Familial synchronous bilateral teratoid Wilms tumor with elevated alpha-fetoprotein level

Abstract

Familial Wilms tumor is a rare entity that accounts for only 1–2% of all Wilms tumor cases, with an earlier age of onset and an increased frequency of bilateral tumors. Teratoid Wilms tumor is a variant of nephroblastoma with a predominance of heterologous tissues comprising more than 50% of the tumor volume. Wilms tumor does not usually secrete any specific tumor marker and all teratoid Wilms tumor cases previously reported were sporadic non-secreting neoplasms. Here we describe an infant with familial synchronous bilateral teratoid Wilms tumor whose serum alpha-fetoprotein level was elevated. To our knowledge, this extremely rare type of case is reported for the first time in the literature.

Extrarenal teratoma with nephroblastoma in the retroperitoneum

“…[ 50 ] This resistance to chemotherapy and radiotherapy may be due to the mature, well-differentiated heterologous elements that form a large part of the tumor. [ 8 , 26 ] Therefore, TWN should be recognized such that the use of potentially toxic chemotherapy can be avoided because such tumors usually do not respond to preoperative chemotherapy. [ 18 ] In the present report, no radiotherapy or chemotherapy regimen was administered to our patient.…”