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A Case of Hepatoblastoma with Precocious Puberty

Journal of the Korean Pediatric Society 2000;43(2):288-293.
Published online February 15, 2000.
A Case of Hepatoblastoma with Precocious Puberty
Chang Won Choi, Hyun Jin Park, Ki Woong Sung, Eun Sun Yoo, Hee Young Shin, Sei Won Yang, Hyo Seop Ahn
Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea
성조숙증을 동반한 간모세포종 1례
최창원, 박현진, 성기웅, 유은선, 신희영, 양세원, 안효섭
서울대학교 의과대학 소아과학교실
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.
Key Words: Hepatoblastoma, Precocious puberty, Virilization

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