All issues > Volume 43(2); 2000

Case Report

J Korean Pediatr Soc. 2000;43(2):288-293. Published online February 15, 2000.

A Case of Hepatoblastoma with Precocious Puberty

Chang Won CW Choi¹, Hyun Jin HJ Park¹, Ki Woong KW Sung¹, Eun Sun ES Yoo¹, Hee Young HY Shin¹, Sei Won SW Yang¹, Hyo Seop HS Ahn¹

¹Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea

Abstract

Hepatoblastoma is the most common primary malignant liver tumor in childhood. Most cases are boys generally below three years of age. Hepatoblastoma are associated with various non-metastatic syndromes. Rarely, human chorionic gonadotropin may be produced by the tumor. Ectopic gonadotropin production from this source is a rare cause of precocious puberty in boys. Since a report by Behrendt in 1931, about 25 cases of hepatoblastoma with precocious puberty have been reported in the literature. We describe here a new case of hepatoblastoma with precocious puberty in a 2-year-9-month-old boy. He presented with precocious puberty and abdominal mass. He had an enlarged penis, pubic hair and deep voice. Laboratory examination revealed that serum α- fetoprotein(AFP) was above 70,000ng/mL, and serum β-human chorionic gonadotropin(β-HCG) 360mIU/mL. Abdominal CT revealed a huge tumor occupying the entire right lobe of the liver. A diagnosis of hepatoblastoma was made by percutaneous needle biopsy. After completing four cycles of chemotherapy, a complete tumor resection was undertaker. Postoperative level of the serum AFP was below 5ng/mL, and serum β-HCG below 3mIU/mL. He received two additional cycles of treatment. He is alive and in a disease-free state for two years after cessation of the treatment.

Keywords :Hepatoblastoma, Precocious puberty, Virilization