A Case of Hepatoblastoma with Precocious Puberty.
- Author:
Chang Won CHOI
1
;
Hyun Jin PARK
;
Ki Woong SUNG
;
Eun Sun YOO
;
Hee Young SHIN
;
Sei Won YANG
;
Hyo Seop AHN
Author Information
1. Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Hepatoblastoma;
Precocious puberty;
Virilization
- MeSH:
Biopsy, Needle;
Chorion;
Chorionic Gonadotropin;
Diagnosis;
Drug Therapy;
Gonadotropins;
Hair;
Hepatoblastoma*;
Humans;
Liver;
Male;
Penis;
Puberty, Precocious*;
Tomography, X-Ray Computed;
Virilism;
Voice
- From:Journal of the Korean Pediatric Society
2000;43(2):288-293
- CountryRepublic of Korea
- Language:Korean
-
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.
