Qiao Nidan, Ye Zhao, Wang Yongfei, Li Shiqi, Mao Yin, Bao Weimin, Che Xiaoming, Qin Zhiyong, Xu Wei, Shen Ming, Chen Hong, Shou Xuefei, Zhao Yao
Shanghai Pituitary Tumor Center, Department of Neurosurgery, HuaShan Hospital, Shanghai Medical College, FuDan University, 12# Middle Wulumuqi Road, Shanghai, 200040, China.
Department of Neuro-pathology, HuaShan Hospital, Shanghai Medical College, FuDan University, 12# Middle Wulumuqi Road, Shanghai, 200040, China.
Clin Neurol Neurosurg. 2014 Nov;126:156-61. doi: 10.1016/j.clineuro.2014.08.034. Epub 2014 Sep 7.
Gangliocytomas occurring in the sellar region are extremely rare. We examined a cohort of these tumors to examine their clinical presentations and prognoses. Between January 2000 and December 2012, 23 patients were diagnosed with sellar region gangliocytomas in Huashan Hospital. These patients were retrospectively reviewed for medical histories, endocrinological examinations, preoperative magnetic resonance imaging (MRI), pathological findings and follow-ups. Endocrinological tests revealed elevated prolactin (PRL) levels in 10 cases (43.5%) and elevated growth hormone (GH) and insulin-like growth factor 1 (IGF-1) levels in 9 cases (39.1%). Scattered ganglion cells admixed with adenomatous components were observed in 16 cases (69.6%). In the remaining 7 cases (30.4%), only fragments with ganglion cells dispersed in the fibrillar matrix without adenohypophyseal components were detected. Immunohistochemistry revealed PRL-positive adenomas in 6 cases (26.1%) and GH-positive adenomas in 8 cases (34.8%). The average follow-up period was 4.2 years (range: 1-12.7 years). Gross total resection was achieved in 20 cases (87.0%). One patient recurred five years after tumor resection (4.3%). One patient died of acute myocardial infarction six years after operation. Gangliocytomas located in the sellar region may represent a unique immunopathological entity. The surgical results and prognoses of the gangliocytomas were comparable with those of pituitary adenomas.
发生于鞍区的神经节细胞瘤极为罕见。我们研究了一组此类肿瘤,以探讨其临床表现和预后。2000年1月至2012年12月期间,华山医院有23例患者被诊断为鞍区神经节细胞瘤。对这些患者的病史、内分泌检查、术前磁共振成像(MRI)、病理结果及随访情况进行了回顾性分析。内分泌检查显示,10例患者(43.5%)催乳素(PRL)水平升高,9例患者(39.1%)生长激素(GH)和胰岛素样生长因子1(IGF-1)水平升高。16例患者(69.6%)观察到散在的神经节细胞与腺瘤成分混合存在。其余7例患者(30.4%)仅检测到散在分布于纤维基质中的神经节细胞碎片,无腺垂体成分。免疫组化显示,6例患者(26.1%)为PRL阳性腺瘤,8例患者(34.8%)为GH阳性腺瘤。平均随访时间为4.2年(范围:1 - 12.7年)。20例患者(87.0%)实现了肿瘤全切。1例患者在肿瘤切除后5年复发(4.3%)。1例患者术后6年死于急性心肌梗死。位于鞍区的神经节细胞瘤可能代表一种独特的免疫病理实体。神经节细胞瘤的手术结果和预后与垂体腺瘤相当。