cancernet@cancerweb.org.uk 1 UI - 21119949 AU - Baskin LS TI - Case 1. Abdominal mass. A 1-year-old with a left abdominal mass and gross hematuria. SO - Tech Urol 2001 Mar;7(1):41; discussion 76 AD - University of California San Francisco, USA. 2 UI - 21285467 AU - Lin F; Krishnamurthy S TI - Fine needle aspiration cytology of a skeletal metastasis of adult Wilms' tumor. A case report. SO - Acta Cytol 2001 May-Jun;45(3):393-8 AD - Department of Pathology, University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030, USA. AB - BACKGROUND: Nephroblastoma (Wilms' tumor) is the most common malignant tumor of the kidney in children but is rare in adults. The stage and histopathology of the tumor are the most important prognostic indicators. The common sites of metastasis are lung, liver and lymph nodes. Skeletal metastasis is exceedingly rare in both pediatric and adult nephroblastoma. We report an unusual case of a skeletal metastasis of adult nephroblastoma that developed nine years after the diagnosis of a typical nephroblastoma of favorable histology and that was diagnosed by computed tomography (CT)-guided fine needle aspiration cytology. CASE: Following a right radical nephrectomy for adult nephroblastoma and two local recurrences two and three years later, a 74-year-old woman presented with low back pain. CT and magnetic resonance imaging revealed lytic lesions in the 10th and 12th thoracic vertebrae. Smears prepared from specimens obtained through CT-guided fine needle aspiration biopsy were moderately cellular, with small, round cells arranged singly and in loosely cohesive clusters. These cells had inconspicuous nucleoli and scanty to moderate amounts of cytoplasm. The cells were also positive for cytokeratin and vimentin and appeared similar to areas of blastema in the original tumor. CONCLUSION: A definitive diagnosis of metastatic adult nephroblastoma in thoracic vertebrae was made possible by CT-guided fine needle aspiration cytology in conjunction with clinical and radiologic findings and by using ancillary modalities, such as immunohistochemical studies. 3 UI - 21310635 AU - Chen CW; Huang SP; Li YC; Chou YH; Huang CH TI - Adult Wilms' tumor associated with polycythemiaa case report. SO - Kaohsiung J Med Sci 2001 Feb;17(2):107-11 AD - Department of Urology, Kaohsiung Medical University, No. 100, Shih-Chuan 1st Road, Kaohsiung 807, Taiwan. AB - Both Wilms' tumor (nephroblastoma) in adults and polycythemia in Wilms' tumor are rare. Herein we report an extremely rare case of adult Wilms' tumor associated with polycythemia. A 41-year-old female was incidentally found to have right renal mass by abdominal sonography in a routine health examination. Laboratory examination revealed polycythemia (hemoglobin 20.2 g/dL). Although physical examination was unremarkable, CT scan revealed an homogeneous mass at the middle pole of right kidney, and chest x-ray revealed no metastatic lesions. Right radical nephrectomy was performed smoothly. Grossly, the tumor of 5 x 4.5 x 4.5 cm in size was well circumscribed, and had no vascular structure or collecting system involvement. Microscopic features were consistent with adult nephroblastoma. The post-operative course was uneventful. At follow-up, the patient was well, showed no evidence of recurrence and her hemoglobin level had returned to normal (hemoglobin 14.5 g/dl). We suggest that the relationship between polycythemia and Wilms' tumor should be carefully evaluated before surgical treatment. 4 UI - 21301299 AU - Sandoval C; Ozkaynak MF; Tugal O; Jayabose S TI - Hyperdiploidy in a case of favorable histology Wilms tumor. SO - Cancer Genet Cytogenet 2001 May;127(1):89-90 AD - Department of Pediatrics, New York Medical College, Munger Pavilion Room 110, Valhalla, NY 10595, USA. AB - Hyperdiploidy is useful in defining histologic variants of Wilms tumor and prognosis in other childhood cancers. We describe a case of hyperdiploid favorable histology Wilms tumor (50,XY,+6,+7,+8,+10,+12,-21[2]/51,idem,+9[6]/46,XY[12]) in a 3-year-old boy, and review the literature for other hyperdiploid childhood renal lesions. 5 UI - 21137121 AU - Rommel D; Pirson Y TI - Medullary sponge kidneypart of a congenital syndrome. SO - Nephrol Dial Transplant 2001 Mar;16(3):634-6 AD - Cliniques Universitaires St-Luc, Department of Nephrology, Universite Catholique de Louvain, Brussels, Belgium. 6 UI - 21324305 AU - Cui H; Niemitz EL; Ravenel JD; Onyango P; Brandenburg SA; Lobanenkov VV; Feinberg AP TI - Loss of imprinting of insulin-like growth factor-II in Wilms' tumor commonly involves altered methylation but not mutations of CTCF or its binding site. SO - Cancer Res 2001 Jul 1;61(13):4947-50 AD - Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA. AB - Loss of imprinting (LOI) is the most common molecular abnormality in Wilms' tumor (WT), other embryonal cancers, and most other tumor types. LOI in WT involves activation of the normally silent maternal allele of the insulin-like growth factor-II (IGF2) gene, silencing of the normally active maternal allele of the H19 gene, and aberrant methylation of a differentially methylated region (DMR) upstream of the maternal copy of H19. Recently, the transcription factor CTCF, which binds to the H19 DMR, has been implicated in the maintenance of H19 and IGF2 imprinting. Here, we show that mutations in the CTCF gene or in the H19 DMR do not occur at significant frequency in WT, nor is there transcriptional silencing of CTCF. We also confirm that methylation of the H19 DMR in WT with LOI includes the CTCF core consensus site. However, some WTs with normal imprinting of IGF2 also show aberrant methylation of CTCF binding sites, indicating that methylation of these sites is necessary but not sufficient for LOI in WT. 7 UI - 21319785 AU - Korones DN; Brown MR; Palis J TI - "Liver function tests" are not always tests of liver function. SO - Am J Hematol 2001 Jan;66(1):46-8 AD - Department of Pediatrics, University of Rochester, School of Medicine and Dentistry, Children's Hospital at Strong, New York 14642, USA. david-korones@urmc.rochester.edu AB - A child with Wilm's tumor and a child with immune thrombocytopenic purpura (ITP) were each noted to have persistent elevations of aspartate aminotransferase (AST), alanine aminotransferase (ALT), and lactate dehydrogenase (LDH). Both children underwent thorough evaluation for liver disease and, as a result, experienced delays in treatment of the Wilm's tumor and ITP. Eventually both children were found to have extremely elevated serum creatine kinase (CK). Muscle biopsy confirmed diagnoses of Duchenne's muscular dystrophy in one child, and Becker's muscular dystrophy in the second. Hematologists/oncologists should consider obtaining a serum CK to rule out muscle disease in patients with unexplained elevations of AST, ALT, and LDH. 8 UI - 21345515 AU - Hogeboom CJ; Grosser SC; Guthrie KA; Thomas PR; D'Angio GJ; Breslow NE TI - Stature loss following treatment for Wilms tumor. SO - Med Pediatr Oncol 2001 Feb;36(2):295-304 AD - Department of Biostatistics, University of California-San Francisco, USA. AB - BACKGROUND: The study was designed to estimate reduction in adult stature induced by megavoltage radiation therapy (RT) of the spine in children treated for Wilms tumor and to ascertain whether the dose reduction in successive National Wilms Tumor Study Group (NWTSG) trials has mitigated late effects of RT in these children. PROCEDURE: Effects of RT dose, age at treatment, and chemotherapy on stature of 2,778 children with Wilms or another solid tumor of the kidney were analyzed using statistical models accounting for the dependence of height on gender and advancing age. Model predictions were validated by descriptive analysis of heights measured at 17 to 18 years of age for 205 patients. RESULTS: Radiation-induced reductions below normal height depended on dose, portal size, and age at treatment and were not augmented by doxorubicin or cyclophosphamide. Younger children were more strongly affected. Predicted height deficit at age 18 years was 1.8 cm for a child treated with 10 Gy to the flank at age 4 years. Observed height deficits at age 1 7 to 18 years were 4.1 cm for 57 patients who received 15-24 Gy at a mean age of 55 months and zero for 16 children who received RT doses under 15 Gy at a mean age of 83 months. CONCLUSIONS: Reduction in stature following RT to the pediatric spine is dose- and age-dependent, persists into adulthood, and is not exacerbated by doxorubicin or cyclophosphamide. Average height deficits observed at maturity for children receiving doses currently recommended by the NWTSG are clinically nonsignificant. 9 UI - 21325336 AU - Papezova M; Mares J; Goetz P TI - [Molecular genetics of Wilms' tumor] SO - Cas Lek Cesk 2001 Jun 7;140(11):323-7 AD - Ustav biologie a lekarske genetiky 2. LF UK, Praha. AB - Molecular genetics of the Wilms' tumor plays an important role in the elucidation of the genetic etiology of the tumor disease generally. Contrary to the genesis of retinoblastoma, where a single gene is inactivated by two hits, the biological signalling pathways determining the origin of the Wilms' tumor are more complex and several genes in several loci may participate. Formation of the Wilms' tumor is accompanied with the most frequent genetic alteration, which is the loss of heterozygosity on the short arm of chromosome 11. It indicates inactivation of one or several tumor suppressor genes located at 11p region. The most studied gene of the Wilms' tumor is WT1 gene, which has been cloned and sequenced. Biological function of WT1 protein is complex one and it requires probably an interaction with other proteins, DNA and also RNA. The development of the tumor determines not only the genetic changes, but also epigenetic changes, e.g., hypermethylation of promoter and genome imprinting. 10 UI - 20342050 AU - Ishida Y; Kato K; Kigasawa H; Ohama Y; Ijiri R; Tanaka Y TI - Synchronous occurrence of pleuropulmonary blastoma and cystic nephroma: possible genetic link in cystic lesions of the lung and the kidney. SO - Med Pediatr Oncol 2000 Jul;35(1):85-7 AD - Division of Hematology, Kanagawa Children's Medical Center, Yokohama, Japan. 11 UI - 21327969 AU - Costelloe CM; Neitzschman H TI - Radiology case of the month. Asymptomatic abdominal mass in a child. Wilms' tumor (Nephroblastoma). SO - J La State Med Soc 2001 May;153(5):229 AD - Tulane University School of Medicine, New Orleans, Louisiana, USA. 12 UI - 21359809 AU - Ishikawa K; Toyoda Y; Fukuzato Y; Kato K; Ijiri R; Tanaka Y TI - Maturation in the primary and metastatic lesions of fetal rhabdomyomatous nephroblastoma. SO - Med Pediatr Oncol 2001 Jul;37(1):62-3 AD - Division of Oncology, Kanagawa Children's Medical Center, Yokohama, Japan.
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