title: hCG-secreting pineal teratoma causing precocious puberty: report of two patients and review of the literature.
abstract: Two boys are described with precocious puberty (PP) due to pineal immature teratoma associated with choriocarcinoma. Patient 1 was a 7 year-old boy with a 2-year history of PP. He had elevated CSF and plasma beta-hCG levels. Magnetic resonance imaging (MRI) showed a 3.0 cm pineal mass. He was initially submitted to a trial with radiotherapy, followed by radical surgical resection, stereotactic radiotherapy and chemotherapy. Long-term follow up included the appearance of acute hydrocephalus requiring CSF shunting, local hemorrhage and extensive radionecrosis. Death occurred 1.5 years after diagnosis. Patient 2 was a 7 year-old boy with an 8-month history of PP. He had elevated CSF and plasma beta-hCG and alpha-fetoprotein levels. MRI showed a 1.0 cm pineal mass. He was submitted to radical surgical resection (which caused normalization of levels of markers) and prophylactic chemotherapy. The boy is doing well 1.5 years after diagnosis. An extensive review of the literature corroborates the idea that this last treatment paradigm (surgery and chemotherapy) probably represents the best treatment regimen for these patients.
authors.affiliation: Department of Endocrinology, Hospital Brigadeiro, São Paulo SP, Brazil.
authors.forename: Katia
authors.initials: K
authors.lastname: Nogueira
authors.forename: Bernardo
authors.initials: B
authors.lastname: Liberman
authors.forename: Fernando Rodrigues
authors.initials: FR
authors.lastname: Pimentel-Filho
authors.forename: Jaime
authors.initials: J
authors.lastname: Goldman
authors.forename: Maria Elizabeth Rossi
authors.initials: ME
authors.lastname: Silva
authors.forename: Joaquim Oliveira
authors.initials: JO
authors.lastname: Vieira
authors.forename: Jose Augusto
authors.initials: JA
authors.lastname: Buratini
authors.forename: Arthur
authors.initials: A
authors.lastname: Cukiert
mesh_terms: D002648:Child; D002822:Choriocarcinoma / Q000150:complications / Q000628:therapy; D006063:Chorionic Gonadotropin / Q000378:metabolism*; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D010871:Pinealoma / Q000150:complications* / Q000378:metabolism; D011629:Puberty, Precocious / Q000209:etiology*; D013724:Teratoma / Q000150:complications* / Q000378:metabolism
chemical_list: D006063:Chorionic Gonadotropin