내분비 기능장애로 발현된 시상하부 과오종의 성공적인 수술적 절제- 증례보고 -

Abstract

Hypothalamic hamartomas are rare developmental malformations of the ventral hypothalamus and tuber cinereum mani-festing by paroxysmal disorders such as gelastic seizures and endocrine disorders, mostly precocious sexual develop-ment. Surgical treatment of hypothalamic hamartoma is associated with a high risk of complications because of the close vicinity of adjacent structures such as the optic tracts and mammillary bodies. The patient was a 20-year-old woman who presented with intermittent headache, and galactorrhea with precocious pu-berty. Although waking electroencephalography (EEG) and laboratory findings were normal, serum prolactin level was increased. Brain magnetic resonance (MR) imaging showed a non-enhancing 2.0×2.2×2.5 cm sized mass in the supra- and retrosellar portion, which was isointense on the T1-weighted image (WI) and slightly hyperintense on the T2-WI. It seemed that the mass was probably originating from mammillary body. She undertook gross total tumor removal via half and half approach, and pathological examination revealed the findings of hamartoma. Postoperative MR imaging demon-strated that the hypothalamic hamartoma was successfully resected, and neurological status was also uneventful. We report a successful surgical case with hypothalamic hamartoma and discuss pertinent literatures.