原发性马尾副神经节瘤35例临床分析

Retrospective analysis on clinical features of 35 cases primary cauda equina paragangliomas

目的分析马尾副神经节瘤35例的临床特点以便进行正确诊断与治疗。方法 2017年6月,徐州医科大学附属连云港医院神经外科收治1例经病理确诊为马尾副神经节瘤的患者。并通过检索1989年10月—2017年3月万方期刊数据库、中国知网期刊数据库和维普中文期刊数据库,搜集原发性马尾副神经节瘤患者35例,对其临床特点进行分析。结果 35例患者中男19例,女16例,平均发病年龄为(48.7±14.6)岁;主要临床表现为腰痛伴下肢疼痛21例(60.0%)、仅腰痛7例(20.0%)以及腰痛伴下肢感觉减退4例(11.4%);肿瘤在T1WI上呈等信号19例(73.1%),低信号5例(19.2%),在T2WI上呈高信号18例(69.2%),等信号6例(23.1%),增强后呈不均匀强化13例(81.2%);肿瘤涉及椎体单位78个,其中腰椎单位64个(82.1%),与马尾神经根或终丝关系密切,血供丰富;易误诊为神经鞘瘤、室管膜瘤或脊膜瘤等椎管内肿瘤;嗜铬素(Cg A)和突触素(Syn)是该肿瘤病理学检查的特征性标志物;手术全切预后良好,需要长期随访。结论马尾副神经节瘤是一类罕见的脊柱肿瘤,术前诊断较困难,了解其临床特点是进行正确诊断与治疗的关键。

Objective To investigate the clinical features of cauda equina paragangliomas and improve the accuracy of diagnosis and treatment.Methods The data of primary cauda equina paragangliomas were retrieved and collected from Wanfang periodical database,CNKI database and VIP database for analyzing the clinical features on 35 cases of primary cauda equina paraganglioma in China.Results The patients had an average age of(48.7±14.6)years old and a male preference(54.3%);The most common three clinical symptoms were lumbago with lower limbs pain(60.0%),lumbago only(20.0%)and lumbago with lower limb hypoesthesia(11.4%);The tumors showed isointense(73.1%)or hypointense(19.2%)on T1 weighted image(T1WI),hyperintense(69.2%)or isointense(23.1%)on T2 weighted image(T2WI)and heterogeneous enhancement on contrast enhanced T1 weighted image(81.2%);The tumors were commonly found in the lumbar region(82.1%),adhered to the auda equina nerve roots or filum with abundant blood supply;Chromogranin A(CgA)and synaptophysin(Syn)could be regarded as characteristic biological markers of tumor pathology;Total resection was first choice with good prognosis.Conclusion Cauda equina paragangliomas are rare in spine with a high misdiagnosis probability.Preoperative diagnosis is difficult,and understanding the clinical features can be helpful to make proper diagnosis and treatment.