PMID- 2543225 OWN - NLM STAT- MEDLINE DCOM- 19890706 LR - 20220309 IS - 0147-5185 (Print) IS - 0147-5185 (Linking) VI - 13 IP - 6 DP - 1989 Jun TI - Rhabdoid tumor of kidney. A report of 111 cases from the National Wilms' Tumor Study Pathology Center. PG - 439-58 AB - We review 111 cases of rhabdoid tumor of kidney (RTK), including 79 entered on the National Wilms' Tumor Study (NWTS). Median age at diagnosis was 11 months, with a range from 0 to 106 months. The male:female ratio was 1.5:1. Gross features included a characteristic involvement of perihilar renal parenchyma. A wide histological spectrum was encountered, including nine major morphological patterns (classical, epithelioid, sclerosing, lymphomatoid, histiocytoid, etc.). These appearances invite confusion with other renal neoplasms. Ultrastructural studies were performed in 20 cases; immunocytochemical studies were performed in 11. Vimentin was demonstrated in all tumors; epithelial membrane antigen was seen in 7. Nonspecific decoration of cytoplasmic inclusions by a variety of immunostains was found in several cases. Several findings suggested that RTK might arise from primitive cells involved in formation of the renal medulla. There was no evidence of a histogenetic relationship to Wilms' tumor, although RTK may overlap with mesoblastic nephroma and clear cell sarcoma. Of the 70 NWTS patients with adequate follow-up, 56 (80%) have died. Every patient presenting with distant metastases died, whereas 10 of 20 with negative nodes survived. Survival rates were higher for girls (56.3% versus 11.1%). None of the histological variables had independent prognostic significance. FAU - Weeks, D A AU - Weeks DA AD - Department of Pathology, Children's Hospital, Denver, CO 80218. FAU - Beckwith, J B AU - Beckwith JB FAU - Mierau, G W AU - Mierau GW FAU - Luckey, D W AU - Luckey DW LA - eng GR - CA-42326/CA/NCI NIH HHS/United States PT - Journal Article PT - Research Support, U.S. Gov't, P.H.S. PT - Review PL - United States TA - Am J Surg Pathol JT - The American journal of surgical pathology JID - 7707904 RN - 0 (Antigens, Neoplasm) RN - 0 (Antigens, Surface) RN - 0 (Vimentin) SB - IM MH - Antigens, Neoplasm/analysis MH - Antigens, Surface/analysis MH - Child MH - Child, Preschool MH - Female MH - Humans MH - Immunohistochemistry MH - Infant MH - Kidney Neoplasms/*pathology/ultrastructure MH - Male MH - Microscopy, Electron MH - Sarcoma/*pathology/ultrastructure MH - Vimentin/analysis MH - Wilms Tumor/*pathology/ultrastructure RF - 74 EDAT- 1989/06/01 00:00 MHDA- 1989/06/01 00:01 CRDT- 1989/06/01 00:00 PHST- 1989/06/01 00:00 [pubmed] PHST- 1989/06/01 00:01 [medline] PHST- 1989/06/01 00:00 [entrez] PST - ppublish SO - Am J Surg Pathol. 1989 Jun;13(6):439-58.