A female case of the HCG-producing ectopic pinealoma associated with precocious puberty.

Abstract

A female case of precocious puberty associated with HCG[human chorionic gonadotropin]-producing ectopic pinealoma was reported. The patient, a 5 yr old girl, was referred to the hospital because of headache and choked discs. Physical examination revealed normal physical growth with breast enlargement. Endocrinological study revealed a high plasma HCG concentration of 1192 ng/ml with a normal FSH [follicle stimulating hormone] level. None of HCG, LH [luteinizing hormone] and FSH responded to the LH-RH [luteinizing hormone-releasing hormone] test. A partial resection of the tumor and an external X-ray irradiation relieved the symptoms and breast enlargement subsided with a remarkable decrease in the plasma HCG level. Histological examination revealed 2-cell-pattern pinealoma and EM findings showed abundant secretory granules in the dark cells. HCG content in the tumor was as high as 400 ng/mg of acetone dried tumor tissue but no FSH was detectable. Previously, all of the reported cases of precocious puberty associated with pineal tumors were exclusively boys. A normal level of plasma FSH concentration with a somewhat elevated prolactin level might be a contributory factor for the development of precocial sexual development in the present case.