Precocious pseudopuberty, a paraneoplastic manifestation: a report of 2 cases

Abstract

Peripheral precocious puberty (PPP) may be a paraneoplastic manifestation, associated with beta human chorionic gonadotropin (β-hCG)–secreting tumors. We describe 2 young children with β-hCG-secreting tumors presenting with signs of pubertal activation. In the first patient, a 16-month-old boy with hepatoblastoma, only initial signs of PPP at presentation were identifiable, with concomitant high levels of β-hCG. Although the tumor had good response to therapy, β-hCG levels were fluctuant until the tumor was resected surgically. The second patient, an 18-month-old boy with intracranial germ cell tumor, presented with clear signs of pubertal activation and genitalia enlargement with no initial alteration of sex hormones. In both cases, the oncologic response to therapy was good. In the first case, full remission of the pubertal signs was observed; in the second, pubertal signs were still visible 20 months after the end of treatment.