Cases reported "Wilms Tumor"

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1/6. Cystic partially differentiated nephroblastoma in an adult: an immunohistochemical, lectin histochemical and ultrastructural study.

    AIMS: Cystic partially differentiated nephroblastoma (CPDN) is an uncommon renal multicystic tumour, usually affecting early infants. To our knowledge, this report describes the first case of CPDN occurring in an adult. methods AND RESULTS: A 45-year-old man was found incidentally to have a left renal cystic tumour, measuring 20 mm in diameter, at the lower pole far from the pelvis. The tumour was composed of multilocular cystic spaces of variable size and intervening septa without solid nodular areas. The cysts were lined by a single layer of flattened, hobnail, or columnar epithelium. The septa were made of mesenchymal cells, which were admixed with small numbers of loosely aggregated blastemal cells, occasional tubular structures in various stages of development, and a few glomeruloid structures. The tumour cells had no anaplasia, and mitoses were rare. Immunohistochemical and lectin histochemical studies revealed that the cyst lining epithelium and the tubular structures in the septa expressed predominantly the markers for distal tubules and collecting ducts. Ultrastructurally, the cyst lining cells closely resembled collecting duct cells while some tubular structures showed an immature nephrogenic morphology. The patient was alive and well without evidence of recurrence 11 months after surgery. CONCLUSIONS: CPDN does occur in adults, as experienced in Wilms' tumour, though its incidence is extremely low. This study suggests that CPDN may show maturation intermediate between cystic nephroma and Wilms' tumour, even in adult cases.
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ranking = 1
keywords = anaplasia
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2/6. bloom syndrome in sibs: first reports of hepatocellular carcinoma and wilms tumor with documented anaplasia and nephrogenic rests.

    The triad of small body size, immunodeficiency, and sun-sensitive facial erythema characterizes the phenotype bloom syndrome (BS), a rare autosomal recessive disorder with a striking predisposition to multiple types of cancers that arise earlier than expected in the general population. Here we report two sibs with BS. The older, a 15-year-old-girl, developed a hepatocellular carcinoma, a neoplasm not yet reported in association with BS. Her younger brother developed an anaplastic wilms tumor (WT) associated with nephrogenic rests at the age of 31/2 years, and this was followed by a myelodysplastic syndrome. Complex cytogenetic abnormalities were identified in all three neoplasms. These examples expand the spectrum of malignancies occurring in BS to include liver cell neoplasms, and confirm the association of nephrogenic rests with WT, even in the setting of BS.
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ranking = 4
keywords = anaplasia
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3/6. Genetic changes of two Wilms tumors with anaplasia and a review of the literature suggesting a marker profile for therapy resistance.

    Cytogenetic data on Wilms tumors (WT) with anaplasia frequently associated with an unfavorable outcome are scarce. We present cytogenetic changes of two WT with anaplasia (primary tumor material) from nonresponders with a synopsis of the literature. The WT were investigated by cytogenetic analysis, comparative genomic hybridization, fluorescence in situ hybridization, immunofluorescence, and flow cytometric analyses. Both tumors exhibited characteristic genetic changes. One tumor was hypodiploid due to loss of entire chromosome 11; losses of 16p, 16q, 17p, chromosome 19 material, and loss of 22q12-qter. The other tumor was hyperdiploid and triploid, and displayed gain of 1q12-q23 and chromosome 9 material. Moreover, two morphological and genetically distinct cell lines have been established from both tumors, demonstrating underrepresentation of chromosomes 13, 14, 16, and 19. karyotype descriptions of 120 WT with known clinical data together with data of this report confirm: (1) inter- and intratumor heterogeneity exists; (2) loss or underrepresentation of chromosome material at 11, 13, 14, 16, 17p, 19, and 22q in various combinations presents a new marker profile of resistance to cytotoxic agents regardless of the histological types; and (3) the prognostic impact of gain at 1q12-q23 sequences warrants further validation.
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ranking = 6
keywords = anaplasia
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4/6. adult Wilms' tumor--a case report.

    A case of Wilms' tumor in a 67 year-old female is presented. The tumor totally replaced the left kidney with extension to Gerota's fascia, the adrenal gland and ureter, with tumor thrombi in the left renal vein. Multiple bony metastases to the skull, shoulder, rib, ilium, acetabulum and femur were noted also. Microscopically, the tumor was composed predominantly of blastemal tissue with diffuse anaplasia with areas of tubular differentiation and chondroid elements. Clear cell nests were found in the proximal ureter with gradual maturation downward.
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ranking = 1
keywords = anaplasia
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5/6. The association of Wilms' tumor, male pseudohermaphroditism and diffuse glomerular disease (Drash syndrome): report of eight cases with clinical and morphologic findings and review of the literature.

    In 8 children (6 males) at least 2 of 3 disorders were found--male pseudohermaphroditism (MPH), Wilm's tumor, and glomerular disease. MPH was present in the 6 males; they had abdominal cryptorchidism (6/6), ambiguous genitalia (6/6), negative sex chromatin (5/5), and 46XY karyotype (2/2). The gonads examined microscopically in 3 patients were dysgenetic testes. The renal tumor present in 7 was nephroblastoma (NB) of the classical type without anaplasia or nephroblastomatosis, bilateral in 1, and unilateral but multinodular in 2. Five underwent nephrectomy for a renal mass, and in 2 NB was found at open biopsy or at autopsy. The mean age at diagnosis was 10 months. Glomerular disease in 6 patients began with onset of the nephrotic syndrome between 20 days and 39 months of age; it was resistant to steroid therapy and led to death from renal failure. Microscopically the glomerular process was a diffuse mesangial sclerosis (DMS). The 2 children with NB and MPH, but without DMS are healthy 2 1/2 and 9 years postnephrectomy. Neither familial incidence nor parental consanguinity was found. This syndrome has complete and partial forms, and its early recognition is important both for patient management and for assessment of prognosis.
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ranking = 1
keywords = anaplasia
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6/6. Bone metastases in Wilms' tumor--report of three cases and review of literature.

    Bone metastases are extremely rare in patients with classical Wilms' tumor (WT). We describe the clinical and radiologic features, treatment and outcome of three patients with WT (one with favorable histology and two with anaplasia) in whom bone metastases were detected at diagnosis or relapse. Bone metastases were documented by skeletal radiographs, computed tomography and/or bone scintigraphy. The patient with favorable histology WT had no evidence of pulmonary metastases and is now free of disease following aggressive chemotherapy and radiotherapy.
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ranking = 1
keywords = anaplasia
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