성조숙증을 동반한 간모세포종 1례 = A Case of Hepatoblastoma with Precocious Puberty

다국어 초록 (Multilingual 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 ■- fetoprotein(AFP) was above 70,000ng/mL, and serum ■-human chorionic gonadotropin(■-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 ■-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.

국문 초록 (Abstract)

저자들은 성조숙증과 복부 종괴를 주소로 내원한 2년 9개월 남아에서 경피 세침생검을 시행하여 간모 세포종을 진단하였고, 혈청 β-HCG가 360mIU/mL, testosterone이 12.7ng/mL로 증가되어 있음을 확인하였다. 저자들은 4주기의 항암화학요법을 시행한 후에 종양을 완전히 절제할 수 있었고, 2주기의 항암화학요법을 더 시행한 후에 치료를 종결하였다. 치료 종결 후 외래에서 확인한 β-HCG는 3mIU/mL 이하, testosterone은 0.1ng/mL 이하로 감소하였고, 환아의 턱수염과 음모는 소실되었지만 목소리와 음경의 크기는 큰 변화를 보이지 않았다. 저자들은 환아를 치료 종결 2년 후까지 추적 관찰 중이며 종양 재발의 증거는 없었다. 저자들은 본 증례를 문헌 고찰과 함께 보고하는 바이다.