A Case of Hepatoblastoma with Precocious Puberty

Choi, Chang Won; Park, Hyun Jin; Sung, Ki Woong; Yoo, Eun Sun; Shin, Hee Young; Yang, Sei Won; Ahn, Hyo Seop

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 alpha-fetoprotein(AFP) was above 70,000ng/mL, and serum beta-human chorionic gonadotropin(beta-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 beta-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.