Cases reported "Wilms Tumor"

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1/32. 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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2/32. adult extrarenal wilms tumor occurring in the uterus.

    Five previous cases of extrarenal wilms tumor (EWT) occurring in the uterus have been reported. The oldest patient was 22 years. We report a case of uterine EWT occurring in a 42-year-old woman. Histologically, there was typical triphasic differentiation, including epithelial, blastemal, and mesenchymal elements. The important differential diagnosis in this age group, the malignant mixed mullerian tumor, is excluded by the absence of glomeruloid structures and primitive tubules. The exact histogenesis of EWT is unknown but most likely relates to the presence of nephrogenic rests occurring in the female genital tract.
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3/32. Metanephric adenoma.

    In a recent survey of more than one hundred childhood renal tumors in our Laboratory files, we identified a unique case characterized by an unusual degree of differentiation and cell maturity. Histologically this case was notable for an orderly array of small and uniformly-packed tubules with a rosette-like configuration. The nuclei were oval, smooth and of a bland appearance. Mitoses were absent. Many glomerular figures were intermingled. This renal tumor picture is somewhat different from that known as tubular Wilms' tumor because of the well-differentiated adenomatous pattern and the absence of any blastema. The term metanephric adenoma is suggested for this tumor, which may represent the benign counterpart of Wilms' tumor.
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4/32. An immunophenotypic comparison of metanephric metaplasia of Bowman capsular epithelium with metanephric adenoma, wilms tumor, and renal development: a case report and review of the literature.

    Metanephric metaplasia of the parietal epithelium of the bowman capsule is a rare pathologic finding of unknown pathogenesis that has occurred in patients with widespread malignant neoplasms of various types. We report this finding in a 25-year-old woman with partial expression of the Carney triad who died of a disseminated gastrointestinal stromal tumor, specifically a gastric stromal sarcoma. The metaplasia involved both kidneys diffusely. It originated in the parietal epithelium of the bowman capsule, extended into the proximal tubules, and focally surrounded the glomeruli in a semicircular manner Immunohistochemical analysis revealed that the cells of metanephric metaplasia expressed the wilms tumor gene product, bcl-2 protein, and CD57 and cytokeratin 7 and keratin AE1/AE3 focally, but not CD56. This immunophenotype parallels that of metanephric adenoma, wilms tumor, and nephrogenic rests and overlaps with antigen expression in certain periods of renal development.
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5/32. A case of adult mesoblastic nephroma: ultrastructure and discussion of histogenesis.

    A benign fibromyomatous kidney tumor with deep seated tubular structures in a 20-year-old woman is reported as a case of adult mesoblastic nephroma. Ultrastructural examination confirmed the fibromyomatous nature of the stroma and the tubules appeared to be an integral part of the tumor. The histogenetic relationship to congenital mesoblastic nephroma, wilms tumor and other tumors is discussed. Radical nephrectomy was performed and the patient remained well 1 year later.
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6/32. Congenital mesoblastic nephroma.

    Congenital mesoblastic nephromas are infiltrating tumors that can contain cystic spaces lined with epithelium similar to that of dysplastic tubules and glomeruli. Urographic contrast material may enter these cystic spaces and suggest the diagnosis.
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7/32. Histochemical evidence for tubule segmentation in a case of Wilms' tumor.

    A lectin histochemical and immunohistochemical approach was used to compare the different histologic elements of an unusual case of Wilms' tumor with normal kidneys. This case was stained with ten lectin-horseradish peroxidase conjugates; immuno-stained for Tamm-Horsfall glycoprotein, epidermal keratin, and vimentin; and compared with 19 control kidneys. The morphologic and cytochemical properties of various tumor elements in this specimen served to identify them as tumor analogs of all segments of the normal kidney tubules except distal tubules. Evidence for Wilms' tumor differentiation is provided by this case, whose epithelium histochemically resembles normal human epithelial cells.
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8/32. A cell line from Wilms' tumour with deletion in short arm of chromosome II.

    A cell line (T3/73) from a Wilms' tumour has been established from a 9 month-old boy with aniridia. The tumour was removed in 1973. On histological examination a diagnosis of Wilms' tumour was made which showed undifferentiated areas, marked tubule formation and abundant striped muscle fibres. The tumour cells, which are fusiform, grew rapidly in culture without the addition of growth factors, and have undergone over 100 passages. Approximately 95% and 5% were positive for desmin and cytokeratin, respectively. The cell doubling time was 28 hr. Cytogenetic studies revealed a karyotype of 46,XY,del(11) (p12::p14). Although the cells stained very intensely with a monoclonal antibody that detects oncogene ras p 21 antigen, Southern blot analysis using c-Ha-ras as a probe failed to reveal an obvious deletion or amplification of either Ha-ras allele.
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9/32. Bilateral diffuse nephroblastomatosis, pancortical type. A case report with immunohistochemical investigations.

    A variation of Perlman's syndrome of the pancortical type is reported in a male neonate whose parents were cousins. The patient was the product of a 35-week pregnancy, the apgar score was 3, and the patient died of respiratory failure one hour and 12 minutes after delivery. autopsy revealed bilateral diffuse nephroblastomatosis, pancortical type, associated with malformations (usually facial), congenital anomalies of the heart, hepatosplenomegaly, pancreatic islet cell hyperplasia, bilateral cryptorchidism, and hyperflexibility of the left knee joint. Renal immunohistochemical investigations revealed positive bindings with peanut and soybean agglutinins and epithelial membrane antigen along the luminal surface of the epithelium in the moderately differentiated tubules, but not in blastoma or primitive epithelium.
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10/32. Extrarenal adult Wilms' tumor.

    Extrarenal Wilms' tumor is a very rare disease, occurring mostly in children. Sixteen cases have been reported in the world medical literature. A case of retroperitoneal extrarenal Wilms' tumor is reported in a 36-year old white male and the literature reviewed. Pathologic examination revealed both undifferentiated renal blastoma plus differentiated areas containing immature tubules and glomeruli. No evidence of extrarenal teratomatous tissue was seen. Theories for the histogenesis of this rare tumor are discussed.
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