1/17. 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.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
2/17. Spectrum of cystic variants of Wilm's tumour: cystic nephroma (multilocular cyst) and cystic partially differentiated nephroma--a report of four cases.Two cases of cystic nephroma (multilocular cyst of the kidney), and one case each of cystic partially differentiated nephroblastoma (CPDN) and rhabdomyomatous Wilms' tumour are described. All were male and in the pediatric age group. Grossly tumours were unilateral, unicentric and multiloculated. The need for proper designation of these lesions is highlighted because of difference in the treatment and prognosis of these tumours.- - - - - - - - - - ranking = 0.2keywords = nephroblastoma (Clic here for more details about this article) |
3/17. Juxtaposed cystic nephroma and Wilms' tumor.We report a case of juxtaposed Wilms' tumor (WT) and cystic nephroma (CN) in a 21-month-old girl which gave rise to radiological diagnostic difficulty. Preoperative chemotherapy was given, resulting in marked tumor necrosis but the cystic nephroma remained untouched. Histological examination showed characteristic features of a triphasic WT and a CN; the two lesions were separated by a thick fibrous capsule. While everybody agrees that WT and cystic partially differentiated nephroblastoma (CPDN) are closely related, there are two opposite views about their relationship to CN. One is that CN may represent the final step in maturation of WT and CPDN. Other authors argue that there is no evidence to support this theory but believe CN might have something in common with nephrogenic rests. We suggest that the two lesions in the present case may have originated from two intralobar nephrogenic rests, which would strengthen the latter view.- - - - - - - - - - ranking = 0.2keywords = nephroblastoma (Clic here for more details about this article) |
4/17. Papillonodular type of cystic partially differentiated nephroblastoma: a case report.We report a case of the papillonodular type of cystic partially differentiated nephroblastoma (CPDN), an extremely rare renal neoplasm that occurs in newborns and infants. The papillonodular type of CPDN is a variant of the conventional form of CPDN. MRI clearly demonstrated the gross pathologic features, distinguishing it from other renal multilocular cystic tumors.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
5/17. Massive infantile nephroblastomatosis: a clinical, radiological, and pathological analysis of four cases.Four cases of massive infantile nephroblastomatosis with up to three and one-half years follow-up are described. Great similarity was found clinically, radiologically and pathologically, which distinguishes this entity from true Wilms' tumor. The course of the renal lesion following treatment was monitored by sequential radiological and biopsy studies. The pathogenesis, natural history, and management are discussed.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
6/17. Cystic partially differentiated nephroblastoma: a clinicopathologic entity in the spectrum of infantile renal neoplasia.Three cases of cystic partially differentiated nephroblastoma (CPDN) are presented and ten cases from literature are reviewed. CPDN has been designated by various terms; it is a cystic encapsulated tumor occurring before 2 years of age. cysts are lined by epithelium; septa of the cysts show a mixture of partially differentiated and undifferentiated metanephrogenic blastema. This histologic feature distinguishes CPDN from multilocular cyst of kidney. In seven cases simple nephrectomy, and in remaining cases nephrectomy with radiation and/or chemotherapy, had been the treatment. The disease-free interval ranged from 5 to 72 months, without reports of recurrence or metastasis. CPDN appears to take a benign course and simple nephrectomy seems to be the treatemtn of choice. However, in view of the possibility of recurrence as shown in rare instances by congenital mesoblastic nephroma, another less aggressive lesion in the spectrum of infantile renal neoplasia, regular follow up is recommended.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
7/17. Cystic partially differentiated nephroblastoma and multilocular cyst of the kidney. Report of two cases of so-called multilocular cyst of the kidney.Two cases of so-called multilocular cyst of the kidney are presented. Although both cases satisfied all of the criteria which characterize the multilocular cyst of the kidney, one had cystic lesions and neoplastic lesions (nephroblastoma-like lesions) and the other had only cystic lesions and was complicated with hamartoma. We prefer the term "cystic partially differentiated nephroblastoma" as the diagnostic term for the former and "multilocular cyst of the kidney" for the latter. A study of 40 reported cases of multilocular cystic lesions of the kidney revealed that cases having only cystic lesions were distributed in all ages from 4.5 months to 71 years and that cases having neoplastic lesions were seen in infants from 4 months to 2 years.- - - - - - - - - - ranking = 1.2keywords = nephroblastoma (Clic here for more details about this article) |
8/17. Cystic nephroma: an ultrastructural and biochemical study.A case of a multilocular renal cyst (cystic nephroma) in an 18-month-girl is presented. The light and electron microscopy studies failed to demonstrate nephroblastomatous elements. Analysis of cystic fluid constituents showed levels resembling serum. Measurable levels of prostaglandins e and F also were found. The possible relationship of a cystic nephroma to a nephroblastoma is discussed.- - - - - - - - - - ranking = 0.4keywords = nephroblastoma (Clic here for more details about this article) |
9/17. Multilocular renal cyst. Scanning and transmission electron microscopic observations.A multilocular renal cyst in a boy aged one year and four months is presented, with particular attention being paid to the nature of the epithelial lining cells. light and transmission electron microscopy showed the cyst to be lined by a single layer of flattened or cuboidal epithelial cells of relatively uniform morphology. Neither embryonic elements nor nephroblastomatous foci were noted in the intervening stroma. The scanning electron microscopy showed hitherto undescribed surface morphological features of the epithelial lining cells: They were characterized by the presence of one or, occasionally two centrally positioned long cilia and by variably oriented microvilli. The observations presented here suggested that the lining cells of the cyst most closely resembled the principal cells of the collecting ducts, especially those located in the inner medulla of the kidney. An unexpected finding was the additional occurrence of a giant bullous lesion in the right lung of this patient.- - - - - - - - - - ranking = 0.2keywords = nephroblastoma (Clic here for more details about this article) |
10/17. Bilateral renal dysplasia with features of nephroblastomatosis.We report a case of bilateral renal dysplasia with "cystic nephroblastomatosis" in a 29-week-old white female infant. The patient presented with bulging flanks, anuria, and respiratory distress. She survived for 1 1/2 days. Both kidneys were markedly enlarged by a diffuse cystic malformation. Microscopically, there was no demarcation between cortex and medulla. Numerous cysts and partially differentiated metanephric blastema cells were seen throughout the cortex and medulla. In addition, dysplastic tissues including cartilage occurred in the medulla. No evidence of Wilm's tumor was found. To our knowledge, this unique form of renal pathology has not been previously reported.- - - - - - - - - - ranking = 1keywords = nephroblastoma (Clic here for more details about this article) |
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