CAJ | 학술논문

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.
목적 통과분석일개단중심교대양본적소세포폐암(SCLC)상관신경계통부종류종합정(PNS)대렬적림상화실험실특정、치료급예후,지재위중국소세포폐암합병PNS적진치제공경다증거.방법 회고성분석자2001년1월지2014년12월경북경협화의원련속수치적SCLC합병PNS환자34례,남22례,녀12례,평균년령55.4세,분석기림상표현류형、상관항체、치료급부분예후특정.결과 91.2％환자이신경계통위수발증상,기병지학진시간평균위250 d;공진단5충PNS림상류형,기중Lambert-Eaton종합정최상견(13/34,38.2％),의차위감각신경원신경병(10/34,29.4％),아급성소뇌변성(7/34,20.6％),변연성뇌염(6/34,17.6％)화뇌간뇌염(1/34,3％).기중5례(14.7％)표현위신경계통다부위수루;대24례환자진행PNS상관항체검측,9례(37.5％)항Hu항체양성;28례(82.4％)환자접수침대종류적치료,기중4례수술절제종류,기여접수화료.기중5례동시급여정맥병충구단백(IVIG)주사혹격소치료.대1례수술+면역치료환자진행3년수방,술후신경공능개선명현.결론 본연구중절대다수SCLC합병PNS적환자이신경계통증상기병,증상조우종류진단,급시식별,유조우종류적조기진단급치료.SCLC합병PNS가표현다충림상종합정,상관항체검측대급시진단비상중요.PNS목전무특효치료,수술절제종류연합면역치료대개선신경계통증상가능유방조.
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.