hCG-secreting pineal teratoma causing precocious puberty: report of two patients and review of the literature.
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.
Nogueira
Katia
K
Department of Endocrinology, Hospital Brigadeiro, São Paulo SP, Brazil.
Liberman
Bernardo
B
Pimentel-Filho
Fernando Rodrigues
FR
Goldman
Jaime
J
Silva
Maria Elizabeth Rossi
ME
Vieira
Joaquim Oliveira
JO
Buratini
Jose Augusto
JA
Cukiert
Arthur
A
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
D006063:Chorionic Gonadotropin