Abstract
IntroductionThe syndrome of isosexual precocious puberty associated with primary malignant hepatic tumors is rare. All previously reported cases in the literature are old and prognosis was grim.Case presentationWe present the case of a 15-month-old Asian male baby who presented with precocious puberty associated with hepatoblastoma. Serum concentrations of alpha-fetoprotein and free testosterone were elevated, as was beta human chorionic gonadotropin hormone. He was treated with six courses of chemotherapy and underwent surgery. His surface markers as well as free testosterone level returned to normal during therapy. The child has now been off therapy for 18 months with no evidence of tumor recurrence at follow-up.ConclusionVirilizing hepatoblastoma is rare and reported with poor outcome, but the development of new chemotherapeutic agents and complete surgical resection are promising.
Highlights
The syndrome of isosexual precocious puberty associated with primary malignant hepatic tumors is rare
Hepatoblastoma is associated with beta human chorionic gonadotropin hormone (b-hCG) secretion [1]
Isosexual precocious puberty was reported in boys with hepatoblastoma [1,3,4,5,6,7,8,9,10,11,12,13,14,15]
Summary
Hepatoblastoma is the most common pediatric hepatic tumor. Using the current modalities of treatment, nonmetastatic hepatoblastoma usually carries a favorable prognosis if completely resected. Case presentation A 15-month-old Asian male baby presented to our center with precocious puberty and a hepatic mass. He was a product of cesarean section at 37 weeks of gestation. A workup revealed a bone age of two years and eight months and blood investigations showed elevated AFP (356,474 ng/mL, normal for age < 12 ng/mL), b-hCG (13.7 mIU/mL, normal for age < 2 mIU/mL), and free testosterone (10 pmol/L, normal for age 0.09-5.4 pmol/ L) along with thrombocytosis (platelet count of 1244 × 103/μl). Our patient was 33 months old on the last visit and no further development of secondary sexual signs were elicited His height was 96 cm (between the 50th and 75th centile) and his weight was 14.2 kg (at 50th centile)
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