抄録
A rare case of true precocious puberty due to hypothalamic hamartoma which was cured endocrinologically by surgical removal is presented.
The patient was a 20-month-old female infant. At the age of 5 months left salpingo-oophorectomy had been performed because of genital bleeding, pubic hair, enlargement of the breasts, and abdominal tumor, but no tumor cells were found histologically. Postoperatively, no regression of sexual characteristics was recognized. She weighed 16 kg and measured 89 cm in length. Neurological examination was normal, and the bone age had advanced to 37-41 months. Enhanced computerized tomography (CT) scans and metrizamide CT cisternograms demonstrated a small mass protruding from the tuber cinereum into the interpeduncular cistern. Diurnal rhythms of serum luteinizing hormone and follicle-stimulating hormone showed nocturnal secretion. Luteinizing hormone-releasing hormone (LH-RH) test and estrogen test demonstrated ‘pubertal response’. Right fronto-temporal craniotomy was performed and the tumor was subtotally removed. The surgical specimen revealed histologically normal brain tissue, and hamartoma was diagnosed. The postoperative course was uneventful and the genital bleeding disappeared. Three months after surgery, the diurnal rhythm of the gonadotropins was normalized, as were responses on the LH-RH and estrogen tests.
