsHealth > For Parents > Liver Tumors Print A A A What's in this ... Malignant (Cancerous) Tumors Symptoms Diagnosis Treatment Coping The liver is the body's largest solid organ. Lying next ...

  4. Rhabdoid Tumor Mimicking Hemangioma

    NARCIS (Netherlands)

    Assen, Yvette J.; Madern, Gerard C.; de Laat, Peter C. J.; den Hollander, Jan C.; Oranje, Arnold P.


    We report a young boy with a malignant tumor, which remained unrecognized for 8 months because it was assumed to be a hemangioma. The presentation of a rhabdoid tumor mimicking hemangioma is very rare. It was reported only on two earlier occasions. Rhabdoid tumors are one of the most aggressive

  5. Molecular-Guided Therapy for Childhood Cancer (United States)


    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

  6. The research on fluorescence in situ hybridization in non-urothelial tumors of urinary system%荧光原位杂交技术在泌尿系统非尿路上皮肿瘤的研究进展

    Institute of Scientific and Technical Information of China (English)

    董德鑫; 李汉忠


    As a new technique, fluorescence in situ hybridization in non-urothelial tumors of urinary system has been widely used, and has got some results: The identification of benign and malignant adrenal tumors; pathological type of renal tumor, metastasis and prognosis of renal tumors; the pathogenesis of Wilms tumor; the gene diagnosis of VHL syndrome; the pathogenesis and prognosis of prostate cancer; the gene diagnosis of testicular cancer and other tumor.%作为一种新技术,荧光原位杂交技术在泌尿系统非尿路上皮肿瘤中得到广泛应用并取得了一些成果:肾上腺肿瘤的良恶性签别;肾肿瘤的病理分型,转移预后;肾母细胞瘤的发病机理;VHL综合症的基因诊断;前列腺癌的发病机理和治疗预后;睾丸癌等其他肿瘤的基因诊断等.

  7. Aberrant p63 and WT-1 expression in myoepithelial cells of pregnancy-associated breast cancer: implications for tumor aggressiveness and invasiveness

    Directory of Open Access Journals (Sweden)

    Zheli Xu, Wan Wang, Chu-Xia Deng, Yan-gao Man


    Full Text Available Our recent studies revealed that focal alterations in breast myoepithelial cell layers significantly impact the biological presentation of associated epithelial cells. As pregnancy-associated breast cancer (PABC has a significantly more aggressive clinical course and mortality rate than other forms of breast malignancies, our current study compared tumor suppressor expression in myoepithelial cells of PABC and non-PABC, to determine whether myoepithelial cells of PABC may have aberrant expression of tumor suppressors. Tissue sections from 20 cases of PABC and 20 cases of stage, grade, and age matched non-PABC were subjected to immunohistochemistry, and the expression of tumor suppressor maspin, p63, and Wilms' tumor 1 (WT-1 in calponin positive myoepithelial cells were statistically compared. The expression profiles of maspin, p63, and WT-1 in myoepithelial cells of all ducts encountered were similar between PABC and non-PABC. PABC, however, displayed several unique alterations in terminal duct and lobular units (TDLU, acini, and associated tumor tissues that were not seen in those of non-PABC, which included the absence of p63 and WT-1 expression in a vast majority of the myoepithelial cells, cytoplasmic localization of p63 in the entire epithelial cell population of some lobules, and substantially increasing WT-1 expression in vascular structures of the invasive cancer component. All or nearly all epithelial cells with aberrant p63 and WT-1 expression lacked the expression of estrogen receptor and progesterone receptor, whereas they had a substantially higher proliferation index than their counterparts with p63 and WT-1 expression. Hyperplastic cells with cytoplasmic p63 expression often adjacent to, and share a similar immunohistochemical and cytological profile with, invasive cancer cells. To our best knowledge, our main finings have not been previously reported. Our findings suggest that the functional status of myoepithelial cells may be

  8. Aberrant p63 and WT-1 expression in myoepithelial cells of pregnancy-associated breast cancer: implications for tumor aggressiveness and invasiveness. (United States)

    Xu, Zheli; Wang, Wan; Deng, Chu-Xia; Man, Yan-Gao


    Our recent studies revealed that focal alterations in breast myoepithelial cell layers significantly impact the biological presentation of associated epithelial cells. As pregnancy-associated breast cancer (PABC) has a significantly more aggressive clinical course and mortality rate than other forms of breast malignancies, our current study compared tumor suppressor expression in myoepithelial cells of PABC and non-PABC, to determine whether myoepithelial cells of PABC may have aberrant expression of tumor suppressors. Tissue sections from 20 cases of PABC and 20 cases of stage, grade, and age matched non-PABC were subjected to immunohistochemistry, and the expression of tumor suppressor maspin, p63, and Wilms' tumor 1 (WT-1) in calponin positive myoepithelial cells were statistically compared. The expression profiles of maspin, p63, and WT-1 in myoepithelial cells of all ducts encountered were similar between PABC and non-PABC. PABC, however, displayed several unique alterations in terminal duct and lobular units (TDLU), acini, and associated tumor tissues that were not seen in those of non-PABC, which included the absence of p63 and WT-1 expression in a vast majority of the myoepithelial cells, cytoplasmic localization of p63 in the entire epithelial cell population of some lobules, and substantially increasing WT-1 expression in vascular structures of the invasive cancer component. All or nearly all epithelial cells with aberrant p63 and WT-1 expression lacked the expression of estrogen receptor and progesterone receptor, whereas they had a substantially higher proliferation index than their counterparts with p63 and WT-1 expression. Hyperplastic cells with cytoplasmic p63 expression often adjacent to, and share a similar immunohistochemical and cytological profile with, invasive cancer cells. To our best knowledge, our main finings have not been previously reported. Our findings suggest that the functional status of myoepithelial cells may be significantly

  9. WT1 peptide vaccination combined with BCG-CWS is more efficient for tumor eradication than WT1 peptide vaccination alone. (United States)

    Nakajima, Hiroko; Kawasaki, Kotomi; Oka, Yoshihiro; Tsuboi, Akihiro; Kawakami, Manabu; Ikegame, Kazuhiro; Hoshida, Yoshihiko; Fujiki, Fumihiro; Nakano, Akiko; Masuda, Tomoki; Wu, Fei; Taniguchi, Yuki; Yoshihara, Satoshi; Elisseeva, Olga A; Oji, Yusuke; Ogawa, Hiroyasu; Azuma, Ichiro; Kawase, Ichiro; Aozasa, Katsuyuki; Sugiyama, Haruo


    A Wilms' tumor gene WT1 is expressed at high levels not only in most types of leukemia but also in various types of solid tumors, including lung and breast cancer. WT1 protein has been reported to serve as a target antigen for tumor-specific immunotherapy both in vitro in human systems and in vivo in murine models. We have shown that mice immunized with WT1 peptide or WT1 cDNA could reject a challenge from WT1-expressing tumor cells (a "prophylactic" model). However, it was not examined whether WT1 peptide vaccination had the potency to reject tumor cells in a "therapeutic" setting. In the present study, we demonstrated for the first time that WT1 peptide vaccination combined with Mycobacterium bovis bacillus Calmette-Guérin cell wall skeleton (BCG-CWS) was more effective for eradication of WT1-expressing tumor cells that had been implanted into mice before vaccination (a "therapeutic" model) compared with WT1 peptide vaccination alone. An intradermal injection of BCG-CWS into mice, followed by that of WT1 peptide at the same site on the next day, generated WT1-specific cytotoxic T lymphocytes (CTLs) and led to rejection of WT1-expressing leukemia or lung cancer cells. These results showed that BCG-CWS, which was well known to enhance innate immunity, could enhance WT1-specific immune responses (acquired immunity) in combination with WT1 peptide vaccination. Therefore, WT1 peptide vaccination combined with BCG-CWS may be applied to cancer immunotherapy in clinical settings.

  10. Optimal time-points for minimal residual disease monitoring change on the basis of the method used in patients with acute myeloid leukemia who underwent allogeneic stem cell transplantation: a comparison between multiparameter flow cytometry and Wilms' tumor 1 expression. (United States)

    Rossi, Giovanni; Carella, Angelo Michele; Minervini, Maria Marta; di Nardo, Francesco; Waure, Chiara de; Greco, Michele Mario; Merla, Emanuela; Cillis, Giovanni Pio de; Di Renzo, Nicola; Melpignano, Angela; Capalbo, Silvana; Palumbo, Gaetano; Pisapia, Giovanni; Cascavilla, Nicola


    Minimal residual disease (MRD) of 30 adult AML patients was monitored by multiparameter flow cytometry (MFC) and WT1 expression before and after allogeneic stem cell transplantation (allo-SCT). Diagnostic performance of pre-transplant MRD measured by MFC was higher than that obtained by WT1 expression. Comparable results were displayed at day +30 post-transplant, while better values by WT1 compared to MFC were found at day +90. Positive MRD by MFC predicted a shorter disease free survival (DFS) before and 1 month after transplant (p=0.006 and p=0.005), while only high WT1 levels at 1 month from the transplant significantly impacted on DFS (p=0.010). Our results support the idea that MRD monitoring by MFC should be suggested before and 30 days after the transplant, while WT1 expression should be preferred after this procedure. The assessment of MRD at day +30 from allo-SCT is recommended as post transplant check-point for the predictive role displayed, independently of the method used.

  11. Calcifying epithelial odontogenic tumor (Pindborg tumor) (United States)

    Singh, Neeraj; Sahai, Sharad; Singh, Sourav; Singh, Smita


    The calcifying epithelial odontogenic tumor (CEOT) is a rare entity and represents less than 1% of all odontogenic tumors. Dr. J J Pindborg (1958) first described four cases of this unusual lesion; subsequently Shafer et al coined the term Pindborg tumor. This lesion is a locally aggressive benign odontogenic neoplasm arising from epithelial tissue. It occurs most commonly in 4th-5th-6th decade of life and bears no gender predilection. A case of CEOT in a 50-year-old male arising in the left body region is described. PMID:22639521

  12. Tumor penetrating peptides

    Directory of Open Access Journals (Sweden)

    Tambet eTeesalu


    Full Text Available Tumor-homing peptides can be used to deliver drugs into tumors. Phage library screening in live mice has recently identified homing peptides that specifically recognize the endothelium of tumor vessels, extravasate, and penetrate deep into the extravascular tumor tissue. The prototypic peptide of this class, iRGD (CRGDKGPDC, contains the integrin-binding RGD motif. RGD mediates tumor homing through binding to αv integrins, which are selectively expressed on various cells in tumors, including tumor endothelial cells. The tumor-penetrating properties of iRGD are mediated by a second sequence motif, R/KXXR/K. This C-end Rule (or CendR motif is active only when the second basic residue is exposed at the C-terminus of the peptide. Proteolytic processing of iRGD in tumors activates the cryptic CendR motif, which then binds to neuropilin-1 activating an endocytic bulk transport pathway through tumor tissue. Phage screening has also yielded tumor-penetrating peptides that function like iRGD in activating the CendR pathway, but bind to a different primary receptor. Moreover, novel tumor-homing peptides can be constructed from tumor-homing motifs, CendR elements and protease cleavage sites. Pathologies other than tumors can be targeted with tissue-penetrating peptides, and the primary receptor can also be a vascular zip code of a normal tissue. The CendR technology provides a solution to a major problem in tumor therapy, poor penetration of drugs into tumors. The tumor-penetrating peptides are capable of taking a payload deep into tumor tissue in mice, and they also penetrate into human tumors ex vivo. Targeting with these peptides specifically increases the accumulation in tumors of a variety of drugs and contrast agents, such as doxorubicin, antibodies and nanoparticle-based compounds. Remarkably the drug to be targeted does not have to be coupled to the peptide; the bulk transport system activated by the peptide sweeps along any compound that is

  13. Tumors in invertebrates

    Directory of Open Access Journals (Sweden)

    F Tascedda


    Full Text Available Tumors are ectopic masses of tissue formed by due to an abnormal cell proliferation. In this review tumors of several invertebrate species are examined. The description of tumors in invertebrates may be a difficult task, because the pathologists are usually inexperienced with invertebrate tissues, and the experts in invertebrate biology are not familiar with the description of tumors. As a consequence, the terminology used in defining the tumor type is related to that used in mammalian pathology, which can create misunderstandings in some occasions.

  14. Benign ear cyst or tumor (United States)

    Osteomas; Exostoses; Tumor - ear; Cysts - ear; Ear cysts; Ear tumors; Bony tumor of the ear canal ... bony tumors of the ear canal (exostoses and osteomas) are caused by excess growth of bone. Repeated ...

  15. Cyst and tumor of liver

    Institute of Scientific and Technical Information of China (English)


    930145 Incidence of human hepatitis B virus(HBV)DNA sequence present in duck hepaticcancer collected from Qidong.JIANG Huiqiu(蒋惠秋),GU Jianren(顾建人).Nation Onco-gene & Relate Gene Lab.,Shanghai Cancer In-stir.Tumor 1992;12(5):206-207.Previous reports have described the identifica-tion and molecular cloning of human HBV DNAsequence in duck hepatoma collected from Qi-Dong.The human HBV-like DNA was se-quenced and it had been demonstrated that theHBV DNA present in duck hepatoma was nothomologous to DHBV,but highly homologous

  16. Tumor interstitial fluid

    DEFF Research Database (Denmark)

    Gromov, Pavel; Gromova, Irina; Olsen, Charlotta J.


    Tumor interstitial fluid (TIF) is a proximal fluid that, in addition to the set of blood soluble phase-borne proteins, holds a subset of aberrantly externalized components, mainly proteins, released by tumor cells and tumor microenvironment through various mechanisms, which include classical...... secretion, non-classical secretion, secretion via exosomes and membrane protein shedding. Consequently, the interstitial aqueous phase of solid tumors is a highly promising resource for the discovery of molecules associated with pathological changes in tissues. Firstly, it allows one to delve deeper...... into the regulatory mechanisms and functions of secretion-related processes in tumor development. Secondly, the anomalous secretion of molecules that is innate to tumors and the tumor microenvironment, being associated with cancer progression, offers a valuable source for biomarker discovery and possible targets...

  17. Epilepsy and Brain Tumors

    Institute of Scientific and Technical Information of China (English)

    Zhi-yi Sha


    @@ Epidemiology It is estimated 61,414 new cases of primary brain tumors are expected to be diagnosed in 2009 in the U.S. The incidence statistic of 61,414 persons diagnosed per year includes both malignant (22,738) and non-malignant (38,677) brain tumors. (Data from American Brain Tumor Association). During the years 2004-2005, approximately 359,000 people in the United States were living with the diagnosis of a primary brain or central nervous system tumor. Specifically, more than 81,000 persons were living with a malignant tumor, more than 267,000 persons with a benign tumor. For every 100,000 people in the United States, approximately 131 are living following the diagnosis of a brain tumor. This represents a prevalence rate of 130.8 per 100,000 person years[1].

  18. Genital soft tissue tumors. (United States)

    Schoolmeester, John K; Fritchie, Karen J


    Mesenchymal neoplasms of the vulvovaginal and inguinoscrotal regions are among the most diagnostically challenging specimens in the pathology laboratory owing largely to their unique intersection between general soft tissue tumors and relatively genital-specific mesenchymal tumors. Genital stromal tumors are a unique subset of soft tissue tumors encountered at this location, and this group includes fibroepithelial stromal polyp, superficial (cervicovaginal) myofibroblastoma, cellular angiofibroma, mammary-type myofibroblastoma, angiomyofibroblastoma and aggressive angiomyxoma. Aside from the striking morphologic and immunophenotypic similarity that is seen with these entities, there is evidence that a subset of genital stromal tumors may be linked genetically. This review will focus on simplifying this group of tumors and provide the pathologist or dermatopathologist with practical management information. Smooth muscle tumors of the external genitalia will also be discussed.

  19. Cryo-ablation improves anti-tumor immunity through recovering tumor educated dendritic cells in tumor-draining lymph nodes

    Directory of Open Access Journals (Sweden)

    He XZ


    Full Text Available Xiao-Zheng He,1,2 Qi-Fu Wang,1,2 Shuai Han,3 Hui-Qing Wang,1,2 Yong-Yi Ye,1,2 Zhi-Yuan Zhu,1,2 Shi-Zhong Zhang1,2 1Department of Neurosurgery, Zhujiang Hospital, Southern Medical University, Guangzhou, People’s Republic of China; 2The National Key Clinic Specialty, The Neurosurgery Institute of Guangdong Province, Guangdong Provincial Key Laboratory on Brain Function Repair and Regeneration, Southern Medical University, Guangzhou, People’s Republic of China; 3Department of General Surgery, Zhujiang Hospital, Southern Medical University, Guangzhou, People’s Republic of China Background: In addition to minimally invasive destruction of tumors, cryo-ablation of tumors to some extent modulated anti-tumor immunity. Cryo-ablated tumors in glioma mice models induced anti-tumor cellular immunologic response which increases the percentage of CD3+ and CD4+T cells in blood as well as natural killer cells. As a crucial role in triggering anti-tumor immunity, dendritic cells (DCs were educated by tumors to adopt a tolerance phenotype which helps the tumor escape from immune monitoring. This study aims to study whether cryo-ablation could influence the tolerogenic DCs, and influence anti-tumor immunity in tumor-draining lymph nodes (TDLNs. Methods: Using the GL261 subcutaneous glioma mouse model, we created a tumor bearing group, cryo-ablation group, and surgery group. We analyzed alteration in phenotype and function of tolerogenic DCs, and evaluated the factors of anti-tumor immunity inhibition. Results: DCs in TDLNs in GL261 subcutaneous glioma mouse model expressed tolerogenic phenotype. In contrast to surgery, cryo-ablation improved the quantity and quality of these tolerogenic DCs. Moreover, the DCs decreased the expression of intracellular interleukin-10 (IL-10 and extra-cellular IL-10. In vitro, DCs from the cryo-ablation group recovered their specific function and induced potent anti-tumor immunity through triggering T cells. In vivo, cryo

  20. Octreotide and Lanreotide in Gastroenteropancreatic Neuroendocrine Tumors. (United States)

    Pokuri, Venkata K; Fong, Mei Ka; Iyer, Renuka


    Neuroendocrine tumors are heterogeneous, rare malignancies that arise most commonly in the gastrointestinal tract and pancreas. They often secrete vasoactive substances resulting in carcinoid syndrome and the tumor cells exclusively express somatostatin receptors. Octreotide and lanreotide are the two synthetic somatostatin analogs used for the control of carcinoid symptoms and tumor progression in advanced inoperable disease. Recent pivotal trials (PROMID and CLARINET studies) established their antitumor activity. We discuss the available data to support their use as symptom controlling and antiproliferative agents. This article also reviews the guidelines (National Comprehensive Cancer Network and North American Neuro Endocrine Tumor Society), cost-analysis (suggesting the cost-effectiveness of lanreotide autogel compared to higher doses of octreotide long acting release formulation in refractory patients), and future directions of somatostatin analogs in the management of patients refractory to conventional doses of octreotide and lanreotide.

  1. Self-association of the WT1 tumor suppressor gene product

    Energy Technology Data Exchange (ETDEWEB)

    Bruening, W.; Nakagama, H.: Bardessy, N. [McGill Univ., Montreal (Canada)] [and others


    Wilms` tumor (WT), an embryonal malignancy of the kidney, occurs most frequently in children under the age of 5 years, affecting {approximately}1 in 10,000 individuals. The WT1 tumor suppressor gene, residing at 11p13, is structurally altered in {approximately}10-15% of WT cases. Individuals with germline mutations within the WT1 gene suffer from predisposition to WT and developmental defects of the urogenital system. Patients with heterozygous deletions of the WT1 gene, or mutations predicted to cause inactivation of one WT1 allele, suffer relatively mild genital system defects (notably hypospadias and cryptorchidism in males) and a predisposition to WT. These results suggest that developing genital system development is sensitive to the absolute concentrations of the WT1 gene products. Patients with missense mutations within the WT1 gene, however, can suffer from a much more severe disorder known as Denys-Drash syndrome (DDS). This syndrome is characterized by intersex disorders, renal nephropathy, and a predisposition to WTs. The increased severity of the developmental defects associated with DDS, compared to those individuals with mild genital system anomalies and WTs, suggests that mutations defined in patients with DDS behave in a dominant-negative fashion. We have identified a novel WT1 mutation in a patient with DDS. This mutation, predicted to produce a truncated WT1 polypeptide encompassing exons 1, 2, and 3, defines a domain capable of behaving as an antimorph. We have also demonstrated that WT1 can self-associate in vivo using yeast two-hybrid systems. Deletion analysis have mapped the interacting domains to the amino terminus of the WT1 polypeptide, within exons 1 and 2. These results provide a molecular mechanism to explain how WT1 mutations can function in a dominant-negative fashion to eliminate wild-type WT1 activity, leading to DDS.

  2. Tumor Cold Ischemia - Office of Cancer Clinical Proteomics Research (United States)

    In a recently published manuscript in the journal of Molecular and Cellular Proteomics, researchers from the National Cancer Institutes (NCI) Clinical Proteomic Tumor Analysis Consortium (CPTAC) investigated the effect of cold ischemia on the proteome of fresh frozen tumors.

  3. Targeted tumor radiotherapy

    Directory of Open Access Journals (Sweden)

    Unak Perihan


    Full Text Available Targeted tumor radiotherapy is selectively delivery of curative doses of radiation to malignant sites. The aim of the targeted tumor radiotherapy is to use the radionuclides which have high LET particle emissions conjugated to appropriate carrier molecules. The radionuclides are selectively collected by tumor cells, depositing lethal doses to tumor cells while no admission occur to normal cells. In theory, targeted radiotherapy has several advantages over conventional radiotherapy since it allows a high radiation dose to be administered without causing normal tissue toxicity, although there are some limitations in the availability of appropriate targeting agents and in the calculations of administered doses. Therefore, for routine clinical applications more progress is still needed. In this article, the potential use of targeted tumor radiotherapy is briefly reviewed. More general aspects and considerations, such as potential radionuclides, mechanisms of tumor targeting was also outlined.

  4. Uterine primitive neuroectodermal tumor. (United States)

    Aminimoghaddam, Soheila; Seifirad, Soroush; Abbasi Dezfouli, Golbahar; Abbasi, Neda; Zare Mehrjardi, Ali; Razavi, Seyed Mohsen; Mahmoudzadeh, Fatemeh


    Primitive neuroectodermal tumors are fairly rare in uterus. A case of uterine body primitive neuroectodermal tumor in a 32-year-old Iranian woman is presented. The patient was admitted with abdominal pain and fever and underwent emergency exploratory surgery with total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymph node dissection. Posterior wall of the uterus was necrotic and ruptured and a huge tumor disrupted the uterine body. The tumor was strongly positive for CD99, NSE, and chromogranin; No reaction was seen for CD10, CD45 and myogenin. To the best of our knowledge, this is the first report of an uterine body primitive neuroectodermal tumor and the second report of uterine primitive neuroectodermal tumor from Iran.

  5. Specificity for the tumor-associated self-antigen WT1 drives the development of fully functional memory T cells in the absence of vaccination. (United States)

    Pospori, Constandina; Xue, Shao-An; Holler, Angelika; Voisine, Cecile; Perro, Mario; King, Judith; Fallah-Arani, Farnaz; Flutter, Barry; Chakraverty, Ronjon; Stauss, Hans J; Morris, Emma C


    Recently, vaccines against the Wilms Tumor antigen 1 (WT1) have been tested in cancer patients. However, it is currently not known whether physiologic levels of WT1 expression in stem and progenitor cells of normal tissue result in the deletion or tolerance induction of WT1-specific T cells. Here, we used an human leukocyte antigen-transgenic murine model to study the fate of human leukocyte antigen class-I restricted, WT1-specific T cells in the thymus and in the periphery. Thymocytes expressing a WT1-specific T-cell receptor derived from high avidity human CD8 T cells were positively selected into the single-positive CD8 population. In the periphery, T cells specific for the WT1 antigen differentiated into CD44-high memory phenotype cells, whereas T cells specific for a non-self-viral antigen retained a CD44(low) naive phenotype. Only the WT1-specific T cells, but not the virus-specific T cells, displayed rapid antigen-specific effector function without prior vaccination. Despite long-term persistence of WT1-specific memory T cells, the animals did not develop autoimmunity, and the function of hematopoietic stem and progenitor cells was unimpaired. This is the first demonstration that specificity for a tumor-associated self-antigen may drive differentiation of functionally competent memory T cells.

  6. Dentinogenic ghost cell tumor

    Directory of Open Access Journals (Sweden)

    Singhaniya Shikha


    Full Text Available Dentinogenic ghost cell tumor (DGCT is a rare tumorous form of calcifying odontogenic cyst and only a small number of cases have been described. It is a locally invasive neoplasm that is characterized by ameloblastoma-like epithelial islands, ghost cells and dentinoid. The present report describes a case of a 21-year-old male with a tumor in the posterior region of the mandible, showing features of DGCT.

  7. Olfactory ensheathing cell tumor

    Directory of Open Access Journals (Sweden)

    Ippili Kaushal


    Full Text Available Olfactory ensheathing cells (OECs are found in the olfactory bulb and olfactory nasal mucosa. They resemble Schwann cells on light and electron microscopy, however, immunohistochemical staining can distinguish between the two. There are less than 30 cases of olfactory groove schwannomas reported in the literature while there is only one reported case of OEC tumor. We report an OEC tumor in a 42-year-old male and discuss the pathology and origin of this rare tumor.



    Tapia M.,Mauricio; Orellana H.,Ricardo; Cisterna C,Patricio; Gazitúa P,Raimundo; Sepúlveda A.,Rodrigo


    Objetivo: Evaluar la frecuencia de tumores anexiales en el embarazo, la histología tumoral y los resultados perinatales. Pacientes y método: Análisis retrospectivo de 33 pacientes con diagnóstico de tumor anexial y embarazo atendidas en el Servicio de Obstetricia del Hospital San Juan de Dios entre febrero de 2001 a julio de 2004. Resultados: La asociación tumor anexial y embarazo fue 1 en 424 embarazos. El tipo histológico más frecuente fue el cistoadenoma seroso (19,2%). La cirugía no alter...

  9. Tumor cell metabolism (United States)

    Romero-Garcia, Susana; Lopez-Gonzalez, Jose Sullivan; B´ez-Viveros, José Luis; Aguilar-Cazares, Dolores


    Cancer is a genetic disease that is caused by mutations in oncogenes, tumor suppressor genes and stability genes. The fact that the metabolism of tumor cells is altered has been known for many years. However, the mechanisms and consequences of metabolic reprogramming have just begun to be understood. In this review, an integral view of tumor cell metabolism is presented, showing how metabolic pathways are reprogrammed to satisfy tumor cell proliferation and survival requirements. In tumor cells, glycolysis is strongly enhanced to fulfill the high ATP demands of these cells; glucose carbons are the main building blocks in fatty acid and nucleotide biosynthesis. Glutaminolysis is also increased to satisfy NADPH regeneration, whereas glutamine carbons replenish the Krebs cycle, which produces metabolites that are constantly used for macromolecular biosynthesis. A characteristic feature of the tumor microenvironment is acidosis, which results from the local increase in lactic acid production by tumor cells. This phenomenon is attributed to the carbons from glutamine and glucose, which are also used for lactic acid production. Lactic acidosis also directs the metabolic reprogramming of tumor cells and serves as an additional selective pressure. Finally, we also discuss the role of mitochondria in supporting tumor cell metabolism. PMID:22057267

  10. Adenomatoid odontogenic tumor, an uncommon tumor

    Directory of Open Access Journals (Sweden)

    K Vasudevan


    Full Text Available Here we report a case of adenomatoid odontogenic tumor (AOT in the maxilla in a young girl aged 14 years and its surgical management. We also review the literature and variations in the nomenclature and classifications of this interesting tumor. The review of literature gives an interesting picture regarding terminologies in the past and dilemma in classifying this tumor. The introduction of the name adenomatoid odontogenic tumour has resulted in the simpler and fruitful surgical management like enucleation and curettage with no reports of recurrences. In the past, similar lesion with the terminology like adeno ameloblastoma has resulted in unnecessary mutilating surgery. The conflicting views whether the lesion is being neoplasm or an anomalous hamartomatous growth is also being discussed.

  11. Study of Fixed vs. Flexible Filgrastim to Accelerate Bone Marrow Recovery After Chemotherapy in Children With Cancer (United States)


    Childhood Choroid Plexus Tumor; Childhood Medulloblastoma; Childhood Pineoblastoma; Childhood Soft Tissue Sarcoma; Childhood Supratentorial Primitive Neuroectodermal Tumor; Neuroblastoma; Osteosarcoma; Retinoblastoma; Wilms Tumor and Other Childhood Kidney Tumors; Recurrent/Refractory Childhood Hodgkin Lymphoma; Unspecified Childhood Solid Tumor, Protocol Specific

  12. Study of Genistein in Pediatric Oncology Patients (UVA-Gen001) (United States)


    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

  13. 78 FR 50425 - Prospective Grant of Exclusive License: Development of Brachyury Tumor Associated Antigens as... (United States)


    ... Brachyury Tumor Associated Antigens as Cancer Vaccines for Colorectal Cancer AGENCY: National Institutes of... INFORMATION: Cancer immunotherapy is a recent approach where tumor associated antigens (TAAs), which are primarily expressed in human tumor cells and not expressed or minimally expressed in normal tissues, are...

  14. Renal inflammatory myofibroblastic tumor

    DEFF Research Database (Denmark)

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


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

  15. Atypically localized glomus tumors

    Directory of Open Access Journals (Sweden)

    Meric Ugurlar


    Conclusion: When a painful mass is found in the body, glomus tumors should be kept in mind. The consideration of symptoms, including pain, temperature sensitivity, point tenderness, and discoloration, common characteristics of glomus tumors, may aid diagnosis. [Hand Microsurg 2016; 5(3.000: 112-117

  16. Pseudoanaplastic tumors of bone

    Energy Technology Data Exchange (ETDEWEB)

    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)


    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.)

  17. Vanishing tumor in pregnancy

    Directory of Open Access Journals (Sweden)

    M V Vimal


    Full Text Available A patient with microprolactinoma, who had two successful pregnancies, is described for management issues. First pregnancy was uneventful. During the second pregnancy, the tumor enlarged to macroprolactinoma with headache and blurring of vision which was managed successfully with bromocriptine. Post delivery, complete disappearance of the tumor was documented.

  18. [Prognosis in tumor diagnosis]. (United States)

    Bruns, G


    This essay on prognosis in tumor diagnosis pathology resp. tumor etiology, cancerogenesis and molecular oncology is the authors personal opinion. Generally tumor diagnosis improves with progress in histo- and cytological methods for example in tumors of the APUD-system and precancerous lesions especially of the breast. Fundamental principle of developed tumor diagnosis is the knowledge of malignant transformation. Its profits favours the etiology in cancerogenesis and e.g. non Hodgkin lymphomas (Burkitt lymphoma, adult T-cell lymphoma) and to result from new methods in molecular biology and viral genetics (DNS-hybridizing, -recombination and gene technology). With the beginning century a stepwise fitted diagnosis of malignant lymphomas is evident up to monoclonal dedifferentiated lymphoid cells and their multifarious phenotypical markers. This concept may be of general significance in tumor diagnosis already indicated in prelymphomas. Finally the present prognosis of tumor diagnosis is evaluated by contents, tasks and strategies of its corresponding research lines in clinical and experimental tumor pathology resp. its organization.

  19. [Circulating "tumor markers" in gastrointestinal tumors]. (United States)

    Borlinghaus, P; Lamerz, R


    Tumor markers (TM) of the neoplastic cell can be divided into non-shedded substances and antigens shedded in blood, urine or other body fluids. For clinicians circulating TM are more important. All relevant circulating TM are not useful in screening of asymptomatic patients because of insufficient sensitivity and specificity. With caution they are useful in the observation of risk groups. Circulating TM have their main significance as additional parameters in monitoring symptomatic patients with malignancies. Several follow up determinations are more important than one single measurement. During follow up of tumor patients TM should not be checked automatically if there are no diagnostic or therapeutical consequences. The clinically most important circulating TM in non-hormone secreting tumors of the gastrointestinal tract are the oncofetal antigens CEA and AFP and antigens defined by monoclonal antibodies e. g. CA 19-9 and CA 72-4. AFP is the primary TM in hepatocellular carcinoma, often elevated in hepatoblastoma and always normal in cholangiocellular carcinoma. CEA is the TM of first choice in patients with colorectal carcinomas and liver metastasis. CA 19-9 is TM of first choice in pancreatic carcinoma and additionally of diagnostic value in cholangiocellular carcinoma and tumors of the bile ducts. In cancer of the stomach CA 19-9 and CEA are secondary TM in combination with CA 72-4 as primary TM. Care should be taken that slight and moderate elevations of TM can be observed in benign diseases of liver, pancreas and bowel.

  20. Pulmonary neuroendocrine (carcinoid) tumors

    DEFF Research Database (Denmark)

    Caplin, M E; Baudin, E; Ferolla, P


    carried out in PubMed for the terms 'pulmonary neuroendocrine tumors', 'bronchial neuroendocrine tumors', 'bronchial carcinoid tumors', 'pulmonary carcinoid', 'pulmonary typical/atypical carcinoid', and 'pulmonary carcinoid and diagnosis/treatment/epidemiology/prognosis'. A systematic review...... of the relevant literature was carried out, followed by expert review. RESULTS: PCs are well-differentiated neuroendocrine tumors and include low- and intermediate-grade malignant tumors, i.e. typical (TC) and atypical carcinoid (AC), respectively. Contrast CT scan is the diagnostic gold standard for PCs......, particularly of low-grade TC and AC. Locoregional or radiotargeted therapies should be considered for metastatic disease. Systemic chemotherapy is used for progressive PCs, although cytotoxic regimens have demonstrated limited effects with etoposide and platinum combination the most commonly used, however...

  1. Familial pituitary tumor syndromes. (United States)

    Elston, Marianne S; McDonald, Kerrie L; Clifton-Bligh, Roderick J; Robinson, Bruce G


    The vast majority of pituitary tumors are benign and occur sporadically; however, they can still result in significant morbidity and even premature mortality through mass effects and hormone dysfunction. The etiology of sporadic tumors is still poorly understood; by contrast, advances have been made in our understanding of familial pituitary adenoma syndromes in the past decade. Currently, four genes are known to be associated with familial pituitary tumor syndromes: MEN1, CDKN1B, PRKAR1A and AIP. The first three genes are associated with a variety of extrapituitary pathologies, for example, primary hyperparathyroidism with multiple endocrine neoplasia type 1, which might aid identification of these syndromes. By contrast, AIP mutations seem to occur in the setting of isolated familial pituitary adenomas, particularly of the growth-hormone-secreting subtype. Awareness and identification of familial pituitary tumor syndromes is important because of potential associated pathologies and important implications for family members. Here, we review the current knowledge of familial pituitary tumor syndromes.

  2. Merkel cell tumor. (United States)

    Kitazawa, M; Watanabe, H; Kobayashi, H; Ohnishi, Y; Shitara, A; Nitto, H


    A Merkel cell tumor appeared on the left cheek of an 83-year-old female was reported. The tumor was located mainly in the dermis and infiltrated to the subcutaneous adipose tissue with an involvement of the blood vessels and lymphatics at the periphery. Electron-microscopically, few of the dense-cored granules and the single globular aggregates of intermediate filaments at the nuclear indentations were observed. Electron-microscopic uranaffin reaction proved positive reaction on the dense-cored granules. Half of the cytoplasmic border was smooth, while the rest had short projections. Desmosomes or junctional complexes were not detected among the tumor cells. Immunohistochemically, the cytoplasm of tumor cell showed positive reaction to both neuron-specific enolase (NSE) and keratin. The single globular positive spots of the latter were localized in accordance with the aggregates of intermediate filaments. These findings suggested a neurogenic origin with double differentiation, epithelial and neuroendocrine, of the Merkel cell tumor.

  3. Gastrointestinal carcinoid tumors Tumores carcinoides digestivos

    Directory of Open Access Journals (Sweden)

    M. J. Varas Lorenzo


    Full Text Available Objective: carcinoid tumors (CTs represent the commonest neuroendocrine tumors. Those in the gastrointestinal tract are diagnosed in surgical specimens, clinically, and using imaging techniques (endoscopy, echoendoscopy, CT, Octreoscan, etc.. The goal of this retrospective study was to review a personal series of gastrointestinal carcinoid tumors, and to compare it to those in the literature. Patients and methods: the medical records of 40 Caucasian patients with over 50 gastrointestinal carcinoid tumors (including multiple cases who were seen for a period of 16 years (1994-2009 were reviewed. Results: mean age at presentation was 52 years, 50% were females, and mean tumor size was 9.9 mm. Most were gastroduodenal (42.5% or rectal (30%, and were treated endoscopically. Metastases and carcinoid syndrome (CS were seen in 5% of patients. Survival at study endpoint was 85%. Conclusions: age and gender were consistent with the literature. There was an increase in gastroduodenal (multifocal and rectal carcinoids, likely because the series was essentially endoscopical in nature (bias. There was a lower rate of CS and higher survival, likely due to earlier diagnosis and treatment.Objetivo: los tumores carcinoides (TC son los tumores neuroendocrinos más frecuentes. Los digestivos se diagnostican en las piezas quirúrgicas, en la clínica, y mediante los métodos de imagen (endoscopia, ecoendoscopia, TAC y Octreoscan, etc.. El objetivo de este trabajo retrospectivo fue revisar una serie personal de tumores carcinoides digestivos y compararla con la literatura. Pacientes y métodos: se revisaron las historias clínicas de 40 pacientes de raza blanca con más de 50 tumores carcinoides digestivos, algunos múltiples, observados durante 16 años (1994-2009. Resultados: la edad media de presentación fue 52 años, 50% mujeres, con un tamaño medio del tumor de 9,9 mm. La mayoría eran gastroduodenales (42,5% y rectales (30% y fueron tratados por vía endosc

  4. Parallel evolution of tumor subclones mimics diversity between tumors

    DEFF Research Database (Denmark)

    Martinez, Pierre; Birkbak, Nicolai Juul; Gerlinger, Marco


    Intratumor heterogeneity (ITH) may foster tumor adaptation and compromise the efficacy of personalized medicines approaches. The scale of heterogeneity within a tumor (intratumor heterogeneity) relative to genetic differences between tumors (intertumor heterogeneity) is unknown. To address this, ...

  5. Iatrogenic Tumor Implantation

    Institute of Scientific and Technical Information of China (English)

    Ying Ma; Ping Bai


    Iatrogenic tumor implantation is a condition that results from various medical procedures used during diagnosis or treatment of a malignancy. It involves desquamation and dissemination of tumor cells that develop into a local recurrence or distant metastasis from the tumor under treatment. The main clinical feature of the condition is nodules at the operation's porous channel or incision, which is easily diagnosed in accordance with the case history. Final diagnosis can be made based on pathological examination. Tumor implantation may occur in various puncturing porous channels, including a laparoscopic port, abdominal wall incision, and perineal incision, etc. Besides a malignant tumor,implantation potential exists with diseases, such as a borderline tumor and endometriosis etc. Once a tumor implantation is diagnosed, or suspected, surgical resection is usually conducted.During the diagnosis and treatment of diseases, avoiding and reducing iatrogenic implantation and dissemination has been regarded as an important principle for surgical treatment of tumors. In a clinical practice setting, if possible, excisional biopsy should be employed, if a biopsy is needed. Repeated puncturing should be avoided during a paracentesis. In a laparoscopic procedure, the tissue is first put into a sample bag and then is taken out from the point of incision. After a laparoscopic procedure, the peritoneum, abdominal muscular fasciae, and skin should be carefully closed, and/or the punctured porous channel be excised. In addition, the sample/tissue should be rinsed with distilled water before surgical closure of the abdominal cavity,allowing the exfoliated tumor cells to swell and rupture in the hypo-osmolar solution. Then surgical closure can be conducted following a change of gloves and equipment. The extent of hysteromyomectomy should as far as possible be away from the uterine cavity. The purpose of this study is to make clinicians aware of the possibility of tumor implantation

  6. Nationalism in Stateless Nations

    DEFF Research Database (Denmark)

    Thomsen, Robert Chr.

    democracies need not, as argued by theories of 'classic' nationalist movements, strive for full independence. In fact, nationalist movements are adapting to circumstances by becoming autonomist rather than separatist, pragmatic rather than dogmatic, and the book illustrates how Scotland and Newfoundland, both...... - intellectuals, political parties and the media - the book combines historical, sociological, political and media studies analyses in an interdisciplinary investigation, providing a comprehensive account of the waxing and waning of nationalism....

  7. The management of parotid tumors

    Institute of Scientific and Technical Information of China (English)

    Guang Yan Yu


    @@ The majority of salivary gland tumors are of epithelial origin. Parotid gland is the most common location of the tumors. Surgery is the main modality for the management of parotid tumors. Radiotherapy, chemotherapy etc are the auxiliary modalities.

  8. General Information about Pituitary Tumors (United States)

    ... Tumors Treatment Pituitary Tumors Treatment (PDQ®)–Patient Version General Information About Pituitary Tumors Go to Health Professional ... by many other glands in the body. Enlarge Anatomy of the inside of the brain, showing the ...

  9. Epilepsy and brain tumors (United States)



    Seizures are common in patients with brain tumors, and epilepsy can significantly impact patient quality of life. Therefore, a thorough understanding of rates and predictors of seizures, and the likelihood of seizure freedom after resection, is critical in the treatment of brain tumors. Among all tumor types, seizures are most common with glioneuronal tumors (70–80%), particularly in patients with frontotemporal or insular lesions. Seizures are also common in individuals with glioma, with the highest rates of epilepsy (60–75%) observed in patients with low-grade gliomas located in superficial cortical or insular regions. Approximately 20–50% of patients with meningioma and 20–35% of those with brain metastases also suffer from seizures. After tumor resection, approximately 60–90% are rendered seizure-free, with most favorable seizure outcomes seen in individuals with glioneuronal tumors. Gross total resection, earlier surgical therapy, and a lack of generalized seizures are common predictors of a favorable seizure outcome. With regard to anticonvulsant medication selection, evidence-based guidelines for the treatment of focal epilepsy should be followed, and individual patient factors should also be considered, including patient age, sex, organ dysfunction, comorbidity, or cotherapy. As concomitant chemotherapy commonly forms an essential part of glioma treatment, enzyme-inducing anticonvulsants should be avoided when possible. Seizure freedom is the ultimate goal in the treatment of brain tumor patients with epilepsy, given the adverse effects of seizures on quality of life. PMID:26948360

  10. Radiological and histopathological study of benign tumors of the mandible

    Energy Technology Data Exchange (ETDEWEB)

    Yoo, Seon Young; Baek, Seung Yon; Choi, Kyung Hee; Suh, Jeung Soo; Rhee, Chung Sik; Kim, Hee Seup [Ewha Womans University College of Medicine, Seoul (Korea, Republic of)


    Benign tumors of the mandible are uncommon lesions. That were classified into odontogenic tumors and nonodontogenic tumors. Author reviewed the radiological evaluation and pathological microscopic finding from 33 benign tumors of the mandible that were confirmed by the biopsy during last 10 years in Dental Clinics, Ewha Womans University Hospital and Seoul National University Dental Hospital. Following results were obtained; 1. Benign tumors of the mandible were classified into odontogenic (66.7%) and non-odontogenic tumors (33.3%). 2. The range of the age distribution was between 6 years and 67 years old. The commonest age group was the second decade (39.4%). 3. There was no difference to sex distribution. 4. The most frequent location was the body of the mandible (42.4%). 5. Radiographic findings were relatively characteristic in odontogenic tumors rather than non-odontogenic tumors. 1) Radiolucent cystic lesions-ameloblastoma, odontogenic myxoma, odontogenic fibtoma, aneurysmal bone cyst and neurofibroma. 2) Radiopaque mass lesions-odontoma, cementoma and osteoma. 3) Mixed patterns-ossifying fibroma, cementifying fibroma, calcifying odontogenic epithelial tumor and hemangioma. It was concluded that the radiographic examination was of value to diagnose the benign tumors of the mandible in symptomless patients.

  11. Laryngeal inflammatory myofibroblastic tumor. (United States)

    Girardi, Fábio M; Fontana, Ciro W; Kroef, Ricardo G; Barra, Marinez B; Detânico, Felipe O; Herter, Nilton T


    Inflammatory myofibroblastic tumor seldom involves the larynx, as only about 50 to 60 cases have been described in the literature. Even though these tumors are often not aggressive, they have the potential for invasion and local recurrence. We describe the case of a 27-year-old man who was admitted to an emergency department with signs of upper airway obstruction secondary to an obstructive mass. Histology identified the mass as an inflammatory myofibroblastic tumor of the subglottis. The patient underwent an emergency tracheotomy followed by a partial laryngectomy. During 14 months of follow-up, he remained free of active disease.

  12. Targeting the tumor microenvironment

    Energy Technology Data Exchange (ETDEWEB)

    Kenny, P.A.; Lee, G.Y.; Bissell, M.J.


    Despite some notable successes cancer remains, for the most part, a seemingly intractable problem. There is, however, a growing appreciation that targeting the tumor epithelium in isolation is not sufficient as there is an intricate mutually sustaining synergy between the tumor epithelial cells and their surrounding stroma. As the details of this dialogue emerge, new therapeutic targets have been proposed. The FDA has already approved drugs targeting microenvironmental components such as VEGF and aromatase and many more agents are in the pipeline. In this article, we describe some of the 'druggable' targets and processes within the tumor microenvironment and review the approaches being taken to disrupt these interactions.

  13. Uterine mesenchymal tumors

    Directory of Open Access Journals (Sweden)

    Nikhil A Sangle


    Full Text Available Uterine mesenchymal tumors are a heterogeneous group of neoplasms that can frequently be diagnostically challenging. Differentiation between the benign and malignant counterparts of mesenchymal tumors is significant due to differences in clinical outcome, and the role of the surgical pathologist in making this distinction (especially in the difficult cases cannot be underestimated. Although immunohistochemical stains are supportive toward establishing a final diagnosis, the morphologic features trump all the other ancillary techniques for this group of neoplasms. This review therefore emphasizes the key morphologic features required to diagnose and distinguish uterine mesenchymal tumors from their mimics, with a brief description of the relevant immunohistochemical features.


    Institute of Scientific and Technical Information of China (English)

    宋文哲; 宋燕; 叶剑桥; 邱东涛


    As a new member of IAP (inhibitors of apoptosis protein) family, survivin has potent anti-apoptotic activities, and involves in the mitosis and angiogenesis. Researches have demonstrated that surviving is a tumor-specific anti-apoptotic factor, expressed in fetal tissues, and common human cancers, while not in normal, terminally differentiated adult tissues. The overexpression of survivin in tumor tissues is correlated with poor prognosis of the patients. Survivin can be used as a prognostic factor and a new target in tumor targeting therapy.

  15. Myoepithelial Tumors: An Update. (United States)

    Jo, Vickie Y


    Primary myoepithelial neoplasms of soft tissue are uncommon, and have been increasingly characterized by clinicopathologic and genetic means. Tumors are classified as mixed tumor/chondroid syringoma, myoepithelioma, and myoepithelial carcinoma, and they share morphologic, immunophenotypic, and genetic features with their salivary gland counterparts. However, soft tissue myoepithelial tumors are classified as malignant based on the presence of cytologic atypia, in contrast to the criterion of invasive growth in salivary gland sites. This review discusses the clinicopathologic and morphologic characteristics, distinct variants, and currently known genetic alterations of myoepithelial neoplasms of soft tissue, skin, and bone.

  16. Tumor-Associated Macrophages and Neutrophils in Tumor Microenvironment

    Directory of Open Access Journals (Sweden)

    Jaehong Kim


    Full Text Available Distinct tumor microenvironment forms in each progression step of cancer and has diverse capacities to induce both adverse and beneficial consequences for tumorigenesis. It is now known that immune cells can be activated to favor tumor growth and progression, most probably influenced by the tumor microenvironment. Tumor-associated macrophages and tumor-associated neutrophils can exert protumoral functions, enhancing tumor cell invasion and metastasis, angiogenesis, and extracellular matrix remodeling, while inhibiting the antitumoral immune surveillance. Considering that neutrophils in inflammatory environments recruit macrophages and that recruited macrophages affect neutrophil functions, there may be various degrees of interaction between tumor-associated macrophages and tumor-associated neutrophils. Platelets also play an important role in the recruitment and regulation of monocytic and granulocytic cells in the tumor tissues, suggesting that platelet function may be essential for generation of tumor-associated macrophages and tumor-associated neutrophils. In this review, we will explore the biology of tumor-associated macrophages and tumor-associated neutrophils and their possible interactions in the tumor microenvironment. Special attention will be given to the recruitment and activation of these tumor-associated cells and to the roles they play in maintenance of the tumor microenvironment and progression of tumors.

  17. Características tumorais e sobrevida de cinco anos em pacientes com câncer de mama admitidas no Instituto Nacional de Câncer, Rio de Janeiro, Brasil Tumor characteristics and five-year survival in breast cancer patients at the National Cancer Institute, Rio de Janeiro, Brazil

    Directory of Open Access Journals (Sweden)

    Gulnar Azevedo e Silva Mendonça


    Full Text Available Vários fatores vêm sendo estudados com respeito ao estabelecimento de critérios mais seguros que influenciam o prognóstico de pacientes com câncer de mama. Este estudo teve como objetivo avaliar as taxas de sobrevida de cinco anos e os principais fatores prognósticos relativos ao tumor em mulheres com carcinoma invasivo de mama submetidas à cirurgia no Instituto Nacional de Câncer, Rio de Janeiro, Brasil, entre maio de 1995 e julho de 1996. As variáveis estudadas foram: presença de linfonodo comprometido, tamanho do tumor, grau de agressividade e presença de receptores hormonais para estrogênio e progesterona. As funções de sobrevida foram calculadas por meio do método de Kaplan-Meier. Foi utilizado o modelo de riscos proporcionais de Cox para avaliação dos fatores prognósticos. A taxa de sobrevida em cinco anos foi de 75,0% para todas as pacientes e, de 64,0% para as com metástase para linfonodo. A análise multivariada identificou o comprometimento de linfonodo como o mais forte preditor do desfecho; ter receptor positivo para estrogênio se associou a um melhor prognóstico. Esses resultados mostram a necessidade de condução de estudos que investiguem novos fatores que, combinados aos já conhecidos, possam melhor orientar a conduta terapêutica.Numerous factors have been studied to establish more secure prognostic criteria in breast cancer patients. This study estimates five-year survival rates and principal prognostic factors related to tumor characteristics in women with invasive breast cancer and submitted to surgery at the National Cancer Institute, Rio de Janeiro, Brazil, from May 1995 to July 1996. Study variables were: lymph node status, tumor size, aggressiveness grade, and presence of estrogen and progesterone receptors. Survival functions were calculated according to the Kaplan-Meyer method. The Cox proportional hazards model was used to evaluate prognostic factors. Five-year survival was 75% for all women and

  18. Primary hepatic carcinoid tumor

    Directory of Open Access Journals (Sweden)

    Gao Jinbo


    Full Text Available Abstract Primary hepatic carcinoid tumor is rare and poses a challenge for diagnosis and management. We presented a case of primary hepatic carcinoid tumor in a 53-year-old female with a complaint of right upper abdominal pain. Computer tomography scans revealed a hypervascular mass in segment 4 of the liver. An ultrasonography-guided biopsy showed a carcinoid tumor. No other lesions were found by the radiological investigations. Surgery resection was performed and histopathological examination revealed a primary hepatic carcinoid tumor. Three years later, recurrence was found and transcatheter arterial chemoembolization was performed. After transcatheter arterial chemoembolization, the patient has been free of symptom and had no radiological disease progression for over 6 months. Surgical resection combination with transcatheter arterial chemoembolization is effective to offer excellent palliation.

  19. Benign Liver Tumors (United States)

    ... A Life After Diagnosis Support for Chronic Illness Corporate Partnerships Interview with Kristen Hanks Liver Lowdown July ... Disease , Liver Transplant , Liver Cancer , Liver Tumor , Liver Failure Help Fight Liver Disease We rely upon donations ...


    African Journals Online (AJOL)

    biology and natural history of a high grade malig- nancy and a ... tumors arise in the gastrointestinal tract and collectively, they are ... jaundice, intra-abdominal mass and bleeding. Patients ... tion of the primary lesion and definition of the extent.

  1. [Radiation induced tumors]. (United States)

    Gutiérrez Bayard, L; Delgado López, L; Tirado Bejarano, C; Gómez Puerto, A; García Fernández, J L


    Radiations at cellular level produce different effects, depending on type of radiation and irradiated tissue. The radiation-induced cancers are associated to non-letals genetics mutations, and to classify like radiation induced tumors is necessary that appear in the treatment volume, a long latency period (years), histolo-different to the primary lesion, enough doses quantitatively and that exists a greater incidence in the irradiated populations. The genetics mutations affect at tumoral suppressors gen(Gen RB I, p53, BRCA I, BRCA 2) and repressors gen (hMSH 2, hMLH I,...), they could be longer and multifocals mutations, and produce lack of cellular control and a greater predisposition to develop tumors and a probable risk of increment of radiosensitivity. We present some of the more representatives studies about radiation-induced tumors.

  2. Nonislet Cell Tumor Hypoglycemia

    Directory of Open Access Journals (Sweden)

    Johnson Thomas


    Full Text Available Nonislet cell tumor hypoglycemia (NICTH is a rare cause of hypoglycemia. It is characterized by increased glucose utilization by tissues mediated by a tumor resulting in hypoglycemia. NICTH is usually seen in large mesenchymal tumors including tumors involving the GI tract. Here we will discuss a case, its pathophysiology, and recent advances in the management of NICTH. Our patient was diagnosed with poorly differentiated squamous cell carcinoma of esophagus. He continued to be hypoglycemic even after starting continuous tube feeds and D5W. General workup for hypoglycemia was negative and insulin-like growth factor II (IGF II was in the normal range. Hypoglycemia secondary to “big” IGF II was considered, and patient was started on steroids. His hypoglycemia resolved within a day of treatment with steroids. Initially patient had hypoglycemia unawareness, which he regained after maintaining euglycemia for 48 hours.

  3. Osteochondroma (Bone Tumor) (United States)

    ... to be the most common benign bone tumor, accounting for 35% to 40% of all benign bone ... imaging scans. Doctors may also request computed tomography (CT) scans or magnetic resonance imaging (MRI) scans to ...

  4. Brain Tumors and Fatigue (United States)

    ... can help calm the mind. Meditation, guided imagery, music therapy, and yoga are just a few worth investigating. Home Donor and Privacy Policies Find Resources Disclaimer Donate Subscribe Login American Brain Tumor Association 8550 W. Bryn Mawr Ave. Ste ...

  5. [Metastatic bronchial carcinoid tumors]. (United States)

    Bouledrak, K; Walter, T; Souquet, P J; Lombard-Bohas, C


    Bronchial carcinoids are uncommon pulmonary neoplasms and represent 1 to 2 % of all lung tumors. In early stage of disease, the mainstay and only curative treatment is surgery. Bronchial carcinoids are generally regarded as low-grade carcinomas and metastatic dissemination is unusual. The management of the metastatic stage is not currently standardized due to a lack of relevant studies. As bronchial carcinoids and in particular their metastatic forms are rare, we apply treatment strategies that have been evaluated in gastrointestinal and pancreatic neuroendocrine tumors. However, bronchial carcinoids have their own characteristic. A specific therapeutic feature of these metastatic tumors is that they require a dual approach: both anti-secretory for the carcinoid syndrome, and anti-tumoral.

  6. Pituitary tumors containing cholecystokinin

    DEFF Research Database (Denmark)

    Rehfeld, J F; Lindholm, J; Andersen, B N


    We found small amounts of cholecystokinin in the normal human adenohypophysis and therefore examined pituitary tumors from 87 patients with acromegaly, Cushing's disease, Nelson's syndrome, prolactinoma, or inactive pituitary adenomas. Five adenomas associated with Nelson's syndrome contained...

  7. Overview of Heart Tumors (United States)

    ... develop in the sac that surrounds the heart (pericardium). Tumors in the pericardium may squeeze (constrict) the heart, preventing it from ... Heart wall Fibroma Hemangioma Rhabdomyoma Outside surface Lipoma Pericardium (outer sac covering heart) Pericardial cyst Base of ...


    Directory of Open Access Journals (Sweden)

    Luigi eTornillo


    Full Text Available Gastrointestinal stromal tumors are the most frequent mesenchymal tumors of the gastrointestinal tract. The discovery that these tumors, formerly thought of smooth muscle origin, are indeed better characterized by specific activating mutation in genes coding for the receptor tyrosine kinases CKIT and PDGFRA and that these mutations are strongly predictive for the response to targeted therapy with receptor tyrosine kinase inhibitors has made GISTs the typical example of the integration of basic molecular knowledge in the daily clinical activity. The information on the mutational status of these tumors is essential to predict (and subsequently to plan the therapy. As resistant cases are frequently wild-type, other possible oncogenic events, defining other entities, have been discovered (e.g. succinil dehydrogenase mutation/dysregulation, insuline growth factor expression, mutations in the RAS-RAF-MAPK pathway. The classification of disease must nowadays rely on the integration of the clinico-morphological characteristics with the molecular data.

  9. Brain Tumor Surgery (United States)

    ... Meningitis Brain swelling Stroke Excess fluid in the brain Coma Death Recovery Time Recovery time depends on: The procedure performed. The part of the brain where the tumor is/was located. The areas ...

  10. Scotland: Nation without Nationalism?

    Directory of Open Access Journals (Sweden)

    Ya. I. Maltsev


    Full Text Available In the period before 1990s two concepts were used to describe the Scottish political development: “Nation without nationalism” and “The stateless nation”. After the devolution of 1998 and electoral achievements of SNP neither of these constructions can properly characterize the processes, undergoing in Scotland. The forming of new alignment of political powers in Scotland is not yet finished, but so far its development leads to the emergence of political configuration separated from that of All-British, with regard to extreme weakening of “big” parties in Scotland. 

  11. Tracheal and bronchial tumors. (United States)

    Varela, Patricio; Pio, Luca; Brandigi, Elisa; Paraboschi, Irene; Khen-Dunlop, Nazhia; Hervieux, Erik; Muller, Cecile; Mattioli, Girolamo; Sarnacki, Sabine; Torre, Michele


    Although primary tracheobronchial tumors are extremely rare in children, recurrent respiratory symptoms resistant to conventional therapy require further investigations to exclude possible malignant obstructive causes. As the matter of fact, early diagnosis may allow minimally invasive surgeries, improving the standard of living and the globally survival rate. The aim of this article is to provide an overview of diagnosis and management of tracheobronchial tumors in the early age, since only few reports are reported in the worldwide literature.

  12. Perlecan and tumor angiogenesis

    DEFF Research Database (Denmark)

    Jiang, Xinnong; Couchman, John R


    Perlecan is a major heparan sulfate proteoglycan (HSPG) of basement membranes (BMs) and connective tissues. The core protein of perlecan is divided into five domains based on sequence homology to other known proteins. Commonly, the N-terminal domain I of mammalian perlecan is substituted with thr...... have unwanted promoting effects on tumor cell proliferation and tumor angiogenesis. Understanding of these attributes at the molecular level may offer opportunities for therapeutic intervention....

  13. Pituitary tumors containing cholecystokinin

    DEFF Research Database (Denmark)

    Rehfeld, J F; Lindholm, J; Andersen, B N


    We found small amounts of cholecystokinin in the normal human adenohypophysis and therefore examined pituitary tumors from 87 patients with acromegaly, Cushing's disease, Nelson's syndrome, prolactinoma, or inactive pituitary adenomas. Five adenomas associated with Nelson's syndrome contained......'s disease and 7 acromegaly with adenomas containing ACTH. The cholecystokinin peptides from the tumors were smaller and less sulfated than cholecystokinin from normal pituitary glands. We conclude that ACTH-producing pituitary cells may also produce an altered form of cholecystokinin....

  14. Adrenocortical tumors in children

    Directory of Open Access Journals (Sweden)

    R.C. Ribeiro


    Full Text Available Childhood adrenocortical tumors (ACT are rare. In the USA, only about 25 new cases occur each year. In Southern Brazil, however, approximately 10 times that many cases are diagnosed each year. Most cases occur in the contiguous states of São Paulo and Paraná. The cause of this higher rate has not been identified. Familial genetic predisposition to cancer (p53 mutations and selected genetic syndromes (Beckwith-Wiedemann syndrome have been associated with childhood ACT in general but not with the Brazilian counterpart. Most of the affected children are young girls with classic endocrine syndromes (virilizing and/or Cushing. Levels of urinary 17-ketosteroids and plasma dehydroepiandrosterone sulfate (DHEA-S, which are abnormal in approximately 90% of the cases, provide the pivotal clue to a diagnosis of ACT. Typical imaging findings of pediatric ACT consist of a large, well-defined suprarenal tumor containing calcifications with a thin capsule and central necrosis or hemorrhage. The pathologic classification of pediatric ACT is troublesome. Even an experienced pathologist can find it difficult to differentiate carcinoma from adenoma. Surgery is the single most important procedure in the successful treatment of ACT. The role of chemotherapy in the management of childhood ACT has not been established although occasional tumors are responsive to mitotane or cisplatin-containing regimens. Because of the heterogeneity and rarity of the disease, prognostic factors have been difficult to establish in pediatric ACT. Patients with incomplete tumor resection or with metastatic disease at diagnosis have a dismal prognosis. In patients with localized and completely resected tumors, the size of the tumor has predictive value. Patients with large tumors have a much higher relapse rate than those with small tumors.

  15. Tumores cartilaginosos da laringe

    Directory of Open Access Journals (Sweden)

    Thomé Robert


    Full Text Available Introdução: Os tumores cartilaginosos da laringe são raros, sendo a cartilagem cricóide a mais acometida. A raridade desses tumores tem como conseqüência experiência limitada e, portanto, o conhecimento a respeito destas neoplasias apresenta pontos obscuros. Objetivo: Apresentar os resultados cirúrgicos bem como o seguimento a longo prazo de 6 pacientes com tumores cartilaginosos da laringe, 4 condrossarcomas de baixa malignidade e 2 condromas. Forma de estudo: Retrospectivo não randomizado. Material e método: Cinco dos tumores acometiam a cartilagem cricóide e 1 a tireóide. Os pacientes foram submetidos a laringectomia parcial (4 e total (2, com seguimento de 6 a 30 anos (média-19,5 anos. Resultados: Nenhum dos pacientes apresentou metástase ou morte relacionada ao tumor. A sobrevida alcançada por 5 anos foi de 100% e a por 20 anos, 67%, sendo a comorbidade responsável por tal decréscimo. Conclusões: Nossos resultados reforçam o conceito de que a laringectomia conservadora seja a modalidade ideal de tratamento, reservando a ressecção total para tumores extensos ou casos de recorrência. Há indícios de que 5 anos pode não ser tempo suficiente para observar recorrência no condrossarcoma de baixo grau de malignidade.

  16. Giant Intradiverticular Bladder Tumor (United States)

    Noh, Mohamad Syafeeq Faeez Md; Aziz, Ahmad Fuad Abdul; Ghani, Khairul Asri Mohd; Siang, Christopher Lee Kheng; Yunus, Rosna; Yusof, Mubarak Mohd


    Patient: Male, 74 Final Diagnosis: Giant intradiverticular bladder tumor with metastasis Symptoms: Hematuria Medication:— Clinical Procedure: — Specialty: Urology Objective: Rare disease Background: Intradiverticular bladder tumors are rare. This renders diagnosis of an intradiverticular bladder tumor difficult. Imaging plays a vital role in achieving the diagnosis, and subsequently staging of the disease. Case Report: A 74-year-old male presented to our center with a few months history of constitutional symptoms. Upon further history, he reported hematuria two months prior to presentation, which stopped temporarily, only to recur a few days prior to coming to the hospital. The patient admitted to having lower urinary tract symptoms. However, there was no dysuria, no sandy urine, and no fever. Palpation of his abdomen revealed a vague mass at the suprapubic region, which was non tender. In view of his history and the clinical examination findings, an ultrasound of the abdomen and computed tomography (CT) was arranged. These investigations revealed a giant tumor that seemed to be arising from a bladder diverticulum, with a mass effect and hydronephrosis. He later underwent operative intervention. Conclusions: Intradiverticular bladder tumors may present a challenge to the treating physician in an atypical presentation; thus requiring a high index of suspicion and knowledge of tumor pathophysiology. As illustrated in our case, CT with its wide availability and multiplanar imaging capabilities offers a useful means for diagnosis, disease staging, operative planning, and follow-up. PMID:28246375

  17. [Enophthalmos in an orbital tumor]. (United States)

    Szabo, Bianca; Szabo, I; Nicula, Cristina; Popescu, Livia Adriana


    Enophtalmus is an unusual sign of the orbital tumors often represented by proptosis. One patient with enophtalmus and intraorbital tumor and aplasy is presented. The treatment of choice of orbital tumor is complete surgical excision and careful follow-up. Considering the more aggressive course followed by recurrent tumor, correct diagnosis and management is essential.

  18. Brain tumors in infants

    Directory of Open Access Journals (Sweden)

    Seyyed Mohammad Ghodsi


    Full Text Available Background: Brain tumors in infants have different clinical presentations, anatomical distribution, histopathological diagnosis, and clinical prognosis compared with older children. Materials and Methods: A retrospective analysis was done in patients <12 months old who were operated on for primary brain tumor in Children's Hospital Medical Center since 2008 to 2014. Results: Thirty-one infants, 20 males and 11 females, with the mean age of 7.13 months (0.5–12 were enrolled. There were 16 supratentorial and 15 infratentorial tumors. The presenting symptoms included increased head circumference (16; bulge fontanel (15; vomiting (15; developmental regression (11; sunset eye (7; seizure (4; loss of consciousness (4; irritability (3; nystagmus (2; visual loss (2; hemiparesis (2; torticollis (2; VI palsy (3; VII, IX, X nerve palsy (each 2; and ptosis (1. Gross total and subtotal resection were performed in 19 and 11 cases, respectively. Fourteen patients needed external ventricular drainage in the perioperative period, from whom four infants required a ventriculoperitoneal shunt. One patient underwent ventriculoperitoneal shunting without tumor resection. The most common histological diagnoses were primitive neuroectodermal tumor (7, followed by anaplastic ependymoma (6 and grade II ependymoma. The rate of 30-day mortality was 19.3%. Eighteen patients are now well-controlled with or without adjuvant therapy (overall survival; 58%, from whom 13 cases are tumor free (disease free survival; 41.9%, 3 cases have residual masses with fixed or decreased size (progression-free survival; 9.6%, and 2 cases are still on chemotherapy. Conclusion: Brain tumors in infants should be treated with surgical resection, followed by chemotherapy when necessary.

  19. Metastatic Brain Tumors

    Directory of Open Access Journals (Sweden)

    Ersin Haciyakupoglu


    Full Text Available Metastatic tumor is secondary spread to the central nervous system of primer systemic cancers originating from tissues other than the central nervous system. In adults; there are metastases respectively from lungs, breasts, malign melanoma, renal cell carcinoma, colon and thyroid cancers. 30-60% of lung cancers metastasis to the brain. In children there are quite a few cerebral metastases. Most commonly leukemia, lymphoma, osteogenic sarcoma, rhabdomyosarcoma and germ cell tumors metastasis to the brain. %50 of malign melanoma, lung, breast and colon cancers intend to make multipl metastases but renal cell cancers intend to make solitary metastasis.While lung cancers metastasis to brain in 6-9 months after the definitive diagnosis, renal cancers in 1 year, colon cancers in 2 years, breast cancers and malign melanoma in 3 years metastasis to brain. In 6% of cases there are cerebral metastasis while there isn’t a symptom of a primary tumor. For treatment corticosteroids, surgery, Radiotherapy(RT, Chemotherapy(CT and Stereotactic Radiosurgery(SRS can be implemented. Small cell lung cancers, lymphoma, germ cell tumors are sensitive to RT and CT. Non small cell lung cancers, renal, colon cancers and malign melanoma are radioresistant. The purposes in the surgery of the metastatic brain tumors are; total resection of tumors without neurologic deficits, decreasing the intracranial pressure and decreasing the dose of postoperative radiotherapy. Key Words: Metastatic brain tumors, Stereotactic radiosurgery, Malign melanoma, Lung cancers, Renal cell carcinoma, Radiotherapy, Chemotherapy [Cukurova Med J 2014; 39(2.000: 191-202

  20. Tumores neonatales bucomaxilofaciales Neonatal buccomaxillofacial tumors

    Directory of Open Access Journals (Sweden)

    Zoila del S. López Díaz


    Full Text Available Se realiza un estudio descriptivo, lineal y retrospectivo por un período de 10 años, de 11 recién nacidos (edad 0-30 días, quienes al nacer presentan en la región bucomaxilofacial un tumor que les ocasiona de manera determinante compromiso para la ventilación y/o alimentación, por lo que se hace necesario realizarles a todos de manera inmediata, tratamiento quirúrgico para preservarles la vida. Se analizaron las variables edad, sexo, color de la piel, diagnóstico, tumoraciones que se presentaron con mayor frecuencia, compromiso para la ventilación y/o alimentación, procederes y mortalidad. Los datos se recogieron en una planilla confeccionada al efecto, lo que permitió establecer resultados y confeccionar tablas. Se concluye que en nuestro estudio este tipo de tumoración afectó con mayor frecuencia al sexo femenino y a niños de piel blanca; y el tipo de tumoración observada con mayor frecuencia fueron las malformaciones vasculares de tipo linfático (linfangiomas gigantes o higromas quísticos, así como y los teratomas bucofaríngeos, con una mortalidad de alrededor del 27,3 % en estas edades neonatales.A descriptive, lineal and retrospective study of 11 newborn infants aged 0-30 days was conducted. They presented a tumor in the buccomaxillofacial region that compromised their ventilation and/or nutrition, which made necessary to immediately perform surgery to preserve their lives. The following variables were analyzed: age, sex, colour of the skin, diagnosis, the most common tumours, compromise for ventilation and/or nutrition, procedures and mortality. Data were collected in a form that allowed to establish results and to make tables. It was concluded that this type of tumor affected mostly females and white children. The most commonly observed tumors were vascular lymphatic malformations (giant lymphangiomas or cystic hygromas, as well as buccopharyngeal teratomas, with a mortality around 27.3 % at these neonatal ages.

  1. Pituitary gland tumors; Hypophysentumoren

    Energy Technology Data Exchange (ETDEWEB)

    Jesser, J.; Schlamp, K.; Bendszus, M. [Radiologische Klinik, Universitaetsklinikum Heidelberg, Abteilung fuer Neuroradiologie, Heidelberg (Germany)


    This article gives an overview of the most common tumors of the pituitary gland and the differential diagnostics with special emphasis on radiological diagnostic criteria. A selective search of the literature in PubMed was carried out. Pituitary adenomas constitute 10-15 % of all intracranial tumors and are the most common tumors of the sellar region. Tumors smaller than 1 cm in diameter are called microadenomas while those larger than 1 cm in diameter are called macroadenomas. Approximately 65 % of pituitary gland adenomas secrete hormones whereby approximately 50 % secrete prolactin, 10 % secrete growth hormone (somatotropin) and 6 % secrete corticotropin. Other tumors located in the sella turcica can also cause endocrinological symptoms, such as an oversecretion of pituitary hormone or pituitary insufficiency by impinging on the pituitary gland or its stalk. When tumors spread into the space cranial to the sella turcica, they can impinge on the optic chiasm and cause visual disorders. A common differential diagnosis of a sellar tumor is a craniopharyngeoma. In children up to 10 % of all intracranial tumors are craniopharyngeomas. Other differential diagnoses for sellar tumors are metastases, meningiomas, epidermoids and in rare cases astrocytomas, germinomas or Rathke cleft cysts As these tumors are located in an anatomically complex region of the skull base and are often very small, a highly focused imaging protocol is required. The currently favored modality is magnetic resonance imaging (MRI) with the administration of a contrast agent. The sellar region should be mapped in thin slices. In cases of suspected microadenoma the imaging protocol should also contain a sequence with dynamic contrast administration in order to assess the specific enhancement characteristics of the tumor and the pituitary gland. (orig.) [German] Diese Arbeit ist eine Uebersicht ueber die haeufigsten Hypophysentumoren und deren Differenzialdiagnosen mit Augenmerk auf die

  2. CNS Tumors in Neurofibromatosis. (United States)

    Campian, Jian; Gutmann, David H


    Neurofibromatosis (NF) encompasses a group of distinct genetic disorders in which affected children and adults are prone to the development of benign and malignant tumors of the nervous system. The purpose of this review is to discuss the spectrum of CNS tumors arising in individuals with NF type 1 (NF1) and NF type 2 (NF2), their pathogenic etiologies, and the rational treatment options for people with these neoplasms. This article is a review of preclinical and clinical data focused on the treatment of the most common CNS tumors encountered in children and adults with NF1 and NF2. Although children with NF1 are at risk for developing low-grade gliomas of the optic pathway and brainstem, individuals with NF2 typically manifest low-grade tumors affecting the cranial nerves (vestibular schwannomas), meninges (meningiomas), and spinal cord (ependymomas). With the identification of the NF1 and NF2 genes, molecularly targeted therapies are beginning to emerge, as a result of a deeper understanding of the mechanisms underlying NF1 and NF2 protein function. As we enter into an era of precision oncology, a more comprehensive awareness of the factors that increase the risk of developing CNS cancers in affected individuals, coupled with a greater appreciation of the cellular and molecular determinants that maintain tumor growth, will undoubtedly yield more effective therapies for these cancer predisposition syndromes.

  3. Extraovarian granulosa cell tumor

    Directory of Open Access Journals (Sweden)

    Paul Prabir


    Full Text Available Extraovarian granulosa cell tumor (GCT is a very uncommon tumor, assumed to arise from the ectopic gonadal tissue along the embryonal route of the genital ridge. One such rare case of extraovarian GCT was encountered in a 58-year-old female who presented with a large intraabdominal lump. Computerized tomography revealed one large retroperitoneal mass measuring 15cm x 16cm and another mesenteric mass of 8cm x 5cm size. The patient had a history of hysterectomy with bilateral salpingooophorectomy 20 years ago for uterine leiomyoma. Ultrasonography-guided aspiration smears revealed cytological features suggestive of GCT. Histopathological examination of the excised masses showed features of adult-type GCT. Because metastatic epithelial tumors, particularly from the ovaries, may show identical morphology, immunostains for inhibin and epithelial membrane antigen (EMA were performed. The tumor showed positivity for inhibin while EMA was negative thus confirming the diagnosis of GCT. As this patient had no previous history of GCT and was oophorectomized 20 years ago, the tumor was considered as extraovarian. A diagnosis of extraovarian GCT should be carried out after excluding any previous history of GCT of the ovary. Immunostains help to differentiate GCTs from other neoplasms.

  4. Application of Adoptive T-Cell Therapy Using Tumor Antigen-Specific T-Cell Receptor Gene Transfer for the Treatment of Human Leukemia

    Directory of Open Access Journals (Sweden)

    Toshiki Ochi


    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.

  5. Endolymphatic sac tumor : a rare cerebellopontine angle tumor.

    Directory of Open Access Journals (Sweden)

    Joseph B


    Full Text Available Endolymphatic sac tumors (ELST are rare papillary tumors of the temporal bone. Previously named as aggressive papillary middle ear tumors, they have recently been shown to arise from the endolymphatic sac. They are a rare in cerebello-pontine angle (CPA. We present a case of an ELST who presented as a CPA tumor with hydrocephalus. He underwent a ventriculo-peritoneal shunt initially. On exploration of the CP angle, the tumor was found to be extremely vascular. He was re-explored following embolization, and a subtotal excision of the tumor was done. Extensive petrous bone infiltration and vascularity of the tumor makes total excision almost impossible with high risk of cranial nerve deficits, excessive blood loss and CSF leak. This tumor should be considered in the differential diagnosis of vascular CPA tumors which erode the petrous temporal bone. The relevant literature is reviewed.

  6. Research Progress of DC Tumor Vaccine Targeted at WT1 Gene%WT1为靶点的DC肿瘤疫苗研究进展

    Institute of Scientific and Technical Information of China (English)



    WT1基因在小儿肾癌中首次被发现,随后的研究中大量数据显示WT1基因在血液系统肿瘤和实体瘤中存在高表达,这提示WT1作为肿瘤免疫治疗新靶点的可行性.树突状细胞(DC)在诱导肿瘤抗原特异性效应和记忆性细胞过程中的作用,以及在人体内呈递抗原的能力,使其成为肿瘤免疫治疗的基础.随着目前DC研究的不断进步,以WT1为靶点的DC肿瘤疫苗也日趋成熟,有的已成功应用于临床.%Wilms tumor gene 1( WT1 gene )was firstly found in child kidney cancer. Evidence has accumulated over the past decade to show that WT1 has high expression in acute leukaemias and solid tumor, which indicates it a suitable target for therapeutic strategies. Dendritic cells( DC )became the base of immune therapy, because of its specific effect in inducing tumor antigen and memory cellular processes, and its ability to present antigen in the human body. Along with the current research progress of DCs, DC cancer vaccine targeting at WT1 is becoming more and more mature, some of which has been used in clinic.

  7. Upper urinary tract tumors

    DEFF Research Database (Denmark)

    Gandrup, Karen L; Nordling, Jørgen; Balslev, Ingegerd


    BACKGROUND: Computed tomography urography (CTU) is used widely in the work-up of patients with symptoms of urinary tract lesions. Preoperative knowledge of whether a tumor is invasive or non-invasive is important for the choice of surgery. So far there are no studies about the distinction...... of invasive and non-invasive tumors in ureter and renal pelvis based on the enhancement measured with Hounsfield Units. PURPOSE: To examine the value of CTU using split-bolus technique to distinguish non-invasive from invasive urothelial carcinomas in the upper urinary tract. MATERIAL AND METHODS: Patients...... obtained at CTU could distinguish between invasive and non-invasive lesions. No patients had a CTU within the last year before the examination that resulted in surgery. CONCLUSION: A split-bolus CTU cannot distinguish between invasive and non-invasive urothelial tumors in the upper urinary tract...

  8. Benign notochordal cell tumors. (United States)

    Martínez Gamarra, C; Bernabéu Taboada, D; Pozo Kreilinger, J J; Tapia Viñé, M


    Benign notochordal cell tumors (TBCN) are lesions with notochordal differentiation which affect the axial skeleton. They are characterized by asymptomatic or non-specific symptomatology and are radiologically unnoticed because of their small size, or because they are mistaken with other benign bone lesions, such as vertebral hemangiomas. When they are large, or symptomatic, can be differential diagnosis with metastases, primary bone tumors and chordomas. We present a case of a TBCN in a 50-year-old woman, with a sacral lesion seen in MRI. A CT-guided biopsy was scheduled to analyze the lesion, finding that the tumor was not clearly recognizable on CT, so the anatomical references of MRI were used to select the appropriate plane. The planning of the approach and the radio-pathological correlation were determinant to reach the definitive diagnosis. Copyright © 2017 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

  9. Renal Tumor Biopsy Technique

    Institute of Scientific and Technical Information of China (English)

    Lei Zhang; Xue-Song Li; Li-Qun Zhou


    Objective:To review hot issues and future direction of renal tumor biopsy (RTB) technique.Data Sources:The literature concerning or including RTB technique in English was collected from PubMed published from 1990 to 2015.Study Selection:We included all the relevant articles on RTB technique in English,with no limitation of study design.Results:Computed tomography and ultrasound were usually used for guiding RTB with respective advantages.Core biopsy is more preferred over fine needle aspiration because of superior accuracy.A minimum of two good-quality cores for a single renal tumor is generally accepted.The use of coaxial guide is recommended.For biopsy location,sampling different regions including central and peripheral biopsies are recommended.Conclusion:In spite of some limitations,RTB technique is relatively mature to help optimize the treatment of renal tumors.

  10. Risk of Local Recurrence of Benign and Borderline Phyllodes Tumors

    DEFF Research Database (Denmark)

    Borhani-Khomani, Kaveh; Talman, Maj-Lis Møller; Kroman, Niels


    PURPOSE: To determine the recurrence rate of benign and borderline phyllodes tumors (PTs) of the breast, the association between the size of resection margin and risk of recurrence and the risk of progression of histological grading at recurrence. METHODS: Nationwide retrospective study on Danish...... in histological grading was found. The results do not justify wide excision margins of nonmalignant phyllodes tumors of the breast....... women aged 18 years or older, operated from 1999 to 2014, with resected benign or borderline PTs. Information on age, size of primary tumor and recurrence, histological grade, surgical treatment, margin size, and local recurrence were collected from the national Danish Pathology Register. RESULTS...

  11. Complications - National (United States)

    U.S. Department of Health & Human Services — Complications - national data. This data set includes national-level data the hip/knee complication measure, and the Agency for Healthcare Research and Quality...

  12. HCAHPS - National (United States)

    U.S. Department of Health & Human Services — The national average for the HCAHPS survey categories. HCAHPS is a national, standardized survey of hospital patients about their experiences during a recent...

  13. Payment - National (United States)

    U.S. Department of Health & Human Services — Payment measures – national data. This data set includes national-level data for the payment measures associated with an episode of care for heart attack, heart...

  14. Aggressive malignant phyllodes tumor

    Directory of Open Access Journals (Sweden)

    Nathan Roberts


    Conclusion: Despite biopsy proven malignant phyllodes tumor, it was near impossible to predict such a rapid course of disease progression in our patient. Our case illustrates the unpredictable nature of this disease in general and it possibly sheds light on a variant of the disease which had undergone an aggressive transformation.

  15. Ghost Cell Tumors. (United States)

    Sheikh, Jason; Cohen, Molly D; Ramer, Naomi; Payami, Ali


    Ghost cell tumors are a family of lesions that range in presentation from cyst to solid neoplasm and in behavior from benign to locally aggressive or metastatic. All are characterized by the presence of ameloblastic epithelium, ghost cells, and calcifications. This report presents the cases of a 14-year-old girl with a calcifying cystic odontogenic tumor (CCOT) and a 65-year-old woman with a peripheral dentinogenic ghost cell tumor (DGCT) with dysplastic changes, a rare locally invasive tumor of odontogenic epithelium. The first patient presented with a 1-year history of slowly progressing pain and swelling at the left body of the mandible. Initial panoramic radiograph displayed a mixed radiolucent and radiopaque lesion. An incisional biopsy yielded a diagnosis of CCOT. Decompression of the mass was completed; after 3 months, it was enucleated and immediately grafted with bone harvested from the anterior iliac crest. The second patient presented with a 3-month history of slowly progressing pain and swelling at the left body of the mandible. Initial panoramic radiograph depicted a mixed radiolucent and radiopaque lesion with saucerization of the buccal mandibular cortex. An incisional biopsy examination suggested a diagnosis of DGCT because of the presence of ghost cells, dentinoid, and islands of ameloblastic epithelium. Excision of the mass with peripheral ostectomy was completed. At 6 and 12 months of follow-up, no evidence of recurrence was noted.

  16. Targeting thapsigargin towards tumors

    DEFF Research Database (Denmark)

    Doan, Nhu Thi Quynh; Paulsen, Eleonora Sandholdt; Sehgal, Pankaj


    substrates for either prostate specific antigen (PSA) or prostate specific membrane antigen (PSMA) prodrugs were created, which selectively affect prostate cancer cells or neovascular tissue in tumors. One of the prodrug is currently tested in clinical phase II. The prodrug under clinical trial has been...

  17. Serodiagnosis for Tumor Viruses (United States)

    Morrison, Brian J.; Labo, Nazzarena; Miley, Wendell J.; Whitby, Denise


    The known human tumor viruses include the DNA viruses Epstein-Barr virus, Kaposi sarcoma herpesvirus, Merkel cell polyomavirus, human papillomavirus, and hepatitis B virus. RNA tumor viruses include Human T-cell lymphotrophic virus type-1 and hepatitis C virus. The serological identification of antigens/antibodies in plasma serum is a rapidly progressing field with utility for both scientists and clinicians. Serology is useful for conducting seroepidemiology studies and to inform on the pathogenesis and host immune response to a particular viral agent. Clinically, serology is useful for diagnosing current or past infection and for aiding in clinical management decisions. Serology is useful for screening blood donations for infectious agents and for monitoring the outcome of vaccination against these viruses. Serodiagnosis of human tumor viruses has improved in recent years with increased specificity and sensitivity of the assays, as well as reductions in cost and the ability to assess multiple antibody/antigens in single assays. Serodiagnosis of tumor viruses plays an important role in our understanding of the prevalence and transmission of these viruses and ultimately in the ability to develop treatments/preventions for these globally important diseases. PMID:25843726

  18. Radioembolization of liver tumors

    NARCIS (Netherlands)

    van den Hoven, AF


    Radioembolization is a therapy during which radioactive microspheres are administered through a microcatheter placed in the hepatic arterial vasculature in order to irradiate liver tumors from within. In the past decennium, this treatment has evolved as a safe and effective treatment option for pati

  19. Tumor ablations in IMRI

    Institute of Scientific and Technical Information of China (English)

    Roberto Blanco Sequeiros


    @@ IntroductionMagnetic resonance imaging based guidance control and monitoring of minimally invasive intervention has developed from a hypothetical concept to a practical possibility. Magnetic-resonance-guided interstitial therapy in principle is defined as a treatment technique for ablating deepseated tumors in the human body.

  20. Intracardiac tumor causing

    Directory of Open Access Journals (Sweden)

    Christopher P. Jordan


    Full Text Available The following report describes the case of newborn girl with an asymptomatic systolic murmur, which on imaging revealed a nearly obstructive mass in the left-ventricular outflow tract. The mass was resected and found to be consistent with a rhabdomyoma. Here, we describe the pathologic and clinical characteristics of this tumor.

  1. [2 infrequent vulvar tumors]. (United States)

    Aguilar, G; Benavides, A; Villanueva, M E


    Two cases of malignant vulvar tumors are presented: a malignant Melanoma and a case of Carcinoma of the Bartholin Gland. Both were diagnosticated during 1993 in the Obstetrics and Gynecology Department of Dr. Félix Bulnes Cerda Hospital. Clinical and anatomophatological aspects are studied.

  2. NO, Immunosuppression and Tumor Immmunotherapy


    Hegardt, Pontus


    This study aims at clarifying the role of NO in the immunosuppression induced by in vivo tumor growth and by tumor immunotherapy, and determining whether the inhibition of NO production can be used as an adjuvant in tumor immunotherapy. We have shown previously that tumor cells, glioma (N32) and colon carcinoma (H1D2), when genetically engineered to express such immune stimulatory cytokines as IFN-g and IL-18, induce strong anti-tumor immune response, in immunized tumor-free rats, whereas onl...

  3. Culinary nationalism. (United States)

    Ferguson, Priscilla Parkhurst


    Culinary consciousness raisers, cooking texts often serve as vehicles of national identification. From Pampille (Marthe Allard Daudet) and her cookbook, Les Bons Plats de France, in 1913 to the international culinary competitions of today such as the Bocuse d'or, culinary distinction promotes national interests. In contrast to the strident nationalism of the early twentieth century, culinary nationalism today operates in an increasingly globalized world. National culinary distinction defines the nation and sells its products in a highly competitive international arena. A recent culinary text, the South Korean film Le Grand Chef [Sik Gaek ] (2007), illustrates the phenomenon, subsuming national culinary promotion in a mega culinary competition, all in the service of Korean culinary achievement.

  4. Rare and Challenging Tumor Entity: Phyllodes Tumor of the Prostate

    Directory of Open Access Journals (Sweden)

    Andreas Bannowsky


    Full Text Available Cystic epithelial-stromal tumors of the prostate are rare, with 82 cases reported in literature. These cases have been published under a variety of diagnoses, including phyllodes tumor and prostatic stromal proliferation of uncertain malignant potential as well as a malignant tumor called “prostatic stromal sarcoma”. We report a case of a 60-year-old man with the histological diagnosis of phyllodes tumor of the prostate in transurethral resection specimen.

  5. Rare and Challenging Tumor Entity: Phyllodes Tumor of the Prostate


    Andreas Bannowsky; Andreas Probst; Helmut Dunker; Tillmann Loch


    Cystic epithelial-stromal tumors of the prostate are rare, with 82 cases reported in literature. These cases have been published under a variety of diagnoses, including phyllodes tumor and prostatic stromal proliferation of uncertain malignant potential as well as a malignant tumor called “prostatic stromal sarcoma”. We report a case of a 60-year-old man with the histological diagnosis of phyllodes tumor of the prostate in transurethral resection specimen.

  6. Advances of Tumor Hyperthermia and Tumor Immunology in Translational Medicine


    Hooshang Lahooti


    Hyperthermia is another important method in the treatment of tumors, secondary to surgery, radiotherapy, chemotherapy and biotherapy. It has been demonstrated the efficacy and versatility of hyperthermia in a lot of randomized trials across various primary cancers. Both heat shock proteins (HSPs) and dendritic cells (DCs) are greatly affected by hyperthermia and closely related to the tumor immunology. Nowadays, tumor hyperthermia and tumor immunology have been attached much attention in the ...

  7. National Intelligence and National Prosperity (United States)

    Hunt, Earl; Wittmann, Werner


    What is the relation between the cognitive competence of a national population that nation's economic prosperity? Lynn and Vanhanen [Lynn, R. & Vanhanen, T. (2002). "IQ and the wealth of nations." Westport, CT: Praeger.] presented data pointing to an exceptionally strong relationship between IQ scores and Gross Domestic Product per capita (GDP/c).…

  8. Epidemiological features of brain tumors

    Directory of Open Access Journals (Sweden)

    Živković Nenad


    Full Text Available Brain tumors account for 1.4% of all cancers and 2.4% of all cancer-related deaths. The incidence of brain tumors varies and it is higher in developed countries of Western Europe, North America, Australia and New Zealand. In Serbia, according to data from 2009, malignant brain tumors account for 2. 2 of all tumors, and from all cancer­related deaths, 3.2% is caused by malignant brain tumors. According to recent statistical reports, an overall incidence of brain tumors for benign and malignant tumors combined is 18.71 per 100,000 persons/year. The most common benign brain tumor in adults is meningioma, which is most present in women, and the most common malignant tumor is glioblastoma, which is most present in adult men. Due to high mortality, especially in patients diagnosed with glioblastoma and significant brain tumor morbidity, there is a constant interest in understanding its etiology in order to possibly prevent tumor occurrence in future and enable more efficient treatment strategies for this fatal brain disease. Despite the continuously growing number of epidemiological studies on possible factors of tumor incidence, the etiology remains unclear. The only established environmental risk factor of gliomas is ionizing radiation exposure. Exposure to radiofrequency electromagnetic fields via cell phone use has gained a lot of attention as a potential risk factor of brain tumor development. However, studies have been inconsistent and inconclusive, so more definite results are still expected.

  9. Neuroendocrine tumors of the pancreas.

    LENUS (Irish Health Repository)

    Davies, Karen


    Pancreatic endocrine tumors are rare neoplasms accounting for less than 5% of pancreatic malignancies. They are broadly classified into either functioning tumors (insulinomas, gastrinomas, glucagonomas, VIPomas, and somatostatinomas) or nonfunctioning tumors. The diagnosis of these tumors is difficult and requires a careful history and examination combined with laboratory tests and radiologic imaging. Signs and symptoms are usually related to hormone hypersecretion in the case of functioning tumors and to tumor size or metastases with nonfunctioning tumors. Surgical resection remains the treatment of choice even in the face of metastatic disease. Further development of novel diagnostic and treatment modalities offers potential to greatly improve quality of life and prolong disease-free survival for patients with pancreatic endocrine tumors.

  10. Beta-2 Microglobulin Tumor Marker (United States)

    ... services. Advertising & Sponsorship: Policy | Opportunities Beta-2 Microglobulin Tumor Marker Share this page: Was this page helpful? Also ... Microglobulin, Serum, Urine, or CSF Related tests: Albumin , Tumor Markers , CSF Analysis All content on Lab Tests Online ...

  11. Neuroendocrine tumors of the pancreas.

    LENUS (Irish Health Repository)

    Davies, Karen


    Pancreatic endocrine tumors are rare neoplasms accounting for less than 5% of pancreatic malignancies. They are broadly classified into either functioning tumors (insulinomas, gastrinomas, glucagonomas, VIPomas, and somatostatinomas) or nonfunctioning tumors. The diagnosis of these tumors is difficult and requires a careful history and examination combined with laboratory tests and radiologic imaging. Signs and symptoms are usually related to hormone hypersecretion in the case of functioning tumors and to tumor size or metastases with nonfunctioning tumors. Surgical resection remains the treatment of choice even in the face of metastatic disease. Further development of novel diagnostic and treatment modalities offers potential to greatly improve quality of life and prolong disease-free survival for patients with pancreatic endocrine tumors.

  12. Primary tumors of the patella

    National Research Council Canada - National Science Library

    Song, Mingzhi; Zhang, Zhen; Wu, Yuxuan; Ma, Kai; Lu, Ming


    The patella is an uncommon location for cancerous occurrence and development. The majority of tumors of the patella are benign, with a significant incidence of giant cell tumors and chondroblastoma...

  13. Biopsy in Musculoskeletal Tumors

    Directory of Open Access Journals (Sweden)

    Mohammad Gharehdaghi


    Full Text Available Diagnosis of bone tumors is based on careful evaluation of clinical, imaging and a pathologic findings. So the biopsy of bone and soft tissue sarcomas is the final step in evaluation and a fundamental step in the diagnosis of the lesion. It should not be performed as a shortcut to diagnosis (1. The biopsy should be performed in order to confirm the diagnosis and differentiate among few diagnoses after careful staged studies. Real and artificial changes in imaging studies will be superimposed after performing biopsy, which may alter the interpretation if done after biopsy is taken (1. The correct management of a sarcoma depends on the accurate diagnosis. Inadequate, inapprppriate, or inaccurate non-representative biopsy leads to poorer outcome in terms of survivorship and limb salvage. An incorrect, unplanned incision and biopsy may unnecessarily contaminate uninvolved compartments which may convert a salvageable limb to amputation. Anatomic approach along with the proper biopsy techniques may lead to success or catastrophe. It is clear that in patients with inappropriate biopsy, the chance of the need to change the treatment to more radical than would originally be expected is significantly higher. Also it is more probable to need to  convert curative to palliative treatment and to require adjuvant radiotherapy in patients with inappropriate biopsies. Patients with sarcoma are best served by early referral to a specialized center where staged investigations and biopsy can be performed with minimal morbidity (3. Open biopsy is still considered the gold standard; however, recent studies suggest comparable results with percutaneous core needle biopsy. Our study on 103 consecutive CNB and open biopsy showed comparable results as well. Surgeons need to answer to two questions prior to performing a biopsy: 1-          Where is the best part of the lesion to be biopsied? 2-          What is the safest route without contaminating

  14. Tumor suppressor and hepatocellular carcinoma

    Institute of Scientific and Technical Information of China (English)

    Juliette Martin; Jean-Frangois Dufour


    A few signaling pathways are driving the growth of hepatocellular carcinoma. Each of these pathways possesses negative regulators. These enzymes, which normally suppress unchecked cell proliferation, are circumvented in the oncogenic process, either the over-activity of oncogenes is sufficient to annihilate the activity of tumor suppressors or tumor suppressors have been rendered ineffective. The loss of several key tumor suppressors has been described in hepatocellular carcinoma. Here, we systematically review the evidence implicating tumor suppressors in the development of hepatocellular carcinoma.

  15. Radiological review of pleural tumors

    Directory of Open Access Journals (Sweden)

    Binit Sureka


    Full Text Available Tumors of the pleura are not uncommon and diagnosis is clinched by combined imaging and clinical correlation. Malignant tumors are more common than benign tumors. Initial imaging modalities are chest radiography and Computed Tomography (CT. Further characterization may be required using Ultrasoundgraphy (USG, Magnetic resonance Imaging (MRI and PET-CT. Biopsy remains gold standard. This article highlights various common and uncommon tumors of pleura and characteristic imaging findings.

  16. Bednar Tumor: An Uncommon Entity


    Gayathri P Amonkar; Asha Rupani; Ajay Shah; Ramesh Deshpande


    Bednar tumor is an uncommon variant of dermatofibrosarcoma protuberans. Also known as pigmented dermatofibrosarcoma protuberans, this tumor is of intermediate grade. It is seen in adults and has a predisposition to affect the shoulder region. We report a rare case of Bednar tumor in a 40-year-old female patient. The diagnosis of Bednar tumor must be considered while reporting pigmented subcutaneous spindle cell lesions.

  17. Phyllodes tumor showing intraductal growth. (United States)

    Makidono, Akari; Tsunoda, Hiroko; Mori, Miki; Yagata, Hiroshi; Onoda, Yui; Kikuchi, Mari; Nozaki, Taiki; Saida, Yukihisa; Nakamura, Seigo; Suzuki, Koyu


    Phyllodes tumor of the breast is a rare fibroepithelial lesion and particularly uncommon in adolescent girls. It is thought to arise from the periductal rather than intralobular stroma. Usually, it is seen as a well-defined mass. Phyllodes tumor showing intraductal growth is extremely rare. Here we report a girl who has a phyllodes tumor with intraductal growth.

  18. Apoptosis in irradiated murine tumors. (United States)

    Stephens, L C; Ang, K K; Schultheiss, T E; Milas, L; Meyn, R E


    Early radiation responses of transplantable murine ovarian (OCaI) and hepatocellular (HCaI) carcinomas were examined at 6, 24, 48, 96, and 144 h after single photon doses of 25, 35, or 45 Gy. Previous studies using tumor growth delay and tumor radiocurability assays had shown OCaI tumors to be relatively radiosensitive and HCaI tumors to be radioresistant. At 6 h, approximately 20% of nuclei in OCaI tumors showed aberrations characteristic of cell death by apoptosis. This contrasted to an incidence of 3% in HCaI tumors. Mitotic activity was eliminated in OCaI tumors but was only transiently suppressed in HCaI tumors. At 24-96 h, OCaI tumors continued to display apoptosis and progressive necrosis, whereas HCaI tumors responded by exhibiting marked pleomorphism. Factors other than mitotic activity may influence tumor radiosensitivity, and one of these may be susceptibility to induction of apoptosis (programmed cell death), because this was a prominent early radiation response by the radiosensitive OCaI tumors.

  19. Tumor Microenvironment in the Brain

    Energy Technology Data Exchange (ETDEWEB)

    Lorger, Mihaela [Leeds Institute of Molecular Medicine, University of Leeds, St. James’s University Hospital, Beckett Street, Leeds, LS9 7TF (United Kingdom)


    In addition to malignant cancer cells, tumors contain a variety of different stromal cells that constitute the tumor microenvironment. Some of these cell types provide crucial support for tumor growth, while others have been suggested to actually inhibit tumor progression. The composition of tumor microenvironment varies depending on the tumor site. The brain in particular consists of numerous specialized cell types such as microglia, astrocytes, and brain endothelial cells. In addition to these brain-resident cells, primary and metastatic brain tumors have also been shown to be infiltrated by different populations of bone marrow-derived cells. The role of different cell types that constitute tumor microenvironment in the progression of brain malignancies is only poorly understood. Tumor microenvironment has been shown to be a promising therapeutic target and diagnostic marker in extracranial malignancies. A better understanding of tumor microenvironment in the brain would therefore be expected to contribute to the development of improved therapies for brain tumors that are urgently required due to a poor availability of treatments for these malignancies. This review summarizes some of the known interactions between brain tumors and different stromal cells, and also discusses potential therapeutic approaches within this context.

  20. Sertoli-Leydig cell tumor (United States)

    Sertoli-stromal cell tumor; Arrhenoblastoma; Androblastoma; Ovarian cancer - Sertoli-Leydig cell tumor ... The exact cause of this tumor is not known. Changes (mutations) in genes may play a role. SLCT occur most often in young women 20 to 30 ...

  1. Notch Signaling and Brain Tumors

    DEFF Research Database (Denmark)

    Stockhausen, Marie; Kristoffersen, Karina; Poulsen, Hans Skovgaard


    Human brain tumors are a heterogenous group of neoplasms occurring inside the cranium and the central spinal cord. In adults and children, astrocytic glioma and medulloblastoma are the most common subtypes of primary brain tumors. These tumor types are thought to arise from cells in which Notch...

  2. Tumor uptake of radioruthenium compounds

    Energy Technology Data Exchange (ETDEWEB)

    Srivastava, S C; Richards, P; Meinken, G E; Larson, S M; Grunbaum, Z


    The use of ruthenium-97 as a scintigraphic agent, particularly for tumor localization, is investigated. The tumor uptake of ruthenium chloride and ruthenium-labelled transferrin is evaluated and their application as tumor-imagine agents is compared to gallium-67 citrate. (ACR)

  3. Notch Signaling and Brain Tumors

    DEFF Research Database (Denmark)

    Stockhausen, Marie; Kristoffersen, Karina; Poulsen, Hans Skovgaard


    Human brain tumors are a heterogenous group of neoplasms occurring inside the cranium and the central spinal cord. In adults and children, astrocytic glioma and medulloblastoma are the most common subtypes of primary brain tumors. These tumor types are thought to arise from cells in which Notch...

  4. Metastasis and circulating tumor cells

    NARCIS (Netherlands)

    Dalum, van G.; Holland, L.; Terstappen, L.W.M.M.


    Cancer is a prominent cause of death worldwide. In most cases, it is not the primary tumor which causes death, but the metastases. Metastatic tumors are spread over the entire human body and are more difficult to remove or treat than the primary tumor. In a patient with metastatic disease, circulati

  5. [Retroperitoneal Tumor: Neurofibroma. (United States)

    Lada, Paul Eduardo; Marriot, Daniela; Sanchez Tasonne, Carlos; Sanchez, Martin; Caballero, Fabian; Massa, Martin


    The neurofibroma is a benign tumor that grows from the sheath of the peripheral nerves, which is often localized on superficial tissues, especially in isolated forms. The neurofibromas can be of two types, localized or diffuse, the last one closely related to Von Recklinghausen disease or NF-1. We described a 37 years old male e patient, 37 years without symptoms, and the computed tomography scan (CT), and magnetic resonance imaging showed a tumor in proximity to the right kidney, the psoas muscle, the spine and compressing the cava vein, but cannot be accurately determinate the invasion of these structures. We review the differential diagnosis, therapeutic and the management of this disease in this clinical case.

  6. [Perihilar cholangiocarcinoma (Klatskin tumor)]. (United States)

    Stavrou, G A; Donati, M; Faiss, S; Jenner, R M; Niehaus, K J; Oldhafer, K J


    Perihilar cholangiocarcinoma or Klatskin tumors are a rare entity arising from the extrahepatic bile duct bifurcation. Considering the close anatomical relationship of the bile duct bifurcation with the portal vein bifurcation and hepatic arteries, surgical treatment is demanding. With an incidence of only 2-4 cases/100,000 population/year patients should be referred to a specialized center. The tumors are usually poorly differentiated adenocarcinomas growing diffusely along the duct and also the perineural sheath. Only radical surgery offers a curative option and currently surgical strategy usually consists of en bloc resection of the bile duct, extended liver resection and portal vein resection. Proximal and lateral safety margin R0 resections are technically very demanding procedures because of the local anatomy.

  7. Retroperitoneal inflammatory myofibroblastic tumor

    Directory of Open Access Journals (Sweden)

    Bapsy Poonamalle P


    Full Text Available Abstract Background Inflammatory myofibroblastic tumor (IMT is a neoplasm of unknown etiology occurring at various sites. By definition, it is composed of spindle cells (myofibroblasts with variable inflammatory component, hence the name is IMT. Case presentation The present case is of a 46 years old woman presented with a history of flank pain, abdominal mass and intermittent hematuria for last 6 months. The initial diagnosis was kept as renal cell carcinoma. Finally, it turned out to be a case of retroperitoneal IMT. The patient was managed by complete surgical resection of the tumor. Conclusion IMT is a rare neoplasm of uncertain biological potential. Complete surgical resection remains the mainstay of the treatment.

  8. Inflammatory myofibroblastic tumor

    Directory of Open Access Journals (Sweden)

    Sangeeta Palaskar


    Full Text Available Inflammatory myofibroblastic tumor is an uncommon lesion of unknown cause. It encompasses a spectrum of myofibroblastic proliferation along with varying amount of inflammatory infiltrate. A number of terms have been applied to the lesion, namely, inflammatory pseudotumor, fibrous xanthoma, plasma cell granuloma, pseudosarcoma, lymphoid hamartoma, myxoid hamartoma, inflammatory myofibrohistiocytic proliferation, benign myofibroblatoma, and most recently, inflammatory myofibroblastic tumor. The diverse nomenclature is mostly descriptive and reflects the uncertainty regarding true biologic nature of these lesions. Recently, the concept of this lesion being reactive has been challenged based on the clinical demonstration of recurrences and metastasis and cytogenetic evidence of acquired clonal chromosomal abnormalities. We hereby report a case of inflammatory pseudotumor and review its inflammatory versus neoplastic behavior.

  9. Pediatric Suprasellar Tumors. (United States)

    McCrea, Heather J; George, Emilie; Settler, Allison; Schwartz, Theodore H; Greenfield, Jeffrey P


    The various childhood suprasellar tumors, while pathologically distinct, present similar clinical and surgical challenges as a result of their common anatomic location. These lesions are in close proximity to or may invade the optic nerve and chiasm, pituitary gland and infundibulum, hypothalamus, and third ventricle, leading to presenting features including visual field loss, impairment in visual acuity, endocrine dysfunction, and hydrocephalus. Though many suprasellar lesions are relatively benign in pathology, treatment may be complicated by high surgical morbidity resulting from damage to the hypothalamic-pituitary axis. Here we review the most frequent pediatric lesions occurring in the suprasellar region: craniopharyngioma, chiasmatic glioma, germ cell tumor, Rathke cleft and arachnoid cysts, pituitary adenoma, and histiocytosis. This review outlines both common presenting features and differentiating aspects of these lesions. It also includes classic radiographic presentations and treatment considerations for each lesion.

  10. Tumor-Induced Hyperlipidemia Contributes to Tumor Growth

    Directory of Open Access Journals (Sweden)

    Jianfeng Huang


    Full Text Available The known link between obesity and cancer suggests an important interaction between the host lipid metabolism and tumorigenesis. Here, we used a syngeneic tumor graft model to demonstrate that tumor development influences the host lipid metabolism. BCR-Abl-transformed precursor B cell tumors induced hyperlipidemia by stimulating very low-density lipoprotein (VLDL production and blunting VLDL and low-density lipoprotein (LDL turnover. To assess whether tumor progression was dependent on tumor-induced hyperlipidemia, we utilized the VLDL production-deficient mouse model, carboxylesterase3/triacylglycerol hydrolase (Ces3/TGH knockout mice. In Ces3/Tgh−/− tumor-bearing mice, plasma triglyceride and cholesterol levels were attenuated. Importantly tumor weight was reduced in Ces3/Tgh−/− mice. Mechanistically, reduced tumor growth in Ces3/Tgh−/− mice was attributed to reversal of tumor-induced PCSK9-mediated degradation of hepatic LDLR and decrease of LDL turnover. Our data demonstrate that tumor-induced hyperlipidemia encompasses a feed-forward loop that reprograms hepatic lipoprotein homeostasis in part by providing LDL cholesterol to support tumor growth.

  11. National Parks (United States)

    Department of Transportation — National Park Service unit boundaries (NTAD). These park boundaries signify legislative boundary definitions and local park names have been consolidated according to...

  12. Neurons and tumor suppressors. (United States)

    Zochodne, Douglas W


    Neurons choose growth pathways with half hearted reluctance, behavior that may be appropriate to maintain fixed long lasting connections but not to regenerate them. We now recognize that intrinsic brakes on regrowth are widely expressed in these hesitant neurons and include classical tumor suppressor molecules. Here, we review how two brakes, PTEN (phosphatase and tensin homolog deleted on chromosome 10) and retinoblastoma emerge as new and exciting knockdown targets to enhance neuron plasticity and improve outcome from damage or disease.

  13. Neurons and Tumor Suppressors


    Douglas W Zochodne


    Neurons choose growth pathways with half hearted reluctance, behavior that may be appropriate to maintain fixed long lasting connections but not to regenerate them. We now recognize that intrinsic brakes on regrowth are widely expressed in these hesitant neurons and include classical tumor suppressor molecules. Here, we review how two brakes, PTEN (phosphatase and tensin homolog deleted on chromosome 10) and retinoblastoma emerge as new and exciting knockdown targets to e...

  14. TRUS Findings of Prostate Tumor or Tumor Like Lesions

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Hak Jong; Jang, Jung Min; Kim, Seung Hyup [Seoul National University College of Medicine, Seoul (Korea, Republic of)


    Tumors or tumor-like lesions in the prostate raise questions concerning their histogenesis and they may have prognoses dissimilar to those of prostatic adenocarcinoma. Several neoplasms involving the prostate have been described and characterized in recent years. In addition to adenocarcinoma, they include mucinous cyst adenocarcinoma, neuroendocrine cancer, lymphoma, spindle cell neoplasm, squamous cell carcinoma, transitional cell carcinoma, and benign prostatic hyperplasia (BPH) mimicking malignancy. In addition, infectious conditions such as tuberculosis and some stages of prostatic abscess can also mimic prostate tumors. Radiologic findings overlap and have limited roles in the diagnoses of these entities. However, knowledge of these variable tumors and tumor-like conditions is helpful when making accurate radiologic diagnoses, which have important clinical implications for treatment and prognosis. Transrectal ultrasound (TRUS) and available pathologic images of unusual tumors and tumor- like lesions are demonstrated in this article

  15. Retroperitoneal calcifying fibrous tumor mimicking an adrenal tumor. (United States)

    Prochaska, Erica C; Sciallis, Andrew P; Miller, Barbra S


    Establishing the etiology of a retroperitoneal tumor may be difficult due to close proximity of multiple organs. Evaluation of retroperitoneal tumors often leads to surgery, many times to obtain a definitive diagnosis and rule out malignancy. Calcifying fibrous tumors (CFT) are very rare soft tissue tumors occurring most often in young patients. They are most often found arising in the thoracic cavity, mediastinum, abdominal cavity and extremities and usually have a benign clinical course. Macrocscopically, the tumors are well circumscribed and firm with a white-tan appearance. Histologically, CFT comprised a hypocellular proliferation of bland spindle cells, densely hyalinized collagen, chronic lymphoplasmacytic inflammation and dystrophic calcifications. Other considerations in the pathologic differential diagnosis include solitary fibrous tumor and inflammatory myofibroblastic tumor.

  16. Characterization of the Tumor Secretome from Tumor Interstitial Fluid (TIF). (United States)

    Gromov, Pavel; Gromova, Irina


    Tumor interstitial fluid (TIF) surrounds and perfuses bodily tumorigenic tissues and cells, and can accumulate by-products of tumors and stromal cells in a relatively local space. Interstitial fluid offers several important advantages for biomarker and therapeutic target discovery, especially for cancer. Here, we describe the most currently accepted method for recovering TIF from tumor and nonmalignant tissues that was initially performed using breast cancer tissue. TIF recovery is achieved by passive extraction of fluid from small, surgically dissected tissue specimens in phosphate-buffered saline. We also present protocols for hematoxylin and eosin (H&E) staining of snap-frozen and formalin-fixed, paraffin-embedded (FFPE) tumor sections and for proteomic profiling of TIF and matched tumor samples by high-resolution two-dimensional gel electrophoresis (2D-PAGE) to enable comparative analysis of tumor secretome and paired tumor tissue.

  17. Renal neuroendocrine tumors

    Directory of Open Access Journals (Sweden)

    Brian R Lane


    Full Text Available Objectives: Neuroendocrine tumors (NETs are uncommon tumors that exhibit a wide range of neuroendocrine differentiation and biological behavior. Primary NETs of the kidney, including carcinoid tumor, small cell carcinoma (SCC, and large cell neuroendocrine carcinoma (LCNEC are exceedingly rare. Materials and Methods: The clinicopathologic features of renal NETs diagnosed at a single institution were reviewed along with all reported cases in the worldwide literature. Results: Eighty renal NETs have been described, including nine from our institution. Differentiation between renal NETs and the more common renal neoplasms (renal cell carcinoma, transitional cell carcinoma can be difficult since clinical, radiographic, and histopathologic features overlap. Immunohistochemical staining for neuroendocrine markers, such as synaptophysin and chromogranin, can be particularly helpful in this regard. Renal carcinoids are typically slow-growing, may secrete hormones, and pursue a variable clinical course. In contrast, SCC and LCNEC often present with locally advanced or metastatic disease and carry a poor prognosis. Nephrectomy can be curative for clinically localized NETs, but multimodality treatment is indicated for advanced disease. Conclusions: A spectrum of NETs can rarely occur in the kidney. Renal carcinoids have a variable clinical course; SCC and LCNEC are associated with poor clinical outcomes. Diagnosis of NETs, especially LCNEC, requires awareness of their rare occurrence and prudent use of immunohistochemical neuroendocrine markers.

  18. [Resection of Klatskin tumors]. (United States)

    Seehofer, D; Kamphues, C; Neuhaus, P


    Curative treatment of Klatskin tumors by radical surgical procedures with surgical preparation distant to the tumor region results in 5-year survival rates of 30-50%. This requires mandatory en bloc liver resection and resection of the extrahepatic bile duct often together with vascular resection. Nevertheless, the ideal safety margin of 0.5-1 cm remote from the macroscopic tumor extensions cannot be achieved in all cases. Based on hilar anatomy the probability of an adequate safety margin is higher using extended right hemihepatectomy together with portal vein resection compared to left hemihepatectomy. However, due to severe atrophy of the left liver lobe solely left-sided hepatectomy is feasible in some patients. In cases of eligibility for both procedures right hemihepatectomy is preferentially used due to the higher oncological radicality if sufficient liver function is present. Postoperative hepatic insufficiency and bile leakage after demanding biliary reconstruction, often with several small orifices, contribute to the postoperative complication rate of this complex surgical disease pattern.

  19. Cardiac tumors: echo assessment. (United States)

    Mankad, Rekha; Herrmann, Joerg


    Cardiac tumors are exceedingly rare (0.001-0.03% in most autopsy series). They can be present anywhere within the heart and can be attached to any surface or be embedded in the myocardium or pericardial space. Signs and symptoms are nonspecific and highly variable related to the localization, size and composition of the cardiac mass. Echocardiography, typically performed for another indication, may be the first imaging modality alerting the clinician to the presence of a cardiac mass. Although echocardiography cannot give the histopathology, certain imaging features and adjunctive tools such as contrast imaging may aid in the differential diagnosis as do the adjunctive clinical data and the following principles: (1) thrombus or vegetations are the most likely etiology, (2) cardiac tumors are mostly secondary and (3) primary cardiac tumors are mostly benign. Although the finding of a cardiac mass on echocardiography may generate confusion, a stepwise approach may serve well practically. Herein, we will review such an approach and the role of echocardiography in the assessment of cardiac masses.

  20. Neuroendocrine Tumor, diagnostic difficulties

    Directory of Open Access Journals (Sweden)

    Pedro Oliveira


    Full Text Available Ectopic adrenocorticotropic hormone (ACTH secretion is a rare disease. A 51 years old woman, with a Cushing syndrome secondary to ectopic ACTH secretion, diagnosed in 2009, with mediastinal lymphadenopathy, whose biopsy was compatible with lung small cell carcinoma, staged as IIIB using TNM classification. No other lesions were found in patient study. The patient was submitted to chemotherapy, associated to ketoconazole 200 mg twice daily, with partial remission of both conditions. Three years later was admitted with an aggravation of Cushing syndrome. There was no evidence of progression of pulmonary disease. A cystic lesion in the pancreatic uncinated process was found by abdominal CT scan and with avid uptake by DOTANOC PET discreet in anterior mediastinal lymphadenopathy. Biopsy of pancreatic mass revealed a neuroendocrine tumor. Pulmonary masses were biopsied again and was in favor of neuroendocrine tumor. It was assumed the diagnosis of pancreatic neuroendocrine tumor with mediastinal metastasis. The patient initiated lanreotid (120 mg, monthly, subcutaneous in association with ketoconazole. After 5 months of therapy, patient died with sepsis secondary to pneumonia. Neuroendocrine tumours are rare, difficult to diagnose and with poor prognosis when associated with ectopic ACTH secreting Cushing syndrome.

  1. Cardiac tumors: echo assessment

    Directory of Open Access Journals (Sweden)

    Rekha Mankad MD


    Full Text Available Cardiac tumors are exceedingly rare (0.001–0.03% in most autopsy series. They can be present anywhere within the heart and can be attached to any surface or be embedded in the myocardium or pericardial space. Signs and symptoms are nonspecific and highly variable related to the localization, size and composition of the cardiac mass. Echocardiography, typically performed for another indication, may be the first imaging modality alerting the clinician to the presence of a cardiac mass. Although echocardiography cannot give the histopathology, certain imaging features and adjunctive tools such as contrast imaging may aid in the differential diagnosis as do the adjunctive clinical data and the following principles: (1 thrombus or vegetations are the most likely etiology, (2 cardiac tumors are mostly secondary and (3 primary cardiac tumors are mostly benign. Although the finding of a cardiac mass on echocardiography may generate confusion, a stepwise approach may serve well practically. Herein, we will review such an approach and the role of echocardiography in the assessment of cardiac masses.

  2. Mouse Leydig Tumor Cells

    Directory of Open Access Journals (Sweden)

    Bo-Syong Pan


    Full Text Available Cordycepin is a natural pure compound extracted from Cordyceps sinensis (CS. We have demonstrated that CS stimulates steroidogenesis in primary mouse Leydig cell and activates apoptosis in MA-10 mouse Leydig tumor cells. It is highly possible that cordycepin is the main component in CS modulating Leydig cell functions. Thus, our aim was to investigate the steroidogenic and apoptotic effects with potential mechanism of cordycepin on MA-10 mouse Leydig tumor cells. Results showed that cordycepin significantly stimulated progesterone production in dose- and time-dependent manners. Adenosine receptor (AR subtype agonists were further used to treat MA-10 cells, showing that A1, A 2A , A 2B , and A3, AR agonists could stimulate progesterone production. However, StAR promoter activity and protein expression remained of no difference among all cordycepin treatments, suggesting that cordycepin might activate AR, but not stimulated StAR protein to regulate MA-10 cell steroidogenesis. Meanwhile, cordycepin could also induce apoptotic cell death in MA-10 cells. Moreover, four AR subtype agonists induced cell death in a dose-dependent manner, and four AR subtype antagonists could all rescue cell death under cordycepin treatment in MA-10 cells. In conclusion, cordycepin could activate adenosine subtype receptors and simultaneously induce steroidogenesis and apoptosis in MA-10 mouse Leydig tumor cells.

  3. [Intraductal papillary mucinous pancreas tumor]. (United States)

    Maev, I V; Kaziulin, A N; Kucheriavyĭ, Iu A


    Data of the literature on the epidemiology, patogenesis, diagnosis, peculiarities of the symptoms and the treatment of the intraduct papillar pancreatic tumor, are analyzed in this review. These tumors are rare, there are up to 1% of the exocrine pancreatic tumors. Intraduct proliferation of the mucin producing cells, that are disposed as papillars is typical of these tumors. There are the symptoms of the acute or chronic pancreatitis, sometimes the diagnosis of this tumor is accidental. The main diagnostic methods are ultrasound (US) and computed tomography (CT). Endoluminal ultrasound (EUS) and magnetic resonance cholangiopancreatography (MRCP) are the main methods to reveal the intraduct growth. The surgical treatment is necessary for these patients.

  4. Incidence of Primary Spinal Cord, Spinal Meninges, and Cauda Equina Tumors in Korea, 2006-2010



    Purpose Primary spinal cord and appendage tumors (PSCAT) originating from the spinal cord, spinal meninges, and cauda equina are uncommon. Worldwide, population-based cancer registry data are mostly based on malignant tumors only, which means few data are available on PSCATs, including non-malignant tumors. Therefore, the objective of this study was to provide information regarding the incidence of both non-malignant and malignant PSCATs in Korea on a national level. Materials and Methods Inc...

  5. Cellular Potts modeling of tumor growth, tumor invasion and tumor evolution

    NARCIS (Netherlands)

    A. Szabó (Andras); R.M.H. Merks (Roeland)


    htmlabstractDespite a growing wealth of available molecular data, the growth of tumors, invasion of tumors into healthy tissue, and response of tumors to therapies are still poorly understood. Although genetic mutations are in general the first step in the development of a cancer, for the mutated

  6. Laser therapy in intraocular tumors (United States)

    Carstocea, Benone D.; Gafencu, Otilia L.; Apostol, Silvia


    Intraocular tumors present special problems of diagnosis and treatment. Diagnostic methods include, in addition to systemic and ophthalmological examinations, ancillary examinations such as transillumination, fluorescein angiography, ultrasonography, radioactive phosphorus uptake test, radiology, computerized tomography, and fine-needle aspiration biopsy with cytological analyses. Previously, enucleation of the involved eye was generally accepted as management of malignant tumors. Improved therapeutic methods such as photocoagulation and better surgical techniques now provide a variety of therapeutical alternatives. This study consists of 21 cases of intraocular tumors that were managed by Argon laser photocoagulation. Four cases were intraocular metastasis and 17 cases were primitive intraocular tumors. Argon laser therapy proved to be totally ineffective for the intraocular metastasis and a very adequate therapy for the primitive tumors. Tumor extirpations (choroidal, cillary body, or iris tumors) using laser lancet proved to be more suitable than classic surgery.

  7. Nanoparticles carry chemotherapy drug deeper into solid tumors

    Institute of Scientific and Technical Information of China (English)


    @@ CAS researchers have developed a new drug delivery method using nano-sized molecules to carry the chemotherapy drug doxorubicin to tumors, improving the effectiveness of the drug in mice and increasing their survival time. Their work has been reported online June 26 in the Journal of the National Cancer Institute.

  8. T Cell Receptors that Recognize the Tyrosinase Tumor Antigen | NCI Technology Transfer Center | TTC (United States)

    The National Cancer Institute, Surgery Branch, Tumor Immunology Section, is seeking statements of capability or interest from parties interested in collaborative research to further develop, evaluate, or commercialize T Cells Attacking Cancer: T Cell Receptors that Recognize the Tyrosinase Tumor Antigen

  9. TumorHoPe: a database of tumor homing peptides.

    Directory of Open Access Journals (Sweden)

    Pallavi Kapoor

    Full Text Available BACKGROUND: Cancer is responsible for millions of immature deaths every year and is an economical burden on developing countries. One of the major challenges in the present era is to design drugs that can specifically target tumor cells not normal cells. In this context, tumor homing peptides have drawn much attention. These peptides are playing a vital role in delivering drugs in tumor tissues with high specificity. In order to provide service to scientific community, we have developed a database of tumor homing peptides called TumorHoPe. DESCRIPTION: TumorHoPe is a manually curated database of experimentally validated tumor homing peptides that specifically recognize tumor cells and tumor associated microenvironment, i.e., angiogenesis. These peptides were collected and compiled from published papers, patents and databases. Current release of TumorHoPe contains 744 peptides. Each entry provides comprehensive information of a peptide that includes its sequence, target tumor, target cell, techniques of identification, peptide receptor, etc. In addition, we have derived various types of information from these peptide sequences that include secondary/tertiary structure, amino acid composition, and physicochemical properties of peptides. Peptides in this database have been found to target different types of tumors that include breast, lung, prostate, melanoma, colon, etc. These peptides have some common motifs including RGD (Arg-Gly-Asp and NGR (Asn-Gly-Arg motifs, which specifically recognize tumor angiogenic markers. TumorHoPe has been integrated with many web-based tools like simple/complex search, database browsing and peptide mapping. These tools allow a user to search tumor homing peptides based on their amino acid composition, charge, polarity, hydrophobicity, etc. CONCLUSION: TumorHoPe is a unique database of its kind, which provides comprehensive information about experimentally validated tumor homing peptides and their target cells. This

  10. [Surgical therapy of proximal extrahepatic bile duct tumors (Klatskin tumors)]. (United States)

    Timm, S; Gassel, H-J; Thiede, A


    Due to their anatomical position, the tendency of early infiltrative growth and their poor prognosis without treatment, klatskin tumors are challenging concerning diagnosis and therapy. In contrast to other tumors of the gastrointestinal tract, for which exact diagnostic and stage dependent therapeutic guidelines could be formulated, clear recommendations for klatskin tumors are missing. Thus, survival rates after local resection, e. g. resection of the bile duct bifurcation alone, show high rates of R1/2 resection and early tumor recurrence. With an additional hepatic resection formally curative resections and long-term survival can be improved. Extended liver resections including the portal vein provide the highest rates of R0 resections for hilar carcinomas of the extrahepatic bile duct. Survival rates after liver transplantation for klatskin tumors are not yet convincing. Promising first results have been reported for the combination of neoadjuvant treatment and liver transplantation and might show future perspectives for the treatment of klatskin tumors.

  11. Breast Tumor Angiogenesis and Tumor-Associated Macrophages: Histopathologist's Perspective

    Directory of Open Access Journals (Sweden)

    Ewe Seng Ch'ng


    Full Text Available Much progress has been made since the conceptualization of tumor angiogenesis—the induction of growth of new blood vessels by tumor—as a salient feature of clinically significant primary or metastatic cancers. From a practicing histopathologist's point of view, we appraise the application of this concept in breast cancer with particular reference to the evaluation of proangiogenic factors and the assessment of new microvessels in histopathological examination. Recently, much focus has also been centered on the active roles played by tumor-associated macrophages in relation to tumor angiogenesis. We review the literature; many data supporting this facet of tumor angiogenesis were derived from the breast cancer models. We scrutinize the large body of clinical evidence exploring the link between the tumor-associated macrophages and breast tumor angiogenesis and discuss particularly the methodology and limitations of incorporating such an assessment in histopathological examination.

  12. Angiogenesis and tumor

    Directory of Open Access Journals (Sweden)

    Kamran Mansouri


    Full Text Available Angiogenesis, the process of new blood vessel formation from existing ones, plays an important role in the physiologic circumstances such as embryonic development, placenta formation, and wound healing. It is also crucial to progress of pathogenic processes of a variety of disorders, including tumor growth and metastasis. In general, angiogenesis process is a multi-factorial and highly structured sequence of cellular events comprising migration, proliferation and differentiation of endothelial cells and finally vascular formation, maturation and remodeling.Thereby, angiogenesis inhibition as a helping agent to conventional therapies such as chemotherapy and radiation has attracted the scientists’ attentions studying in this field.

  13. Responsible nations

    DEFF Research Database (Denmark)

    Lippert-Rasmussen, Kasper


    In National Responsibility and Global Justice, David Miller defends the view that a member of a nation can be collectively responsible for an outcome despite the fact that: (i) she did not control it; (ii) she actively opposed those of her nation's policies that produced the outcome; and (iii......) actively opposing the relevant policy was costly for her. I argue that Miller's arguments in favor of this strong externalist view about responsibility and control are insufficient. Specifically, I show that Miller's two models of synchronic collective responsibility*the like-minded group model...

  14. Imaging in Pediatric Infratentorial Tumors

    Directory of Open Access Journals (Sweden)

    S. Hajiahmadi


    Full Text Available Intracranial tumors are the second cause of malignancies in childhood following leukemia. The overall incidence varies between 1:20000 and 1:100000 in different series. They are the most common solid tumors that occur in childhood .The most important diagnostic feature of an intracranial mass is its location. They can be supratentorial or infratentorial. With the exception of the first year of life, infratentorial brain tumors are more frequent than supratentorial tumors in the first decade of life. In particular, these are cerebellar low-grade astrocytomas, medulloblastomas, brain stem gliomas and ependymomas of the fourth ventricle. .Posterior fossa tumors also are readily identified with both CT and MRI. Spectroscopy in the analysis of brain tumors has recently come on the scene but may be of limited practical value when it comes to differentiating tumors. However, CT and especially MRI are the primary imaging modalities for the investigation of brain tumors. Sonography can be used in the neonates. With modern imaging, it is relatively easy to detect the presence of a tumor in most patients. The purpose of this essay is to illustrate the imaging features of various infratentorial brain tumors to make a clue for differentiation them by these features.

  15. Mechanisms of Metastatic Tumor Dormancy

    Directory of Open Access Journals (Sweden)

    Mary Osisami


    Full Text Available Tumor metastasis can occur years after an apparent cure due to a phenomenon known as metastatic tumor dormancy; in which tumor masses or individual tumor cells are growth restricted for extended periods of time. This period of dormancy is induced and maintained by several mechanisms, including: (1 Tumor microenvironment factors such as cytokine expression, immunosurveillance and angiogenesis; (2 Metastasis suppressor gene activity; and (3 Cancer therapeutics. Disseminated tumor cells (DTC are the key cells that result in dormant tumors. However, many challenges exist towards isolating DTCs for mechanistic studies. The main DTC that may represent the dormant cell is the cancer stem cells (CSC as they have a slow proliferation rate. In addition to limited knowledge regarding induction of tumor dormancy, there are large gaps in knowledge regarding how tumors escape from dormancy. Emerging research into cancer stem cells, immunotherapy, and metastasis suppressor genes, may lead to new approaches for targeted anti-metastatic therapy to prevent dormancy escape. Overall, an enhanced understanding of tumor dormancy is critical for better targeting and treatment of patients to prevent cancer recurrence.

  16. Imaging tumors of the patella

    Energy Technology Data Exchange (ETDEWEB)

    Casadei, R., E-mail: [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Kreshak, J., E-mail: [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy); Rinaldi, R. [Department of Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Rimondi, E., E-mail: [Department of Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Bianchi, G., E-mail: [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Alberghini, M., E-mail: [Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy); Ruggieri, P. [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Vanel, D., E-mail: [Department of Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy)


    Background: Patellar tumors are rare; only a few series have been described in the literature and radiographic diagnosis can be challenging. We reviewed all patellar tumors at one institution and reviewed the literature. Materials and methods: In an evaluation of the database at one institution from 1916 to 2009, 23,000 bone tumors were found. Of these, 41 involved the patella. All had imaging studies and microscopic diagnostic confirmation. All medical records, imaging studies, and pathology were reviewed. Results: There were 15 females and 26 males, ranging from 8 to 68 years old (average 30). There were 30 benign tumors; eight giant cell tumors, eight chondroblastomas, seven osteoid osteomas, two aneurysmal bone cysts, two ganglions, one each of chondroma, exostosis, and hemangioma. There were 11 malignant tumors: five hemangioendotheliomas, three metastases, one lymphoma, one plasmacytoma, and one angiosarcoma. Conclusion: Patellar tumors are rare and usually benign. As the patella is an apophysis, the most frequent lesions are giant cell tumor in the adult and chondroblastoma in children. Osteoid osteomas were frequent in our series and easily diagnosed. Metastases are the most frequent malignant diagnoses in the literature; in our series malignant vascular tumors were more common. These lesions are often easily analyzed on radiographs. CT and MR define better the cortex, soft tissue extension, and fluid levels. This study presents the imaging patterns of the more common patellar tumors in order to help the radiologist when confronted with a lesion in this location.

  17. What is a pediatric tumor?

    Directory of Open Access Journals (Sweden)

    Mora J


    Full Text Available Jaume Mora1,21Department of Oncology, 2Developmental Tumor Biology Laboratory, Hospital Sant Joan de Deu, Fundacio Sant Joan de Deu, Barcelona, SpainAbstract: Working together with medical oncologists, the question of whether a Ewing sarcoma in a 25-year-old is a pediatric tumor comes up repeatedly. Like Ewing's, some tumors present characteristically at ages that cross over what has been set as the definition of pediatrics (15 years, 18 years, or 21 years?. Pediatric oncology textbooks, surprisingly, do not address the subject of defining a pediatric tumor. They all begin with an epidemiology chapter defining the types of tumors appearing at distinct stages of childhood, adolescence, and young adulthood. Describing the epidemiology of tumors in relation to age, it becomes clear that the disease is related to the phenomenon of aging. The question, however, remains: is there a biological definition of what pediatric age is? And if so, will tumors occurring during this period of life have anything to do with such biological definition? With the aim of finding an objective definition, the fundamental concepts of what defines "pediatrics" was reviewed and then the major features of tumors arising during development were analyzed. The tumors were explored from the perspective of a host immersed in the normal process of growth and development. This physiological process, from pluripotential and undifferentiated cells, makes possible the differentiation, maturation, organization, and function of tissues, organs, and apparatus. A biological definition of pediatric tumors and the infancy–childhood–puberty classification of developmental tumors according to the infancy–childhood–puberty model of normal human development are proposed.Keywords: growth and development, pediatric tumor, infant, childhood and adolescence, pubertal tumors

  18. Multiparametric classification links tumor microenvironments with tumor cell phenotype.

    Directory of Open Access Journals (Sweden)

    Bojana Gligorijevic


    Full Text Available While it has been established that a number of microenvironment components can affect the likelihood of metastasis, the link between microenvironment and tumor cell phenotypes is poorly understood. Here we have examined microenvironment control over two different tumor cell motility phenotypes required for metastasis. By high-resolution multiphoton microscopy of mammary carcinoma in mice, we detected two phenotypes of motile tumor cells, different in locomotion speed. Only slower tumor cells exhibited protrusions with molecular, morphological, and functional characteristics associated with invadopodia. Each region in the primary tumor exhibited either fast- or slow-locomotion. To understand how the tumor microenvironment controls invadopodium formation and tumor cell locomotion, we systematically analyzed components of the microenvironment previously associated with cell invasion and migration. No single microenvironmental property was able to predict the locations of tumor cell phenotypes in the tumor if used in isolation or combined linearly. To solve this, we utilized the support vector machine (SVM algorithm to classify phenotypes in a nonlinear fashion. This approach identified conditions that promoted either motility phenotype. We then demonstrated that varying one of the conditions may change tumor cell behavior only in a context-dependent manner. In addition, to establish the link between phenotypes and cell fates, we photoconverted and monitored the fate of tumor cells in different microenvironments, finding that only tumor cells in the invadopodium-rich microenvironments degraded extracellular matrix (ECM and disseminated. The number of invadopodia positively correlated with degradation, while the inhibiting metalloproteases eliminated degradation and lung metastasis, consistent with a direct link among invadopodia, ECM degradation, and metastasis. We have detected and characterized two phenotypes of motile tumor cells in vivo, which

  19. Advances of Tumor Hyperthermia and Tumor Immunology in Translational Medicine

    Institute of Scientific and Technical Information of China (English)

    Hooshang Lahooti


    Hyperthermia is another important method in the treatment of tumors, secondary to surgery, radiotherapy, chemotherapy and biotherapy. It has been demonstrated the efifcacy and versatility of hyperthermia in a lot of randomized trials across various primary cancers. Both heat shock proteins (HSPs) and dendritic cells (DCs) are greatly affected by hyperthermia and closely related to the tumor immunology. Nowadays, tumor hyperthermia and tumor immunology have been attached much attention in the field of translational medicine. In this article, the action mechanism and immunological effects of hyperthermia, activation of HSPs and DCs as well as HSP- and DC-based cancer vaccine were reviewed from the perspective of translational medicine.

  20. Advances of Tumor Hyperthermia and Tumor Immunology in Translational Medicine

    Directory of Open Access Journals (Sweden)

    Hooshang Lahooti


    Full Text Available Hyperthermia is another important method in the treatment of tumors, secondary to surgery, radiotherapy, chemotherapy and biotherapy. It has been demonstrated the efficacy and versatility of hyperthermia in a lot of randomized trials across various primary cancers. Both heat shock proteins (HSPs and dendritic cells (DCs are greatly affected by hyperthermia and closely related to the tumor immunology. Nowadays, tumor hyperthermia and tumor immunology have been attached much attention in the field of translational medicine. In this article, the action mechanism and immunological effects of hyperthermia, activation of HSPs and DCs as well as HSP- and DC-based cancer vaccine were reviewed from the perspective of translational medicine.

  1. A rare tumor of the lung: inflammatory myofibroblastic tumor

    Directory of Open Access Journals (Sweden)

    Hammas Nawal


    Full Text Available Abstract Inflammatory myofibroblastic tumor is a rare benign lesion whose tumor origin is now proven. It represents 0.7% of all lung tumors. We report the case of a three-year-old child who suffered from a chronic cough with recurrent respiratory infections. Chest X-ray and computed tomography revealed the presence of a left lower lobe lung mass. After pneumonectomy, histological examination combined with immunohistochemical study discovered an inflammatory myofibroblastic tumor. The virtual slide(s for this article can be found here:


    Directory of Open Access Journals (Sweden)

    Sayyed Tarannum, Garje Dattatray H


    Full Text Available Nanomedicine is the process of diagnosing, treating, and preventing disease and traumatic injury, of relieving pain, and of preserving and improving human health, using molecular tools and molecular knowledge of the human body. In the relatively near term, nanomedicine can address many important medical problems by using nanoscale-structured materials and simple nanodevices that can be manufactured today, including the interaction of nanostructured materials with biological systems. The authors predict that technology-assisted medicine and robotics in particular, will have a significant impact over the next few decades. Robots will augment the surgeon’s motor performance, diagnosis capability, and senses with haptics (feel, augmented reality (sight, and ultrasound (sound. Robotic devices have been used in cardiac surgery, urology, fetal surgery, pediatrics, neurosurgery, orthopedics, and many other medical disciplines. In this article, we present the Nanorobot drug delivery to brain tumor, paying special attention to the transformation trends of organizations, and the integration of robots in brain tumor and underscoring potential repercussions which may deserve more attention and further research.

  3. Tumor triquilemal proliferante

    Directory of Open Access Journals (Sweden)

    Fulin Yu-Tseng


    Full Text Available El tumor triquilemal proliferante es una dermatosis tumoral poco frecuente y derivada de la capa externa de la raíz del folículo piloso. Generalmente, es de características benignas, tiende a ser recidivante y se han reportado casos de transformaciones malignas en la bibliografía mundial. El caso trata de una paciente femenina de 59 años, con una neoformación exofítica nodular lobulada de crecimiento lento en piel cabelluda, de 5 años de evolución, con recidivas después de 3 cirugías. No se documentó compromiso óseo ni cerebral en los estudios de gabinete. La biopsia mostró proliferación de nidos de células escamosas en dermis, separados por bandas de tejido fibroso de la epidermis. El abordaje quirúrgico es el tratamiento de elección de esta patología.

  4. Imaging Tumor Necrosis with Ferumoxytol.

    Directory of Open Access Journals (Sweden)

    Maryam Aghighi

    Full Text Available Ultra-small superparamagnetic iron oxide nanoparticles (USPIO are promising contrast agents for magnetic resonance imaging (MRI. USPIO mediated proton relaxation rate enhancement is strongly dependent on compartmentalization of the agent and can vary depending on their intracellular or extracellular location in the tumor microenvironment. We compared the T1- and T2-enhancement pattern of intracellular and extracellular USPIO in mouse models of cancer and pilot data from patients. A better understanding of these MR signal effects will enable non-invasive characterizations of the composition of the tumor microenvironment.Six 4T1 and six MMTV-PyMT mammary tumors were grown in mice and imaged with ferumoxytol-enhanced MRI. R1 relaxation rates were calculated for different tumor types and different tumor areas and compared with histology. The transendothelial leakage rate of ferumoxytol was obtained by our measured relaxivity of ferumoxytol and compared between different tumor types, using a t-test. Additionally, 3 patients with malignant sarcomas were imaged with ferumoxytol-enhanced MRI. T1- and T2-enhancement patterns were compared with histopathology in a descriptive manner as a proof of concept for clinical translation of our observations.4T1 tumors showed central areas of high signal on T1 and low signal on T2 weighted MR images, which corresponded to extracellular nanoparticles in a necrotic core on histopathology. MMTV-PyMT tumors showed little change on T1 but decreased signal on T2 weighted images, which correlated to compartmentalized nanoparticles in tumor associated macrophages. Only 4T1 tumors demonstrated significantly increased R1 relaxation rates of the tumor core compared to the tumor periphery (p<0.001. Transendothelial USPIO leakage was significantly higher for 4T1 tumors (3.4±0.9x10-3 mL/min/100cm3 compared to MMTV-PyMT tumors (1.0±0.9x10-3 mL/min/100 cm3. Likewise, ferumoxytol imaging in patients showed similar findings with

  5. National database

    DEFF Research Database (Denmark)

    Kristensen, Helen Grundtvig; Stjernø, Henrik


    Artikel om national database for sygeplejeforskning oprettet på Dansk Institut for Sundheds- og Sygeplejeforskning. Det er målet med databasen at samle viden om forsknings- og udviklingsaktiviteter inden for sygeplejen.......Artikel om national database for sygeplejeforskning oprettet på Dansk Institut for Sundheds- og Sygeplejeforskning. Det er målet med databasen at samle viden om forsknings- og udviklingsaktiviteter inden for sygeplejen....

  6. Nation First

    Institute of Scientific and Technical Information of China (English)

    He Tao


    In addition to the success of onomic integration,the ultural effects of globalization have been considerable.The increasingly globalized culture creates the potential to alter the way people identify themselves,eroding geographic boundaries as well as local and national cultures.While the West was happily appreciating this gift from globalization,they found themselves involved in an unexpected wave of reemerging nationalism in many countries.

  7. Pericytes limit tumor cell metastasis

    DEFF Research Database (Denmark)

    Xian, Xiaojie; Håkansson, Joakim; Ståhlberg, Anders


    Previously we observed that neural cell adhesion molecule (NCAM) deficiency in beta tumor cells facilitates metastasis into distant organs and local lymph nodes. Here, we show that NCAM-deficient beta cell tumors grew leaky blood vessels with perturbed pericyte-endothelial cell-cell interactions...... and deficient perivascular deposition of ECM components. Conversely, tumor cell expression of NCAM in a fibrosarcoma model (T241) improved pericyte recruitment and increased perivascular deposition of ECM molecules. Together, these findings suggest that NCAM may limit tumor cell metastasis by stabilizing...... the microvessel wall. To directly address whether pericyte dysfunction increases the metastatic potential of solid tumors, we studied beta cell tumorigenesis in primary pericyte-deficient Pdgfb(ret/ret) mice. This resulted in beta tumor cell metastases in distant organs and local lymph nodes, demonstrating a role...

  8. Primary renal primitive neuroectodermal tumor. (United States)

    Goel, V; Talwar, V; Dodagoudar, C; Singh, S; Sharma, A; Patnaik, N


    Primitive Neuroectodermal Tumor of the kidney is a rare entity. Very few cases of primary renal PNET have been reported to date. Most literature about rPNET is isolated case reports. We report a case of rPNET in a 39-year-old male with a pre-operative diagnosis of renal cell carcinoma with renal vein thrombosis. The patient underwent radical nephrectomy with thrombolectomy, and histopathological examination revealed a highly aggressive tumor composed of monotonous sheets of round cells. Tumor cells were positive for CD 99 and FLI-1, hence confirming the diagnosis of Primitive Neuroectodermal Tumor. Post-surgery, patient was given VAC/IE-based adjuvant chemotherapy. In view of highly aggressive nature of this tumor, prompt diagnosis and imparting effective chemotherapy regimen to the patient is required, and it is important to differentiate PNET from other small round-cell tumors because of different therapeutic approach.

  9. Ultrastructural localization of intravenously injected carbon nanohorns in tumor

    Directory of Open Access Journals (Sweden)

    Matsumura S


    Full Text Available Sachiko Matsumura,1 Ryota Yuge,2 Shigeo Sato,3 Akihiro Tomida,3 Toshinari Ichihashi,2 Hiroshi Irie,4 Sumio Iijima,2,5,6 Kiyotaka Shiba,1 Masako Yudasaka6 1Cancer Institute, Japanese Foundation for Cancer Research, Tokyo, Japan; 2Smart Energy Research Laboratories, NEC Corporation, Tsukuba, Japan; 3Cancer Chemotherapy Center, Japanese Foundation for Cancer Research, Koto-ku, Tokyo, Japan; 4Teikyo University School of Medicine, Tokyo, Japan; 5Faculty of Science and Technology, Meijo University, Tenpaku, Nagoya, Japan; 6Nanotube Research Center, National Institute of Advanced Industrial Science and Technology, Higashi, Tsukuba, Japan Abstract: Nanocarbons have many potential medical applications. Drug delivery, diagnostic imaging, and photohyperthermia therapy, especially in the treatment of tumors, have attracted interest. For the further advancement of these application studies, the microscopic localization of nanocarbons in tumor tissues and cells is a prerequisite. In this study, carbon nanohorns (CNHs with sizes of about 100 nm were intravenously injected into mice having subcutaneously transplanted tumors, and the CNHs in tumor tissue were observed with optical and electron microscopy. In the tumor tissue, the CNHs were found in macrophages and endothelial cells within the blood vessels. Few CNHs were found in tumor cells or in the region away from blood vessels, suggesting that, under these study conditions, the enhanced permeability of tumor blood vessels was not effective for the movement of CNHs through the vessel walls. The CNHs in normal skin tissue were similarly observed. The extravasation of CNHs was not so obvious in tumor but was easily found in normal skin, which was probably due to their vessel wall structure difference. Proper understanding of the location of CNHs in tissues is helpful in the development of the medical uses of CNHs. Keywords: ultrastructural localization, carbon nanohorn, tumor, in vivo, extravasation

  10. Differentiated thyroid tumors: surgical indications. (United States)

    Lucchini, R; Monacelli, M; Santoprete, S; Triola, R; Conti, C; Pecoriello, R; Favoriti, P; Di Patrizi, M S; Barillaro, I; Boccolini, A; Avenia, S; D'Ajello, M; Sanguinetti, A; Avenia, N


    Thyroid gland tumors represent 1% of malignant tumors. In Italy their incidence is in constant growth. The aggressiveness depends on the histological type. The relative non-aggressive grade of different forms of tumors is the basis for discussing the treatment of choice: total thyroidectomy vs lobectomy with or without lymphadenectomy of the sixth level in the absence of metastasis. Authors report about their experience, and they advocate, given the high percentage of multicentric forms, total thyroidectomy as treatment of choice.

  11. Glutamate antagonists limit tumor growth



    Neuronal progenitors and tumor cells possess propensity to proliferate and to migrate. Glutamate regulates proliferation and migration of neurons during development, but it is not known whether it influences proliferation and migration of tumor cells. We demonstrate that glutamate antagonists inhibit proliferation of human tumor cells. Colon adenocarcinoma, astrocytoma, and breast and lung carcinoma cells were most sensitive to the antiproliferative effect of the N...

  12. Self-scaling tumor growth

    DEFF Research Database (Denmark)

    Schmiegel, Jürgen

    We study the statistical properties of the star-shaped approximation of in vitro tumor profiles. The emphasis is on the two-point correlation structure of the radii of the tumor as a function of time and angle. In particular, we show that spatial two-point correlators follow a cosine law....... Based on this similarity, we provide a Lévy based model that captures the correlation structure of the radii of the star-shaped tumor profiles....

  13. Glutathione Levels in Human Tumors (United States)

    Gamcsik, Michael P.; Kasibhatla, Mohit S.; Teeter, Stephanie D.; Colvin, O. Michael


    This review summarizes clinical studies in which glutathione was measured in tumor tissue from patients with brain, breast, gastrointestinal, gynecological, head and neck and lung cancer. Glutathione tends to be elevated in breast, ovarian, head and neck and lung cancer and lower in brain and liver tumors compared to disease-free tissue. Cervical, colorectal, gastric and esophageal cancers show both higher and lower levels of tumor glutathione. Some studies show an inverse relationship between patient survival and tumor glutathione. Based on this survey, we recommend approaches that may improve the clinical value of glutathione as a biomarker. PMID:22900535

  14. Tumors of the lacrimal gland

    DEFF Research Database (Denmark)

    Von Holstein, Sarah Linéa; Rasmussen, Peter Kristian; Heegaard, Steffen


    Tumors of the lacrimal gland comprise a wide spectrum, of which the most common demonstrate epithelial and lymphoid differentiation. The diagnosis of lacrimal gland tumors depends primarily on histological evaluation, as do the choice of treatment and prognosis. For some lacrimal gland neoplasms......, such as adenoid cystic carcinoma, the outlook is grave. Optimal treatment for several lacrimal gland tumors is also a matter of controversy. However, recent progress has been made in the molecular and genetic understanding of tumorigenesis for such lesions. This article presents an overview of the histopathology...... of lacrimal gland tumors, together with their epidemiological features, clinical characteristics, and treatment strategies....

  15. Tumors of the sublingual gland

    DEFF Research Database (Denmark)

    Andreasen, Simon; Bjørndal, K; Agander, T K


    Tumors of the salivary glands are a heterogeneous group of diseases most often originating in the major salivary glands. Only a minor proportion of mainly malignant tumors arise in the sublingual gland. Due to the rarity of sublingual gland tumors (SGTs), little is known about the clinicopathologic...... (ACC). Patient demography was similar to salivary gland tumors in other locations. All fine needle aspiration cytologies (FNACs) interpreted as benign were from ACCs. Metastatic disease was found in 12.5 % of ACCs at diagnosis with one third of all ACC patients having metastases at the end of follow...

  16. Subarachnoid hemorrhage in pituitary tumor

    Directory of Open Access Journals (Sweden)

    Ashis Patnaik


    Full Text Available Subarachnoid hemorrhage (SAH is the bleeding into the subarachnoid space containing cerebrospinal fluid. The most common cause of SAH is trauma. Rupture of aneurysms, vascular anomalies, tumor bleeds and hypertension are other important etiologies. SAH in the setting of pituitary tumor can result from various causes. It can be due to intrinsic tumor related pathology, injury to surrounding the vessel during the operative procedure or due to an associated aneurysm. We discuss the pathological mechanisms and review relevant literature related to this interesting phenomenon. Early and accurate diagnosis of the cause of the SAH in pituitary tumors is important, as this influences the management.

  17. Surgical Treatment in Uveal Tumors

    Directory of Open Access Journals (Sweden)

    Kaan Gündüz


    Full Text Available Surgical treatment in uveal tumors can be done via iridectomy, partial lamellar sclerouvectomy (PLSU and endoresection. Iridectomy is done in iris tumors without angle and ciliary body involvement. PLSU is performed in tumors with ciliary body and choroidal involvement. For this operation, a partial thickness scleral flap is dissected, the intraocular tumor is excised, and the flap is sutured back in position. PLSU surgery is done in iridociliary and ciliary body tumors with less than 3 clock hours of iris and ciliary body involvement and in choroidal tumors with a base diameter less than 15 mm. However, it can be employed in any size tumor for biopsy purposes. Potential complications of PLSU surgery include vitreous hemorrhage, cataract, retinal detachment, and endophthalmitis. Endoresection is a technique whereby the intraocular tumor is excised using vitrectomy techniques. The rationale for performing endoresection is based on the fact that irradiated uveal melanomas may be associated with exudation and neovascular glaucoma and removing the dead tumor tissue may contribute to better visual outcome. There are some centers where endoresection is done without prior radiotherapy. Allegedly, avoidance of radiation retinopathy and papillopathy are the main advantages of using endoresection without prior radiotherapy. (Turk J Ophthalmol 2014; 44: Supplement 29-34

  18. Notch Signaling and Brain Tumors

    DEFF Research Database (Denmark)

    Stockhausen, Marie; Kristoffersen, Karina; Poulsen, Hans Skovgaard


    Human brain tumors are a heterogenous group of neoplasms occurring inside the cranium and the central spinal cord. In adults and children, astrocytic glioma and medulloblastoma are the most common subtypes of primary brain tumors. These tumor types are thought to arise from cells in which Notch...... signaling plays a fundamental role during development. Recent findings have shown that Notch signaling is dysregulated, and contributes to the malignant potential of these tumors. Growing evidence point towards an important role for cancer stem cells in the initiation and maintenance of glioma...

  19. Monitoring Radiographic Brain Tumor Progression

    Directory of Open Access Journals (Sweden)

    John H. Sampson


    Full Text Available Determining radiographic progression in primary malignant brain tumors has posed a significant challenge to the neuroncology community. Glioblastoma multiforme (GBM, WHO Grade IV through its inherent heterogeneous enhancement, growth patterns, and irregular nature has been difficult to assess for progression. Our ability to detect tumor progression radiographically remains inadequate. Despite the advanced imaging techniques, detecting tumor progression continues to be a clinical challenge. Here we review the different criteria used to detect tumor progression, and highlight the inherent challenges with detection of progression.

  20. Practice guidelines for tumor marker use in the clinic. (United States)

    Sturgeon, Catharine


    Increasing interest in implementing the practice of evidence-based medicine in oncology has encouraged the development of clinical guidelines, many of which include recommendations about the appropriate use of serum tumor markers. Recent national and international guidelines relating to the use of tumor markers in germ cell, colorectal, breast, ovarian, prostate, lung, neuroendocrine, and thyroid cancers were identified from the scientific literature and other sources and tabulated. Guideline recommendations developed by national and international groups and relating to the use of tumor markers for specific cancers are reviewed and compared, considering the recommendations made for their use in screening, diagnosis, prognosis, and monitoring of therapy. Potential advantages and disadvantages of clinical guidelines, how best to implement them, and means of auditing their effectiveness are also considered. Excellent clinical guidelines, including recommendations for the most appropriate use of tumor markers, are already available for many cancers. Many questions relating to optimal use of these important tests remain to be answered, but current guidelines already contain much valuable information and advice. Further dissemination and implementation of the guidelines should encourage better use of tumor markers in clinical practice. Careful audit studies are also required to establish the impact of these guidelines on the practice of evidence-based medicine.

  1. Connecting Genomic Alterations to Cancer Biology with Proteomics: The NCI Clinical Proteomic Tumor Analysis Consortium

    Energy Technology Data Exchange (ETDEWEB)

    Ellis, Matthew; Gillette, Michael; Carr, Steven A.; Paulovich, Amanda G.; Smith, Richard D.; Rodland, Karin D.; Townsend, Reid; Kinsinger, Christopher; Mesri, Mehdi; Rodriguez, Henry; Liebler, Daniel


    The National Cancer Institute (NCI) Clinical Proteomic Tumor Analysis Consortium is applying the latest generation of proteomic technologies to genomically annotated tumors from The Cancer Genome Atlas (TCGA) program, a joint initiative of the NCI and the National Human Genome Research Institute. By providing a fully integrated accounting of DNA, RNA, and protein abnormalities in individual tumors, these datasets will illuminate the complex relationship between genomic abnormalities and cancer phenotypes, thus producing biologic insights as well as a wave of novel candidate biomarkers and therapeutic targets amenable to verifi cation using targeted mass spectrometry methods.

  2. Pediatric Mediastinal Tumors and Tumor-Like Lesions. (United States)

    Singh, Achint K; Sargar, Kiran; Restrepo, Carlos S


    This article reviews the imaging findings of pediatric mediastinal tumors and tumor-like lesions. The classification of the mediastinum is discussed with normal imaging appearance of the thymus in pediatric age group followed by a discussion on multiple mediastinal lesions in different compartments with emphasis on their imaging characteristics.

  3. Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors (United States)


    Desmoplastic Small Round Cell Tumor; Ewing Sarcoma of Bone or Soft Tissue; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  4. Cathepsins mediate tumor metastasis

    Institute of Scientific and Technical Information of China (English)

    Gong-Jun; Tan; Zheng-Ke; Peng; Jin-Ping; Lu; Fa-Qing; Tang


    Cathepsins are highly expressed in various human cancers, associated with tumor metastasis. It is superfamily, concluding A, B, C, D, E, F, G, H, L, K, O, S, V, and W family members. As a group of lysosomal proteinases or endopeptidases, each member has a different function, playing different roles in distinct tumorigenic processes such as proliferation, angiogenesis, metastasis, and invasion. Cathepsins belong to a diverse number of enzyme subtypes, including cysteine proteases, serine proteases and aspartic proteases. The contribution of cathepsins to invasion in human cancers is well documented, although the precise mechanisms by which cathepsins exert their effects are still not clear. In the present review, the role of cathepsin family members in cancer is discussed.

  5. Solitary fibrous tumor

    Directory of Open Access Journals (Sweden)

    Manlio Mencoboni


    Full Text Available Solitary fibrous tumor (SFT is a rare mesenchymal neoplasm which may be found everywhere in the body. It is now distinguished into two forms, pleural and extrapleural, which morphologically resemble each other. Abdomi­nal localizations are quite rare, with 10 cases only reported in bladder; rarely they can be source of paraneoplastic syndromes (i.e., hypoglycemia secondary to insulin-like growth factor. In April 2006 a 74-year-old white male presented with chills, diaphoresis and acute abdominal pain with hematuria. At admission in emergency he underwent an abdominal X-ray (no pathological findings and an ultrasound examination of the kidneys and urinary tract, which revealed a pelvic hyperechogenic neoformation measuring approximately 10¥8¥7 cm, compressing the bladder. Blood chemistry at admission revealed only a mild neutrophilic leucocytosis (WBC 16600, N 80%, L 11%, elevated fibrinogen and ESR, and hypoglycemia (38 mg/dL. Macro­scopic hematuria was evident, while urinocolture was negative. Contrast enhanced CT scan of the abdomen and pelvic region revealed a large round neoformation dislocating the bladder, with an evident contrast-enhanced periphery and a central necrotic area. Continuous infusion of glucose 5% solution was necessary in order to maintain blood glucose levels above 50 mg/dL. The patient underwent complete surgical resection of an ovoidal mass coated by adipose tissue, with well delimited margins; histological findings were consistent with solitary fibrous tumor (SFT. Hypoglycemia resolved completely with removal of the growth. In this case report we describe a SFT growing in the bladder, a quite rare localization, which presented a unique hypoglycemia. In contrast to the majority of cases reported in the literature, the behavior of this SFT was not aggressive, and, since the patient is still alive, surgical resection was considered conclusive.

  6. Childhood brain tumor epidemiology: a brain tumor epidemiology consortium review. (United States)

    Johnson, Kimberly J; Cullen, Jennifer; Barnholtz-Sloan, Jill S; Ostrom, Quinn T; Langer, Chelsea E; Turner, Michelle C; McKean-Cowdin, Roberta; Fisher, James L; Lupo, Philip J; Partap, Sonia; Schwartzbaum, Judith A; Scheurer, Michael E


    Childhood brain tumors are the most common pediatric solid tumor and include several histologic subtypes. Although progress has been made in improving survival rates for some subtypes, understanding of risk factors for childhood brain tumors remains limited to a few genetic syndromes and ionizing radiation to the head and neck. In this report, we review descriptive and analytical epidemiology childhood brain tumor studies from the past decade and highlight priority areas for future epidemiology investigations and methodological work that is needed to advance our understanding of childhood brain tumor causes. Specifically, we summarize the results of a review of studies published since 2004 that have analyzed incidence and survival in different international regions and that have examined potential genetic, immune system, developmental and birth characteristics, and environmental risk factors. Cancer Epidemiol Biomarkers Prev; 23(12); 2716-36. ©2014 AACR.

  7. Computer aided tumor documentation, follow-up, and letter-writing to physicians in radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Leitner, H.; Hackl, A.; Gell, G.; Wagner, M.


    Without electronic data processing the recording and statistical evaluation of a great number of tumor patients in only hardly possible. A tumor register and follow-up program is described which has been established for radiotherapy during the development of a national tumor register organized at present. This tumor register also serves as a basis for letter-writing to physicians. The software can easily be adapted to the needs of every hospital because a commercial data bank system has been used. The data input and output is carried out over several terminals. The hardware and software system as well as the incorporation of the data bank into the national tumor register are described.

  8. Management of childhood brain tumors: consensus report by the Pediatric Hematology Oncology (PHO) Chapter of Indian Academy of Pediatrics (IAP). (United States)

    Bhat, Sunil; Yadav, Satya Prakash; Suri, Vaishali; Patir, Rana; Kurkure, Purna; Kellie, Stewart; Sachdeva, Anupam


    Brain tumors are the second most common childhood tumors and remain the leading cause of cancer related deaths in children. Appropriate diagnosis and management of these tumors are essential to improve survival. There are no clinical practical guidelines available for the management of brain tumors in India. This document is a consensus report prepared after a National Consultation on Pediatric Brain Tumors held in Delhi on 06 Nov 2008. The meeting was attended by eminent experts from all over the country, in the fields of Neurosurgery, Radiation Oncology, Pediatric Oncology, Neuropathology, Diagnostic Imaging, Pediatric Endocrinology and Allied Health Professionals. This article highlights that physicians looking after children with brain tumors should work as part of a multidisciplinary team to improve the survival, quality of life, neuro-cognitive outcomes and standards of care for children with brain tumors. Recommendations for when to suspect, diagnostic workup, initial management, long-term follow up and specific management of individual tumors are outlined.

  9. National medalist (United States)

    President Clinton has chosen eight prominent American scientists to receive the 1994 National Medal of Science, the nation's highest scientific honor. Frank Press, an AGU member and geophysicist at the Carnegie Institution of Washington, numbers among the eight science medalists who will receive their medals in October, according to an announcement last week by Jack Gibbons, assistant to the president for science and technology. The president chose the other seven recipients from the fields of structural engineering, organic photochemistry, theoretical physics, biology, computer science, sociology, and biochemistry.

  10. [Local treatment of liver tumors

    DEFF Research Database (Denmark)

    Pless, T.K.; Skjoldbye, Bjørn Ole


    Local treatment of non-resectable liver tumors is common. This brief review describes the local treatment techniques used in Denmark. The techniques are evaluated according to the evidence in literature. The primary local treatment is Radiofrequency Ablation of both primary liver tumors and liver...

  11. Tumors STING adaptive antitumor immunity. (United States)

    Bronte, Vincenzo


    Immunotherapy is revolutionizing the treatment of cancer patients, but the molecular basis for tumor immunogenicity is unclear. In this issue of Immunity, Deng et al. (2014) and Woo et al. (2014) provide evidence suggesting that dendritic cells detect DNA from tumor cells via the STING-mediated, cytosolic DNA sensing pathway.

  12. Tumors of the optic nerve

    DEFF Research Database (Denmark)

    Lindegaard, Jens; Heegaard, Steffen


    A variety of lesions may involve the optic nerve. Mainly, these lesions are inflammatory or vascular lesions that rarely necessitate surgery but may induce significant visual morbidity. Orbital tumors may induce proptosis, visual loss, relative afferent pupillary defect, disc edema and optic...... tumor-specific histologic features are given. Finally, treatment modalities and prognosis are discussed....

  13. Management of salivary gland tumors

    NARCIS (Netherlands)

    Andry, Guy; Hamoir, Marc; Locati, Laura D.; Licitra, Lisa; Langendijk, Johannes A.


    Surgery after proper imaging (MRI or CT scan) is the main stay of treatment for salivary gland tumors. Although excision margins should be >= 5 mm for malignant tumors in cases of parotid gland carcinoma, the facial nerve should be preserved whenever it is not infiltrated. Adjuvant external radiatio

  14. Surgical strategies in endocrine tumors

    NARCIS (Netherlands)

    Schreinemakers, J.M.J.


    Endocrine surgery has become more custom-made throughout the years. Endocrine tumors can be sporadic or develop as part of familial syndromes. Several familial syndromes are known to cause endocrine tumors. The most common are multiple endocrine neoplasia (MEN) syndromes type 1, 2A and 2B. This

  15. Brain Tumor Epidemiology Consortium (BTEC) (United States)

    The Brain Tumor Epidemiology Consortium is an open scientific forum organized to foster the development of multi-center, international and inter-disciplinary collaborations that will lead to a better understanding of the etiology, outcomes, and prevention of brain tumors.

  16. Primitive neuroectodermal tumor: CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Chang Soo; Eun, Tchoong Kie; Cha, Seong Sook; Han, Sang Suk; Chung, Duck Hwan [Inje College, Paik Hospital, Busan (Korea, Republic of)


    Primitive neuroectodermal tumor is a neoplasm of young individuals that occurs predominantly in the supratentorial compartment. Authors experienced three cases of primitive neuroectodermal tumor. On the brain CT scans, all three cases reveal the large, irregular, hyperdense mass lesions with calcifications and cystic or necrotic areas, and show dense heterogeneous contrast enhancement.

  17. [Intraoperative staging of colorectal tumors]. (United States)

    Abdurakhmonov, Iu B; Mel'nikov, O R; Egorenkov, V V; Moiseenko, V M


    The effectiveness of intraoperative staging of tumor by sentinel node staining with lymphotropic dyes was evaluated in 60 patients with colorectal tumors (colon carcinoma -39, rectal cancer- 21). High sensitivity (84.6% and 87.5%, respectively) and specificity (100% and 100%, respectively) for regional lymph node assessment were identified for both colonic and rectal cancer.


    Directory of Open Access Journals (Sweden)



    Full Text Available Untuk meningkatkan kelangsungan hidup penderita kanker ovarium, dilakukan upaya untukmemprediksi keganasan tumor tersebut sebelum dilakukan pembedahan, karena adanya perbedaanpenanganan pada tumor jinak dan kanker ovarium. Terdapat berbagai modalitas untuk mendeteksikeganasan tumor ovarium prabedah. Mulai dari pemeriksaan klinis melalui anamnesis danpemeriksaan fisik, serta pemeriksaan penunjang seperti petanda tumor dan ultrasonografi. Untukmeningkatkan akurasi dalam mendeteksi keganasan tumor ovarium prabedah, dibuat berbagai sistemskoring. Indeks keganasan Sudaryanto, memakai batasan total skor e” 3 menunjukkan risiko keganasantinggi, sedangkan total skor < 3 menunjukkan risiko keganasan rendah. Melalui pemeriksaan USG,dibuat sistem skoring morfologi berdasarkan struktur permukaan dalam tumor, ketebalan dinding,septa, dan echogenitas tumor yang disebut indeks morfologi Sassone-Timor Tritsch. Denganmenggunakan batas skor 9, ditetapkan skor < 9 menunjukkan prediksi jinak dan skor e” 9 menunjukkanprediksi ganas. Batasan ini memiliki sensitifitas 94%, spesifisitas 87%, nilai duga positif 60%, dannilai duga negatif 93,6%. Metode lainnya adalah Risk of Malignancy Index (RMI. RMI mengintegrasikanstatus menopause penderita, temuan USG, dan kadar CA 125 serum. Nilai cut-off 200 digunakanuntuk membedakan tumor ovarium yang jinak dan ganas serta mempunyai sensitifitas 87% danspesifisitas 97%. The Risk of Ovarian Malignancy Algorithm (ROMA merupakan upaya koreksi RMIdengan menambahkan biomarker human protein epididymis 4 (HE4 dan menghilangkan USG. Untukmemprediksi kanker ovarium tipe epitel, ROMA mempunyai sensitifitas dan spesifisitas masingmasingsebesar 89% dan 83%. Jadi, terdapat berbagai modalitas untuk memprediksi keganasantumorovarium dengan akurasi berbeda-beda. Penerapannya disesuaikan dengan sarana dan prasaranayangtersedia sesuai dengan kondisi tempat pelayanan kesehatan.

  19. Tumor microenvironment and therapeutic response. (United States)

    Wu, Ting; Dai, Yun


    The tumor microenvironment significantly influences therapeutic response and clinical outcome. Microenvironment-mediated drug resistance can be induced by soluble factors secreted by tumor or stromal cells. The adhesion of tumor cells to stromal fibroblasts or to components of the extracellular matrix can also blunt therapeutic response. Microenvironment-targeted therapy strategies include inhibition of the extracellular ligand-receptor interactions and downstream pathways. Immune cells can both improve and obstruct therapeutic efficacy and may vary in their activation status within the tumor microenvironment; thus, re-programme of the immune response would be substantially more beneficial. The development of rational drug combinations that can simultaneously target tumor cells and the microenvironment may represent a solution to overcome therapeutic resistance. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  20. [Radiological evaluation of congenital tumors]. (United States)

    Aguado del Hoyo, A; Ruiz Martín, Y; Lancharro Zapata, Á; Marín Rodríguez, C; Gordillo Gutiérrez, I


    In this article, we consider tumors that are diagnosed during pregnancy or in the first three months of life. This is a heterogeneous group of neoplasms with special biological and epidemiological characteristics that differentiate them from tumors arising in children or adults. In the last two decades, the prenatal detection of congenital tumors has increased due to the generalized use of prenatal sonographic screening. Advances in imaging techniques, especially in fetal magnetic resonance imaging, have enabled improvements in the diagnosis, follow-up, clinical management, and perinatal treatment of these tumors. This image-based review of the most common congenital tumors describes their histologic types, locations, and characteristics on the different imaging techniques used.

  1. Benign mixed tumor of the lacrimal sac

    Directory of Open Access Journals (Sweden)

    Jong-Suk Lee


    Full Text Available Neoplasms of the lacrimal drainage system are uncommon, but potentially life-threatening and are often difficult to diagnose. Among primary lacrimal sac tumors, benign mixed tumors are extremely rare. Histologically, benign mixed tumors have been classified as a type of benign epithelial tumor. Here we report a case of benign mixed tumor of the lacrimal sac.

  2. Pathogenesis and progression of fibroepithelial breast tumors

    NARCIS (Netherlands)

    Kuijper, Arno


    Fibroadenoma and phyllodes tumor are fibroepithelial breast tumors. These tumors are biphasic, i.e. they are composed of stroma and epithelium. The behavior of fibroadenomas is benign, whereas phyllodes tumors can recur and even metastasize. Classification criteria for both tumors show considerable

  3. The history of tumor virology. (United States)

    Javier, Ronald T; Butel, Janet S


    In the century since its inception, the field of tumor virology has provided groundbreaking insights into the causes of human cancer. Peyton Rous founded this scientific field in 1911 by discovering an avian virus that induced tumors in chickens; however, it took 40 years for the scientific community to comprehend the effect of this seminal finding. Later identification of mammalian tumor viruses in the 1930s by Richard Shope and John Bittner, and in the 1950s by Ludwik Gross, sparked the first intense interest in tumor virology by suggesting the possibility of a similar causal role for viruses in human cancers. This change in attitude opened the door in the 1960s and 1970s for the discovery of the first human tumor viruses--EBV, hepatitis B virus, and the papillomaviruses. Such knowledge proved instrumental to the development of the first cancer vaccines against cancers having an infectious etiology. Tumor virologists additionally recognized that viruses could serve as powerful discovery tools, leading to revolutionary breakthroughs in the 1970s and 1980s that included the concept of the oncogene, the identification of the p53 tumor suppressor, and the function of the retinoblastoma tumor suppressor. The subsequent availability of more advanced molecular technologies paved the way in the 1980s and 1990s for the identification of additional human tumor viruses--human T-cell leukemia virus type 1, hepatitis C virus, and Kaposi's sarcoma virus. In fact, current estimates suggest that viruses are involved in 15% to 20% of human cancers worldwide. Thus, viruses not only have been shown to represent etiologic agents for many human cancers but have also served as tools to reveal mechanisms that are involved in all human malignancies. This rich history promises that tumor virology will continue to contribute to our understanding of cancer and to the development of new therapeutic and preventive measures for this disease in the 21st century.

  4. Sports Nation

    Institute of Scientific and Technical Information of China (English)


    China is working to become a global sports power International Olympic Committee President Jacques Rogge watched China’s 11th National Games at Jinan’s stadium in Shandong Province on October 16, 2009. His high-profile attendance has set the

  5. Nations Within. (United States)

    Sheets, Rosa Hernandez


    Karen Gayton Swisher (Sioux), chair of teacher education at Haskell Indian Nations University (Kansas), discusses the history of American Indian education, its effects on Indian families and cultures, overcoming stereotypes, how teachers can teach Indian children more effectively, the importance of Indian interpretations of Indian education and…

  6. Happy Nation

    DEFF Research Database (Denmark)

    Baron, Christian

    Happy Nation er et stykke eksperimentel teknologiformidling, der er udformet som en skønlitterær roman. Værket tager udgangspunkt i et fremtidsscenarie, hvor virtual reality er blevet en hverdagsteknologi, hvis sansedel bliver understøttet af implantater, der kan foretage dyb hjernestimulation...

  7. Nations Within. (United States)

    Sheets, Rosa Hernandez


    Karen Gayton Swisher (Sioux), chair of teacher education at Haskell Indian Nations University (Kansas), discusses the history of American Indian education, its effects on Indian families and cultures, overcoming stereotypes, how teachers can teach Indian children more effectively, the importance of Indian interpretations of Indian education and…

  8. Establishment of HLA-DR4 transgenic mice for the identification of CD4+ T cell epitopes of tumor-associated antigens.

    Directory of Open Access Journals (Sweden)

    Junji Yatsuda

    Full Text Available Reports have shown that activation of tumor-specific CD4(+ helper T (Th cells is crucial for effective anti-tumor immunity and identification of Th-cell epitopes is critical for peptide vaccine-based cancer immunotherapy. Although computer algorithms are available to predict peptides with high binding affinity to a specific HLA class II molecule, the ability of those peptides to induce Th-cell responses must be evaluated. We have established HLA-DR4 (HLA-DRA*01:01/HLA-DRB1*04:05 transgenic mice (Tgm, since this HLA-DR allele is most frequent (13.6% in Japanese population, to evaluate HLA-DR4-restricted Th-cell responses to tumor-associated antigen (TAA-derived peptides predicted to bind to HLA-DR4. To avoid weak binding between mouse CD4 and HLA-DR4, Tgm were designed to express chimeric HLA-DR4/I-E(d, where I-E(d α1 and β1 domains were replaced with those from HLA-DR4. Th cells isolated from Tgm immunized with adjuvant and HLA-DR4-binding cytomegalovirus-derived peptide proliferated when stimulated with peptide-pulsed HLA-DR4-transduced mouse L cells, indicating chimeric HLA-DR4/I-E(d has equivalent antigen presenting capacity to HLA-DR4. Immunization with CDCA155-78 peptide, a computer algorithm-predicted HLA-DR4-binding peptide derived from TAA CDCA1, successfully induced Th-cell responses in Tgm, while immunization of HLA-DR4-binding Wilms' tumor 1 antigen-derived peptide with identical amino acid sequence to mouse ortholog failed. This was overcome by using peptide-pulsed syngeneic bone marrow-derived dendritic cells (BM-DC followed by immunization with peptide/CFA booster. BM-DC-based immunization of KIF20A494-517 peptide from another TAA KIF20A, with an almost identical HLA-binding core amino acid sequence to mouse ortholog, successfully induced Th-cell responses in Tgm. Notably, both CDCA155-78 and KIF20A494-517 peptides induced human Th-cell responses in PBMCs from HLA-DR4-positive donors. Finally, an HLA-DR4 binding DEPDC1191

  9. Improving drug delivery to solid tumors: priming the tumor microenvironment. (United States)

    Khawar, Iftikhar Ali; Kim, Jung Ho; Kuh, Hyo-Jeong


    Malignant transformation and growth of the tumor mass tend to induce changes in the surrounding microenvironment. Abnormality of the tumor microenvironment provides a driving force leading not only to tumor progression, including invasion and metastasis, but also to acquisition of drug resistance, including pharmacokinetic (drug delivery-related) and pharmacodynamic (sensitivity-related) resistance. Drug delivery systems exploiting the enhanced permeability and retention (EPR) effect and active targeting moieties were expected to be able to cope with delivery-related drug resistance. However, recent evidence supports a considerable barrier role of tumors via various mechanisms, which results in imperfect or inefficient EPR and/or targeting effect. The components of the tumor microenvironment such as abnormal tumor vascular system, deregulated composition of the extracellular matrix, and interstitial hypertension (elevated interstitial fluid pressure) collectively or cooperatively hinder the drug distribution, which is prerequisite to the efficacy of nanoparticles and small-molecule drugs used in cancer medicine. Hence, the abnormal tumor microenvironment has recently been suggested to be a promising target for the improvement of drug delivery to improve therapeutic efficacy. Strategies to modulate the abnormal tumor microenvironment, referred to here as "solid tumor priming" (vascular normalization and/or solid stress alleviation leading to improvement in blood perfusion and convective molecular movement), have shown promising results in the enhancement of drug delivery and anticancer efficacy. These strategies may provide a novel avenue for the development of new chemotherapeutics and combination chemotherapeutic regimens as well as reassessment of previously ineffective agents. Copyright © 2014 Elsevier B.V. All rights reserved.

  10. A New Type of Uterine Trophoblastic Tumor: Epithelioid Trophoblastic Tumor

    Institute of Scientific and Technical Information of China (English)

    Langdi Fan; Zhanhong Wang; Xiurong Wang; Yingge Xing


    OBJECTIVE To describe a case of a patient with an epithelioid trophoblastic tumor (ETT) and review the literature regarding this new type of uterine trophoblastic tumor which is being reported with increasing frequency.There have been only 42 cases described in the world literature.METHODS Routine sections of the tumor were prepared and stained with H&E. Using the SP method, immunohistochemical staining, for AE1/AE3,hPL, PLAP, and α-inhibin antigens was conducted.RESULTS The patient was a 34 years old female who had delivered 12 years previously. She presented with amenorrhea for three months and vaginal irregular bleeding for 2 months. Her serum hCG level was 2,240 IU/L. After diagnostic curettage, an ETT was identified, and total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAHBSO) performed.On microscopic examination it was found that the tumor was composed of chorionic-type intermediate- trophoblastic cells. The tumor cell nests were distributed in a geographical pattern. Some cells were filled with an eosinophilic hyalinized degenerative material. Study of the immunophenotype of the tumor showed that AE1/AE3, hPL, hCG, and α-inhibin were positively expressed.CONCLUSION This is the 4th case report of an ETT in China. The tumor was identified as a new type of trophoblastic tumor combined with a focal chorioepithelioid carcinoma, a condition that is extremely rare. It consists of chorionic-type intermediate-trophoblastic cells, and is considered to have a Iowgrade of malignancy. ETT should be differentiated from a placenta-site trophoblastic tumor, placenta-site nodule, chorioepithelioid carcinoma, and cervical squamous cell carcinoma.

  11. IMRT in hypopharyngeal tumors

    Energy Technology Data Exchange (ETDEWEB)

    Studer, G.; Luetolf, U.M.; Davis, J.B.; Glanzmann, C. [Dept. of Radiation Oncology, Univ. Hospital, Zurich (Switzerland)


    Background and purpose: intensity-modulated radiation therapy (IMRT) data on hypopharyngeal cancer (HC) are scant. In this study, the authors report on early results in an own HC patient cohort treated with IMRT. A more favorable outcome as compared to historical data on conventional radiation techniques was expected. Patients and methods: 29 consecutive HC patients were treated with simultaneous integrated boost (SIB) IMRT between 01/2002 and 07/2005 (mean follow-up 16 months, range 4-44 months). Doses of 60-71 Gy with 2.0-2.2 Gy/fraction were applied. 26/29 patients were definitively irradiated, 86% received simultaneous cisplatin-based chemotherapy. 60% presented with locally advanced disease (T3/4 Nx, Tx N2c/3). Mean primary tumor volume measured 36.2 cm{sup 3} (4-170 cm{sup 3}), mean nodal volume 16.6 cm{sup 3} (0-97 cm{sup 3}). Results: 2-year actuarial local, nodal, distant control, and overall disease-free survival were 90%, 93%, 93%, and 90%, respectively. In 2/4 patients with persistent disease (nodal in one, primary in three), salvage surgery was performed. The mean dose to the spinal cord (extension of > 5-15 mm) was 26 Gy (12-38 Gy); the mean maximum (point) dose was 44.4 Gy (26-58.9 Gy). One grade (G) 3 dysphagia and two G4 reactions (laryngeal fibrosis, dysphagia), both following the schedule with 2.2 Gy per fraction, have been observed so far. Larynx preservation was achieved in 25/26 of the definitively irradiated patients (one underwent a salvage laryngectomy); 23 had no or minimal dysphagia (G0-1). Conclusion: excellent early disease control and high patient satisfaction with swallowing function in HC following SIB IMRT were observed; these results need to be confirmed based on a longer follow-up period. In order to avoid G4 reactions, SIB doses of < 2.2 Gy/fraction are recommended for large tumors involving laryngeal structures. (orig.)

  12. Initiative action of tumor-associated macrophage during tumor metastasis

    Directory of Open Access Journals (Sweden)

    Saroj Singh


    In this review article, we present an overview of mechanisms responsible for TAMs recruitment and highlight the roles of TAMs in the regulation of tumor angiogenesis, invasion, metastasis, immunosuppression, and chemotherapeutic resistance. We describe the interplay between Th17 cells and other immune cells in the tumor microenvironment, and we assess both the potential antitumorigenic and pro-tumorigenic activities of Th17 cells and their associated cytokines. Understanding the nature of Th17 cell responses in the tumor microenvironment will be important for the design of more efficacious cancer immunotherapies. Finally, we discuss TAM-targeting therapy as a promising novel strategy for an indirect cancer therapy.

  13. SIRT1, Is It a Tumor Promoter or Tumor Suppressor?

    Directory of Open Access Journals (Sweden)

    Chu-Xia Deng


    Full Text Available SIRT1 has been considered as a tumor promoter because of its increased expression in some types of cancers and its role in inactivating proteins that are involved in tumor suppression and DNA damage repair. However, recent studies demonstrated that SIRT1 levels are reduced in some other types of cancers, and that SIRT1 deficiency results in genetic instability and tumorigenesis, while overexpression of SIRT1 attenuates cancer formation in mice heterozygous for tumor suppressor p53 or APC. Here, I review these recent findings and discuss the possibility that activation of SIRT1 both extends lifespan and inhibits cancer formation.

  14. Tumor cerebral e gravidez Brain tumors and pregnancy

    Directory of Open Access Journals (Sweden)

    José Carlos Lynch


    Full Text Available O diagnóstico de um tumor cerebral durante a gravidez é um fato raro que coloca a mãe e o concepto em risco de vida. OBJETIVO: Avaliar a melhor forma de conduzir uma paciente grávida portadora de um tumor cerebral. MÉTODO: Realizamos análise retrospectiva dos prontuários e imagens de seis pacientes grávidas portadoras de tumor cerebral. RESULTADOS: Vários tipos histológicos de tumor cerebral podem estar associados à gravidez. O meningioma é o mais freqüente. Nessa série não observamos óbito cirúrgico materno. Em duas pacientes, o parto ocorreu antes da craniotomia e em outras quatro o parto foi realizado após a neurocirurgia. CONCLUSÃO: O momento mais adequado para a realização da craniotomia para remoção tumoral irá depender da gravidade do quadro neurológico, do tipo histológico presumível da lesão, e da idade gestacional do embrião.BACKGROUND: Despite not being a common fact, the occurrence of brain tumors during pregnancy poses a risk to both the mother and infant. AIM: To identify the best medical procedure to be followed for a pregnant patient harboring a brain tumor. METHOD: The records of 6 patients with brain tumors, diagnosed during pregnancy were examined. RESULTS: Several types of brain tumors have been associated with pregnancy, but the meningioma is, by far, the most frequent. It seems that pregnancy aggravates the clinical course of intracranial tumors. There were no operative mortality in these series. In 2 patients the labor occurred before the craniotomy and in others, the delivery occurred after the surgery. CONCLUSION: The best moment to recommend the craniotomy and the neurosurgical removal of the tumor will depend of the mother’s neurological condition, the tumor histological type as well as the gestational age.

  15. Extra-Abdominal Desmoid Tumors (Aggressive Fibromatoses) (United States)

    ... Centers Broken Bones & Injuries Diseases & Conditions Arthritis Tumors Sports Injuries & Prevention Children Bone Health Health & Safety Treatment ... tissue. Because of this, they are also called aggressive fibromatoses. The tumors ... erratic behavior makes treatment challenging. Treatment for a tumor depends ...

  16. Cushing syndrome due to adrenal tumor (United States)

    Adrenal tumor - Cushing syndrome ... Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the ... or cancerous (malignant). Noncancerous tumors that can cause ... Adrenal adenomas Micronodular hyperplasia Cancerous tumors that ...

  17. What Are the Symptoms of Pituitary Tumors? (United States)

    ... Clinical Trials Resources and Publications What are the symptoms of pituitary tumors? Skip sharing on social media links Share this: Page Content Symptoms of Functioning Tumors The symptoms of functioning tumors ...

  18. Tumor targeting via integrin ligands

    Directory of Open Access Journals (Sweden)

    Udaya Kiran eMarelli


    Full Text Available Selective and targeted delivery of drugs to tumors is a major challenge for an effective cancer therapy and also to overcome the side effects associated with current treatments. Overexpression of various receptors on tumor cells is a characteristic structural and biochemical aspect of tumors and distinguishes them from physiologically normal cells. This abnormal feature is therefore suitable for selectively directing anticancer molecules to tumors by using ligands that can preferentially recognize such receptors. Several subtypes of integrin receptors that are crucial for cell adhesion, cell signaling, cell viability and motility have been shown to have an upregulated expression on cancer cells. Thus, ligands that recognize specific integrin subtypes represent excellent candidates to be conjugated to drugs or drug carrier systems and be targeted to tumors. In this regard, integrins recognizing the RGD cell adhesive sequence have been extensively targeted for tumor specific drug delivery. Here we review key recent examples on the presentation of RGD-based integrin ligands by means of distinct drug delivery systems, and discuss the prospects of such therapies to specifically target tumor cells.

  19. Laser therapy in ocular tumors (United States)

    Carstocea, Benone D.; Gafencu, Otilia L.; Apostol, Silvia; Ionita, Marcel A.; Moroseanu, A.; Dascalu, Traian; Lupei, Voicu; Ionita-Manzatu, V.


    The medical laser equipments made at NILPRP have been exploited intensively for more than 10 years at CMH. The availability and reliability of the first like-on equipment have increased, following improvements in optical delivery system and cooling circuit. This paper shows the impact of technical advances on the development of ophthalmologic laser therapy. Intraocular tumors pose special problems of diagnosis and treatment. Diagnostic methods include addition to systemic and ophthalmologic examinations, ancillary examinations, such as transillumination, fluorescence angiography, ultrasonography, radioactive phosphorus uptake tests, radiology, computerized tomography and fine-needle aspiration biopsy with cytological analyses. The enucleation of the involved eye used to be a generally accepted management of malignant tumors. Improved therapeutic methods such as photocoagulation and better surgical techniques now provide a variety of therapeutic alternatives. This study covers 31 cases of intraocular tumors that were managed either by Argon Laser photocoagulation and/or by Nd:YAG laser surgical treatment. Four cases were intraocular metastasse and 17 cases were primitive intraocular tumors. Argon laser therapy proved to be totally ineffective for intraocular metastasse but very adequate therapy for primitive tumors. Tumor extirpations (choroidal, cillary body or iris tumors) using Nd:YAG laser lancet proved to be more suitable than classic surgery.

  20. [Orbital tumor emergencies in childhood]. (United States)

    Morax, S; Desjardins, L


    Emergencies in childhood orbital tumorals are rare. The absolute emergency involves malignant primary orbital tumors, such as rhabdomyosarcoma or secondary malignant tumors (metastatic neuroblastoma, leukemia), involving a vital prognosis requiring prompt diagnosis. Delayed emergencies are usually vascular lesions. Among these lesions, immature orbital hemangioma, with a good prognosis, must be distinguished from orbital adnexal lymphangiomas, which are less frequent but can lead to dramatic cosmetic and functional disorders. In rare cases, they can be responsible for sudden, painful proptosis, due to orbital hemorrhage, with a risk of optic nerve compression, requiring emergency surgical treatment. Neurogenous lesions, either isolated, such as in gliomas, or associated with a systemic disease, such as Recklinghausen neurofibromatosis, threaten the functional prognosis. Diagnosis of pediatric orbital tumors is based on a good clinical examination, precise imaging investigations, and evaluation of the locoregional extension of the tumor. Biopsy is required in emergency situations, when rhabdomyosarcoma is suspected, in order to start the chemotherapy. However, the biopsy can be superfluous, and even useless or dangerous, when clinical and imaging investigations are sufficient to provide a diagnosis of capillary hemangioma, lymphangioma, or metastatic tumor from an abdominal malignancy. Treatment is closely related to the etiopathogenesis of the tumor. The outcomes are vital, functional and cosmetic. They may require orbital surgery (biopsy, tumoral resection, orbital decompression in case of a compressive hemorrhage), systemic corticotherapy (as in immature adnexal hemangioma), radiation, and chemotherapy (rhabdomyosarcoma, secondary malignant tumor). These diseases require a pediatric ophthalmological medical center specializing in orbital surgery, with close collaboration of multiple specialists such as onco-pediatricians and neurosurgeons.