| A Case of Hepatoblastoma with Precocious Puberty |
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Chang Won Choi, Hyun Jin Park, Ki Woong Sung, Eun Sun Yoo, Hee Young Shin, Sei Won Yang, Hyo Seop Ahn |
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Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea |
| 성조숙증을 동반한 간모세포종 1례 |
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최창원, 박현진, 성기웅, 유은선, 신희영, 양세원, 안효섭 |
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서울대학교 의과대학 소아과학교실 |
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| Abstract |
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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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