Testosterone-producing hepatoblastoma in a 3-year-old boy with precocious puberty.
The syndrome of isosexual precocious puberty (PP) associated with a primary malignant hepatic tumor is rare and previously reported in only 17 cases with poor prognosis. Twelve cases are well-documented gonadotropin-producing tumors. We here describe a new case of virilizing hepatoblastoma in a 2-year-10-month-old boy with evidence of testosterone (T) production by the tumor itself, and survival with a 3 1/2-year follow-up after an extended right hepatic lobectomy. Preoperative laboratory findings showed high levels of serum alpha-fetoprotein (AFP) and T:350,000 ng/mL and 4.92 ng/mL, respectively, which normalized after surgery. There was no circulating gonadotropin nor stimulation of the hypothalamic-pituitary axis. Testicular biopsy showed neither interstitial-cell maturation nor Leydig-cell hyperplasia. Moreover, demonstration of T secretion by tumor cells and T synthesis in presence of C14 progesterone was performed in an in vitro culture system. These data seem to provide supportive evidence of a T-producing hepatoblastoma.
D006528:Carcinoma, Hepatocellular / Q000150:complications / Q000378:metabolism* / Q000601:surgery; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications / Q000378:metabolism* / Q000601:surgery; D008297:Male; D011379:Prognosis; D011629:Puberty, Precocious / Q000209:etiology; D013739:Testosterone / Q000378:metabolism*
D013739:Testosterone