胃肠道神经鞘瘤19例临床病理特点分析及预后

Clinical pathological characteristics and prognosis of gastrointestinal schwannoma in 19 cases

目的探讨胃肠道神经鞘瘤的临床及病理特征,与其他间叶源性肿瘤的鉴别要点及预后。方法回顾性分析我院1999年7月~2017年12月间住院的19例胃肠道神经鞘瘤的临床及病理资料、免疫组化分析。结果肿瘤位于胃部14例,结肠2例,食道、阑尾及肠系膜各1例。其中恶性2例,胃神经鞘瘤同时合并腹壁神经鞘瘤1例。男女比例1:1.4,年龄31~78岁,中位年龄51岁。腹痛、腹胀及腹块最常见。13例行CT,12例显示边界较清晰的圆形或类圆形软组织影,囊样改变1例,密度不均匀伴钙化1例。7例行CT增强,其中6例均匀强化。超声内镜显示固有肌层低回声肿物,边界清,10例内部回声均匀,2例欠均匀。病理上良性17例,恶性2例。肿物无包膜,镜下梭状细胞为主,胞核呈模糊栅栏状排列,外周淋巴细胞套形成,肿块内淋巴细胞浸润。免疫组化S-100和Vimentin强阳性,CD117、SMA、DOG 1、Desmin阴性。4例CD34阳性。17例患者行外科手术治疗,2例患者行内镜下治疗,随访时间3~176个月。1例患者在随访18个月时出现肿瘤复发,二次手术后1年死亡。结论胃肠道神经鞘瘤需注意与胃肠道间质瘤等间叶组织来源肿瘤相鉴别,CT及超声内镜有一定的价值,但确诊主要依赖病理检查及免疫组化检测,S-100蛋白是其重要标志物。绝大部分患者术后预后良好,但肿物较大的患者术后应注意随访。

Objective To investigate the clinical and pathological characteristics of gastrointestinal schwannoma and the identification points from other mesenchymal tumors and investigate the prognosis. Methods The clinical and pathological data and immunohistochemical analysis of 19 cases of gastrointestinal schwannoma hospitalized in our hospital from July 1999 to December 2017 were analyzed retrospectively. Results The tumors were located in the stomach in 14 cases, colon in 2 cases, esophagus, appendix and mesentery in 1 case. Two cases of them were malignant, and one case was gastric schwannoma combined with abdominal schwannoma. Male to female ratio was 1： 1.4, aged 31 to78 years old, with the median age of 51 years old. Abdominal pain, abdominal distension and abdominal mass were the most common symptoms. 13 patients underwent CT, and 12 cases showed clear round or oval soft tissue images. One case had cystic change and one case had uneven density with calcification. 7 cases underwent contrast-enhanced CT,of which 6 cases had uniform enhancement. Endoscopic ultrasound showed that there was hypoechoic mass in muscularis propria, with clear border, and 10 cases had internal echo uniform and 2 cases had less uniform. 17 cases were pathologically benign and 2 were malignant. Tumor had no envelope, mainly with spindle cell in microscopy, and the nucleus was in fuzzy palisade arrangement. The peripheral lymphocytes sleeve was formed, and lymphocytes were infiltrated within the tumor. S-100 and vimentin were strong positive and CD117, SMA, DOG 1, Desmin were negative in immunohistochemistry. And there were 4 cases with CD34 positive. 17 patients underwent surgical treatment. 2 patients underwent endoscopic treatment with a follow-up of 3 to 176 months. 1 patient developed tumor recurrence at 18 months of follow-up and died at 1 year after second surgery. Conclusion Gastrointestinal schwannoma should be distinguished from gastrointestinal stromal tumor derived from mesenchymal tissue. CT and endoscopic ultrasonography have some value, but the diagnosis depends mainly on pathological examination and immunohistochemistry. S-100 protein is an important marker. Most patients have good prognosis after surgery, but those with larger tumors should be paid attention to the follow-up after surgery.