【作者】 谢京城； 陈晓东； 钟延丰； 郑丹枫；

【Author】 XIE Jing-cheng;CHEN Xiao-dong;ZHONG Yan-feng;Department of Neurosurgery,Peking University Third Hospital;Department of Pathology,Peking University Health center;

【机构】 北京大学第三医院神经外科； 北京大学医学部病理学系；

【摘要】 目的:总结椎管内外哑铃型节细胞神经瘤的临床特点及影像学特征,探讨其手术治疗效果。方法:回顾性分析我院自2001年6月～2018年11月手术治疗并经过病理学证实的椎管内外哑铃型节细胞神经瘤患者16例,男9例,女7例,年龄21～65岁,平均年龄43.7岁。9例以根性疼痛为主要表现,7例以肿瘤压迫平面以下肌力下降为主要表现,查体10例患者有与肿瘤所在水平相关的感觉障碍平面。MRI检查显示肿瘤在T1WI呈低信号、T2WI呈高信号,增强后有强化;在轴位像上肿瘤位于髓外-硬膜内外椎管内外者7例,9例肿瘤位于硬脊膜外-椎间孔内外呈哑铃形;C₁～C₂水平者5例,C₃～C₄ 2例,C₄～C₅ 2例,C₅～C₆ 2例,C₄～C₆ 1例,T₃～T₄ 1例,T₆～T₇ 1例,T₉～T₁₀1例,T₁₁～L₄ 1例。采用患侧半椎板-小关节突切除显露,于显微镜下行肿瘤分离切除。5例哑铃形肿瘤椎管外部分较大,附加后外侧入路切除椎旁部分。结果:肿瘤全切除15例,次全切除1例。术后病理检查,可见成熟或未成熟的肿瘤性节细胞散在或成束穿插于肿瘤性雪旺氏细胞间质中,肿瘤中可见正常的神经纤维呈束状排列。术后发生脑脊液漏3例。术前9例神经根痛患者,7例术后症状缓解或消失,2例无变化。7肢体运动障碍者,5例术后功能改善,2例无变化。随访0.5年～17.3年,平均8.2年,末次随访时,脊髓功能状态按Mc Cormick分级标准评价,好转14例,不变2例。随访期间,1例术后2年肿瘤原位硬膜下复发,再次手术切除,14例无复发,1例肿瘤椎旁残余无变化。结论:椎管内外哑铃型节细胞神经瘤临床上以根性疼痛及感觉运动障碍为主要表现,肿瘤主体大多数于硬膜外,沿椎间孔向椎旁呈哑铃形生长趋势,手术全切除后预后良好。更多还原

【Abstract】 Objective: To investigate the clinical manifestation and surgical outcome of ganglioneuroma of spine. Methods: From June 2001 to November 2018, 16 patients with spine ganglioneuromas surgically treated were analyzed retrospectively. There were 9 males and 7 females with average age of 43.7 years（range from 21 ～ 65 years）. Clinically, 9 patients presented with radicular neuralgia, and 7 patients presented with motor deficit below the mass effects, 10 patients showed sensory deficit below the lesion of cervical spinal cord on neurological physical examination. MRI revealed low density on T1 weighted, and high density signal on T2 weighted images with apparent enhancement. The tumors located in the extramedullary-subdural and/or epidural in 7 cases, and 9 cases in epidural space, both 16 cases presented of dumbbell shaped across neural foramen. There were 5 cases located at the level of C₁～C₂,2 cases at C₃～C₄,2 case at C₄～C₅,2 cases at C₅～C₆,and 1 case at C₄～C₆. 3 cases at Thoracic spine, and 1 case at T₁₁ ～L₄. The surgical approach included hemilaminectomy and facetactomy on the lesion side, 5 additional procedures were performed for removal of the para-spinal part of dumbbell shaped tumor. The tumors were dissected and removed under microscope. Results: 16 operations had been performed with gross total of tumor resection in 15 cases and subtotal resection of tumor in 1 case. Pathologically, ganglioneuromas show mature or degenaration ganglion cells in neoplastic Schwann stroma containing normal spindle shaped cells. Surgical complications included CSF leakage in 3 patients. Clinically, in the 9 patients with neuralgia prior operation, there were 7 patients achieved relief while other 2 patients were no change. Among the 7 patients with upper and lower limbs motor deficit pre-operation, there were 5 patients had been improved, 2 patients no change. The period of follow-up was ranged from 0.5 year to 17.3 years（with average of 8.2 years）. Neurological status had been improved in 14 patients no change in 2 patient evaluated by McCormick grading scale of spinal function status during the last follow-up. During the follow-up, 1 tumor recurred at the same site of subdural space in 2 years post-operation which dictated redo operation for removal. There was no recurrence in the other 14 cases and no change in 1 case which with para-spinal part residual. Conclusions: Spine ganglioneuroma present with chronic radicular neuralgia, sensory and motor deficit clinically. The ganglioneuroma usually presented as dumbbell shaped across neural foramen mostly located extradural space. Surgical excision is the primary treatment of choice, and total resection of the tumor guarantee satisfactory outcome.更多还原