title: 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.
authors: Department of Pediatric Oncology, Gazi University Medical Faculty, Ankara, Turkey. arzu-okur@hotmail.com
authors: Arzu
authors: A
authors: Okur
authors: Faruk Guclu
authors: FG
authors: Pinarli
authors: Ceyda
authors: C
authors: Karadeniz
authors: Aylar
authors: A
authors: Poyraz
authors: Kibriya
authors: K
authors: Fidan
authors: Can
authors: C
authors: Basaklar
authors: Aynur
authors: A
authors: Oguz
mesh_terms: D014408:Biomarkers, Tumor / Q000097:blood*; D003645:Death, Sudden; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000097:blood / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D008297:Male; D008487:Medical History Taking; D042965:Methylenetetrahydrofolate Reductase (NADPH2) / Q000235:genetics*; D009154:Mutation; D018365:Neoplasm, Residual / Q000188:drug therapy / Q000532:radiotherapy; D009378:Neoplasms, Multiple Primary / Q000097:blood / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D009392:Nephrectomy; D010530:Peritoneal Dialysis / Q000009:adverse effects; D012074:Remission Induction; D014883:Water-Electrolyte Imbalance / Q000209:etiology; D009396:Wilms Tumor / Q000097:blood / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D000509:alpha-Fetoproteins / Q000378:metabolism*
chemical_list: D014408:Biomarkers, Tumor; D000509:alpha-Fetoproteins; C573423:MTHFR protein, human; D042965:Methylenetetrahydrofolate Reductase (NADPH2)