[Precocious pseudo-puberty due to HCG-secreting androblastoma of the ovary. One case (author's transl)].
A case of ovarian androblastoma (arrhenoblastoma) of the intermediate differentiation type in a 5-year old girl is reported. The clinical features were precocious isosexual puberty with secondary masculinization. The tumour was removed but relapsed 26 months later, requiring ovariectomy and salpingectomy. The patient, who has now been followed up for 7 years and 6 months, is definitely cured. Biochemically, the androblastoma was accompanied by high plasma levels of oestrogens, androgens and, quite exceptionally, human chorionic ganodotropin, alpha HCG and beta HCG. Light and electron microscope examination failed to differentiale Sertoli cells from granulosa cells and Leyding cells from luteinized ovarian stroma cells. Areas resembling syncytiotrophoblastic cells were very limited and could not be seen under the electron microscope. Some cells looked like undifferentiated germ cells. The HCG secreted was biologically active on rabbit ovaries (Reiprich's reaction) but inactive on the girl's contralateral ovary, which was found on two biopsies to be unstimulated and prepubertal.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D006063:Chorionic Gonadotropin / Q000378:metabolism*; D004958:Estradiol / Q000378:metabolism; D005260:Female; D006801:Humans; D010051:Ovarian Neoplasms / Q000150:complications / Q000378:metabolism* / Q000473:pathology; D011629:Puberty, Precocious / Q000209:etiology*; D018310:Sertoli-Leydig Cell Tumor / Q000150:complications / Q000378:metabolism* / Q000473:pathology; D013739:Testosterone / Q000378:metabolism
D006063:Chorionic Gonadotropin; D013739:Testosterone; D004958:Estradiol
Pseudo-puberté précoce par androblastome ovarien avec sécrétion d'alpha et de bêta HCG. Une observation.