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

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

TUMORI, cilt.98, sa.6, 2012 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 98 Sayı: 6
  • Basım Tarihi: 2012
  • Doi Numarası: 10.1700/1217.13518
  • Dergi Adı: TUMORI
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Gazi Üniversitesi Adresli: Evet

Özet

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.