Asada H, Otani M, Furuhata S, Inoue H, Toya S, Ogawa Y
Department of Neurosurgery, School of Medicine, Keio University, Tokyo.
Neurol Med Chir (Tokyo). 1990 Aug;30(8):628-32. doi: 10.2176/nmc.30.628.
A gangliocytoma in the sellar region is very rare. We report a case of an intrasellar gangliocytoma complicated by pituitary adenoma presenting with acromegaly. A 52-year-old female was admitted to our hospital with headache, mild acromegaly, and bitemporal hemianopsia, and endocrinological study found a high serum level of growth hormone (GH). A computed tomographic scan revealed a tumor in the sellar region, which was almost totally removed by trans-sphenoidal surgery. Histological examination of the resected specimen showed diffuse, chromophobe-type pituitary adenoma, partially containing cholesterin clefts. Areas of clusters of dysmorphic neurons, adjacent to or mixed with pituitary adenoma, were diagnosed as gangliocytoma. The immunohistochemical examination showed GH-releasing hormone (GRH)-positive dysmorphic neurons and GH-positive pituitary adenoma. We consider that the trophic effect of GRH secreted by the neurons of GRH-producing intrasellar gangliocytoma probably caused the GH-producing pituitary adenoma.
鞍区神经节细胞瘤极为罕见。我们报告一例鞍内神经节细胞瘤合并垂体腺瘤并伴有肢端肥大症的病例。一名52岁女性因头痛、轻度肢端肥大症和双颞侧偏盲入院，内分泌学检查发现血清生长激素（GH）水平升高。计算机断层扫描显示鞍区有一肿瘤，经蝶窦手术几乎将其完全切除。对切除标本的组织学检查显示为弥漫性嫌色型垂体腺瘤，部分含有胆固醇裂隙。与垂体腺瘤相邻或混合存在的异形神经元簇区域被诊断为神经节细胞瘤。免疫组织化学检查显示生长激素释放激素（GRH）阳性的异形神经元和GH阳性的垂体腺瘤。我们认为，鞍内产生GRH的神经节细胞瘤神经元分泌的GRH的营养作用可能导致了产生GH的垂体腺瘤。
