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

OKUR A., PINARLI F. G., KARADENİZ C., POYRAZ A., Fidan K., Basaklar C., ...More

TUMORI, vol.98, no.6, 2012 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 98 Issue: 6
  • Publication Date: 2012
  • Doi Number: 10.1700/1217.13518
  • Journal Name: TUMORI
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Gazi University Affiliated: Yes

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.