腰椎椎管内副神经节瘤的临床表现

Clinical characteristics of lumbar intraspinal paragangliomas

目的总结腰椎椎管内副神经节瘤的临床特点。方法回顾性分析5例腰椎椎管内副神经节瘤的临床资料,术前诊断为室管膜瘤1例,神经鞘瘤4例。均采用脊柱后正中入路手术切除肿瘤。结果所有病人均获得肉眼或镜下全切除。术后临床表现不同程度缓解4例,仍有腰腿痛1例。术后未出现明显的神经系统并发症。所有病例均经神经病理学证实为副神经节瘤。免疫组化:嗜铬颗粒素A(CgA)阳性,胶质纤维酸性蛋白(GFAP)阴性,细胞核增殖相关抗原Ki-67的单克隆抗体(MIB-1)的指标指数(LI)为2%。经MRI随访3例,时间9～24个月,均未复发,有较好的生活质量。结论腰椎椎管内副神经节瘤的临床症状及影像学检查无特异性表现,其确诊依靠病理学检查,手术全切除是治疗椎管内副神经节瘤的首选方法,预后良好,复发率低。

Objective To summarize the clinical characteristics of lumbar intraspinal paragangliomas（LIP）.Methods The clinical data of 5 patients with LIP were analyzed retrospectively,including 1 ependymoma and 4 neurilemmomas diagnosed preoperatively.All the tumors were resected via posterior midline approach.Results Total resection was achieved in all the tumors microscopically or macroscopically.The clinical manifestations improved to different degree occurred in 4 cases,lumbocrural pain still existed in 1 and no obvious neurological complication postoperatively.All the tumors were confirmed as paragangliomas by neuropathological diagnosis.Immunohistochemistry was positive for chromogranin A（CgA） and negative for glial fibrillary acidic protein（GFAP）.MIB-1 LI was 2%.During the follow-up period from 9 to 24 months by MRI,no recurrence was found in 3 patients who had good quality-of-life.Conclusions There is no specificity in clinical symptoms and imaging findings of LIP,diagnosis of which depends on pathological examination.Total resection by surgery is the first-line treatment for LIP,with a good prognosis and low recurrence rate.