ESPE Abstracts (2022) 95 P1-178

ESPE2022 Poster Category 1 Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology (56 abstracts)

Human chorionic gonadotropin (hCG)–secreting hepatoblastoma in an male infant with peripheral precocious puberty and 11-year follow-up

Hua-mei MA , Yan-hong LI , Qiu-li Chen & Min-lian DU

Department of Pediatrics,The First Affiliated Hospital of Sun Yat-sen University, GUANGZHOU, China

Background: Hepatoblastoma is the most common malignant pediatric hepatic tumor virtually confined to infants and young children. Accompanied by raised levels of alpha fetoprotein (AFP),rarely, the beta human chorionic gonadotropin (β hCG) levels may also be elevated, which can cause male peripheral precocious puberty (PPP).

Objective: To report a case of HB presented with PPP with elevated serum AFP, β hCG, and testosterone levels, and suppression of follicle stimulating and luteinizing hormone(FSH,LH) levels.

Methods: We described the clinical data of the infant and reviewed the relative literatures.

Results: An asymptomatic 23-month old male infant presented with a history of rapid linear growth after birth and enlarged penile size and pubic hair and deepening of the voice for two months. On examination, he was tall with height 98.5 cm (+3SD). Abdominal examination revealed slightly protuberant abdomen, hepatomegaly (Palpable 5 cm below the costal margin). Hair sparsely over the pubes, enlarged testes (volume 6 ml/6 ml), and enlarged penile size (7.2*2.7 cm) were noted on physical examination. Laboratory investigation revealed normal hemoglobin, white blood cell count and platelet count, normal transaminase levels, increased testosterone (>15 ng/ml), AFP 1892.7μg/L,β-HCG 240.57 mIU/ml levels, DHEA 2.13μmol/L,△4-A 20.3nmol/L,and 17(OH)P 6.38ng/ml level. Serum FSH(<0.05IU/L) and LH(0.2IU/L) levels were suppressed. Bone age is 4 years. Ultrasonographic examination showed a heterogenic liver tissue. The diagnosis of hepatoblastoma was made by liver biopsy. Chemotherapy was administered for seven cycles accompanied with serum AFP and β-HCG level reduction, and the patient was referred to surgery followed by 5 more cycles of Chemotherapy. Serum β-HCG and AFP level normalized 10 days and 3 months respectively after surgery. Meanwhile, linear growth slowed down, penile size and testicular volume reduced, and pubic hair disappeared 6 months later. The patient underwent five more operations for a biliary fistula at 1, 2, 4.5, 15 and 18 months, respectively, after surgery. The boy is still alive with a 11-year follow-up and no tumoral recurrence.

Conclusion: We report a male infant case of peripheral precocious puberty attributed to the elevated hCG secreted by hepatoblastoma and good outcome after chemotherapy and surgery.

Volume 95

60th Annual ESPE (ESPE 2022)

Rome, Italy
15 Sep 2022 - 17 Sep 2022

European Society for Paediatric Endocrinology 

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