1/5. 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 = 1keywords = nephroblastoma (Clic here for more details about this article) |
2/5. Fetal rhabdomyomatous nephroblastoma: diagnosis by fine-needle aspiration cytology--a case report.The present report describes the case of a 21-mo-old boy with a fetal rhabdomyomatous nephroblastoma (FRN) diagnosed by fine-needle aspiration cytology (FNAC). The smears contained numerous isolated or clustered, deeply eosinophilic, thin, elongated rhabdomyoblasts with oval nuclei. Some of these cells presented cross-striations. Peculiar clusters of small, deeply eosinophilic, round cells with and without rounded nuclei in a mosaic pattern were interpreted as representing transversally oriented rhabdomyoblasts. Fetal rhabdomyoblasts were by far the predominant type of cell. histology of the surgical specimen proved 90% of the mass to be constituted of fetal rhadomyomatous tissue. Adequate recognition of FRN is useful for the planning of treatment.- - - - - - - - - - ranking = 5keywords = nephroblastoma (Clic here for more details about this article) |
3/5. Fetal rhabdomyomatous nephroblastoma: report of 14 cases confirming chemotherapy resistance.The authors retrospectively reviewed their experience with 14 cases of fetal rhabdomyomatous nephroblastoma (15.6% of all renal tumors seen in the 1984-1998 period), analyzing the diagnostic and treatment approach as well outcome. Mean age at presentation was 27 months; 10 (71%) patients were younger than 36 months (5 of these were less than 12 months) at diagnosis. Three patients had bilateral tumors. One case was extrarenal. Nine patients received preoperative chemotherapy after fine-needle aspiration biopsy. The response was null in five children and mild in three; in the remaining one the tumor increased in size. The only patient showing greater than 50% tumor shrinkage was the one treated with radiotherapy. Postoperative treatment according to staging followed the SIOP protocol. In the follow-up (mean 59.5 months) of 12 patients, 6 were in stages 1-2: 4 are alive and free of disease and 2 died (one due to intractable local recurrence and the other in complete clinical remission). All six patients presenting in advanced stages died (four due to disease progression and two due to complications while in complete clinical remission). patients with fetal rhabdomyomatous nephroblastoma presenting with distant metastatic disease and high stage have unfavorable outcomes, confirming data from the literature indicating that chemotherapy is not as effective as in other variants of nephroblastoma. New protocols are needed to overcome this situation, for which surgery appears the only truly effective option.- - - - - - - - - - ranking = 7keywords = nephroblastoma (Clic here for more details about this article) |
4/5. Hepatic rhabdomyomatous tumor: late sequel of a fetal rhabdomyomatous nephroblastoma.A mesenchymal tumor with the macroscopic and microscopic features of a fetal rhabdomyoma arose in the liver of a 14-year-old boy. Thirteen years previously this boy had been treated for a fetal rhabdomyomatous nephroblastoma with nephrectomy and--for subsequent peritoneal disseminations--with surgical excision, radiotherapy, and chemotherapy. The unusual hepatic location of the rhabdomyomatous tumor in this patient supports the view that this mature tumor developed from a metastasis of the original nephroblastoma. As such, this case may represent an example of irreversible change of a malignant process into a benign tumor probably caused by the action of systemic chemotherapy.- - - - - - - - - - ranking = 6keywords = nephroblastoma (Clic here for more details about this article) |
5/5. Fetal rhabdomyomatous nephroblastoma.A case of fetal rhabdomyomatous nephroblastoma is reported. The neoplasm distended the renal pelvis, resembling a sarcoma botryoides. The patient died postoperatively of pneumonia and an autopsy showed no residual neoplasm.- - - - - - - - - - ranking = 5keywords = nephroblastoma (Clic here for more details about this article) |