ESPE Abstracts

Background: Neoplastic causes of precocious puberty include brain, gonadal, adrenal and germ cell tumors; hepatoblastoma (HB) is only rarely noted [1,2]. HB, is a rare primary hepatic tumor of childhood [3]. It is accompanied by raised levels of alpha-fetoprotein (α-FP). Rarely, beta-human chorionic gonadotropin (β-hCG) levels are elevated, resulting in peripheral precocious puberty (PPP).

Clinical Case: We present a case of rapidly progressive precocious puberty over a one-month period in an otherwise healthy 3-year-old boy. Serum concentrations of α-FP, β-hCG and testosterone were significantly elevated, while follicle-stimulating, and luteinizing hormone levels were suppressed. Imaging was obtained in view of significantly elevated tumor markers. CT of chest and abdomen confirmed a large right-sided hepatic tumor with two metastatic lung nodules. The child underwent liver biopsy confirming hepatoblastoma. He started chemotherapy, and on follow up eventually tumor size did significantly shrink and lung metastasis completely disappeared. Repeat β-hCG was within normal limits after 3 months of chemotherapy. He then underwent right lobectomy and histopathology confirmed it to be hepatoblastoma epithelial type.

Conclusion: β-hCG-secreting HB is an uncommon cause of isosexual precious puberty. β-hCG binds to LH receptors in the testes stimulating testosterone production. Precocious puberty in boys invariably has an underlying pathologic cause and requires prompt and thorough investigation. Urgent, comprehensive evaluation including baseline hormonal labs with tumor markers to determine central or peripheral cause, and comprehensive imaging are required.

References:

1. Chung E, Biko D, Schroeder J, Cube R, Conran R. From the Radiologic Pathology Archives: Precocious Puberty: Radiologic-Pathologic Correlation. RadioGraphics. 2012;32(7):2071-2099.

2. Wendt S, Shelso J, Wright K, Furman W. Neoplastic causes of abnormal puberty. Pediatric Blood & Cancer. 2013;61(4):664-671.

3. Chung E, Lattin G, Cube R, Lewis R, Marichal-Hernández C, Shawhan R et al. From the Archives of the AFIP: Pediatric Liver Masses: Radiologic-Pathologic Correlation Part 2. Malignant Tumors. RadioGraphics. 2011;31(2):483-507.