垂体节细胞瘤的临床病理特征及外科治疗

The clinical, pathological features and surgical treatment of pituitary gangliocytoma

目的 总结垂体节细胞瘤的临床病理特征及治疗方法.方法 回顾性分析8例经手术和病理证实的垂体节细胞瘤患者的临床、病理资料及术后随访结果.结果 5例全切除,3例次全切除.病理显示节细胞瘤与垂体腺瘤细胞合并存在是其典型特征,其中4例为垂体生长激素（GH）腺瘤伴节细胞瘤,2例为垂体多激素腺瘤伴节细胞瘤,1例为垂体无激素型腺瘤伴节细胞瘤,1例为垂体节细胞瘤.平均随访4.7年,所有患者症状均缓解;3例次全切除患者中2例行伽玛刀治疗,l例观察随访;鞍区MRI复查示肿瘤7例消失,1例残留未见增大.结论 绝大多数垂体节细胞瘤与垂体腺瘤合并存在.临床多表现为内分泌紊乱症状,以肢端肥大症常见.诊断主要依靠病理组织学、免疫组化标记,GH及生长激素释放激素（GHRH）阳性为其重要特征.经蝶窦手术切除是首选治疗方法,若有残留可行伽玛刀治疗,预后良好.

Objective To summarize the clinical pathological characteristics and treatment of pituitary gangliocytoma.Methods The clinical,pathological features and follow-up results of 8 patients with pituitary gangliocytoma were retrospectively analyzed.Results Total resection was achieved in 5 patients and subtotal in 3.This kind of lesion was pathologically characterized by the coexistence of pituitary gangliocytoma and pituitary adenoma.Four cases were diagnosed as pituitary growth hormone（GH） adenoma with gangliocytoma,2 pituitary plurihormonal adenoma with gangliocytoma,1 pituitary hormonenegative adenoma with gangliocytoma,and 1 pituitary gangliocytoma.The mean follow-up period was 4.7 years.Of the 3 patients who failed to achieve total resection,2 underwent gamma knife surgery,1 was intimately followe-up.The postoperative MRI examinations showed that the tumors disappeared in 7 cases,the remnant tumor of the patient who did not undergo gamma knife surgery remained unchanged.Conclusions The majority of pituitary ganliocytomas coexisted with pituitary adenomas.The endocrine disorders,especially acromegaly,were the most common clinical manifestations.The correct diagnosis of pituitary gangliocytoma should be based on the histopathological and immunohistochemical results.The positive result of GH and growth hormone releasing hormone （GHRH） staining was a key feature of this kind of tumor.Although transsphenoidal microsurgery was the choice of treatment,gamma knife surgery should be considered if the total resection could not be achieved.Pituitary gangliocytoma was a benign tumor with optimistic prognosis.