小细胞肺癌相关神经副肿瘤综合征的临床特点及治疗  

Clinical features and treatment of paraneoplastic neurological syndrome associated with small cell lung cancer

作　　者：周立新[1] 关鸿志[1] 刘洪生[2] 朱以诚[1] 彭斌[1] 李单青[2] 崔丽英[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院神经科,100730 [2]中国医学科学院北京协和医学院北京协和医院胸外科,100730

出　　处：《中华医学杂志》2015年第37期3023-3026,共4页National Medical Journal of China

摘　　要：目的 通过分析一个单中心较大样本的小细胞肺癌（SCLC）相关神经系统副肿瘤综合征（PNS）队列的临床和实验室特征、治疗及预后,旨在为中国小细胞肺癌合并PNS的诊治提供更多证据.方法 回顾性分析自2001年1月至2014年12月经北京协和医院连续收治的SCLC合并PNS患者34例,男22例,女12例,平均年龄55.4岁,分析其临床表现类型、相关抗体、治疗及部分预后特征.结果 91.2％患者以神经系统为首发症状,起病至确诊时间平均为250 d;共诊断5种PNS临床类型,其中Lambert-Eaton综合征最常见（13/34,38.2％）,依次为感觉神经元神经病（10/34,29.4％）,亚急性小脑变性（7/34,20.6％）,边缘性脑炎（6/34,17.6％）和脑干脑炎（1/34,3％）.其中5例（14.7％）表现为神经系统多部位受累;对24例患者进行PNS相关抗体检测,9例（37.5％）抗Hu抗体阳性;28例（82.4％）患者接受针对肿瘤的治疗,其中4例手术切除肿瘤,其余接受化疗.其中5例同时给予静脉丙种球蛋白（IVIG）注射或激素治疗.对1例手术＋免疫治疗患者进行3年随访,术后神经功能改善明显.结论 本研究中绝大多数SCLC合并PNS的患者以神经系统症状起病,症状早于肿瘤诊断,及时识别,有助于肿瘤的早期诊断及治疗.SCLC合并PNS可表现多种临床综合征,相关抗体检测对及时诊断非常重要.PNS目前无特效治疗,手术切除肿瘤联合免疫治疗对改善神经系统症状可能有帮助.Objective The aim of this study is to explore the clinical characteristics,treatment and prognosis of pareneoplastic neurological syndrome （PNS） associated with small cell lung cancer （SCLC） in a single-center cohort.Methods We retrospectively and consecutively reviewed 34 patients （22 male and 12 female,mean age 55.4 years old） diagnosed as PNS associated with SCLC in our hospital from 2001 to 2014 and investigated the clinical features,related antibodies,treatment and prognosis in this cohort.Results In a total of 34 patients,31 （91.4%） presented with neurological symptoms prior to the diagnosis of cancer.Lambert-Eaton syndrome （13/34,38.2%） was the most common subtype,followed by paraneoplastic sensory neuronopathy （10/34,29.4%）,paraneoplastic cerebellar degeneration （7/34,20.6%）,limbic encephalitis （6/34,17.6%） and brainstem encephalitis （1/34,3%）.Anti-neuronal antibodies were positive in 9 out of a total of 24 patients （37.5%） who performed the tests.There were 28 （82.4%） patients who received the treatment for the primary tumor.Of them,operation of removing the tumor was performed in 4 patients.5 patients received treatment of suppression of the immune response to PNS.Significant neurological improvement was found in a patient who received operation combined with immunotherapy after a 3-year follow-up.Conclusions The majority of patients with PNS are unknown to have cancer at the time of diagnosis.Early recognition of PNS is helpful to the treatment of the tumor.PNS has diverse presentations,affecting both the central and peripheral nervous system,which makes the diagnosis hard.Operation of removing the tumor and immune suppression may slow the immune response,creating the greatest chance for neurologic improvement or symptom stabilization.

关 键 词：神经系统副肿瘤综合征 小细胞肺癌 抗神经元抗体 预后 

分 类 号：R734.2[医药卫生—肿瘤]