视神经脊髓炎谱系疾病合并恶性肿瘤临床特征及预后分析  

Analysis of clinical characteristics and prognosis of neuromyelitis optic spectrum disease complicated with malignant tumor

作　　者：黄巧[1] 徐竹 李蕊[1] 王玉鸽[1] 舒崖清[1] 杨渝[1] 胡学强[1] 楚兰 邱伟[1] Huang Qiao;Xu Zhu;Li Rui;Wang Yuge;Shu Yaqing;Yang Yu;Hu Xueqiang;Chu Lan;Qiu Wei(Department of Neurology,the Third Affiliated Hospital of Sun Yat-sen University,Guangzhou 510630,China)

机构地区：[1]中山大学附属第三医院神经内科,广州510630 [2]贵州医科大学附属医院神经内科

出　　处：《中华医学杂志》2018年第31期2496-2500,共5页National Medical Journal of China

摘　　要：目的探讨视神经脊髓炎谱系疾病（NMOSD）合并恶性肿瘤患者的临床特征及预后。方法回顾性分析2012年6月至2017年3月在中山大学附属第三医院及贵州医科大学附属医院神经科收治的473例NMOSD患者临床资料，筛查出11例NMOSD合并恶性肿瘤患者（3例乳腺癌、2例宫颈癌、2例直肠癌、2例白血病、1例鼻咽癌、1例甲状腺癌），并与50例未合并恶性肿瘤的NMOSD患者进行对比分析。结果NMOSD患者多合并低－中分化鳞癌/腺癌，常见部位依次为乳腺、生殖、消化及血液系统。合并恶性肿瘤的NMOSD与未合并恶性肿瘤的NMOSD在性别比例、自身抗体方面相似，但前者发病年龄相对晚，脑部首发症状比例及复发风险更低，而首发EDSS评分、脑脊液中蛋白含量、延髓极后区首发症状及影像学特异性病灶比例更高。8例我院诊断的合并恶性肿瘤NMOSD病例中，2例合并乳腺癌及1例宫颈癌NMOSD预后良好（随访EDSS评分〈3分），此3例患者均在肿瘤发生期进行积极手术及化疗；其他5例预后欠佳（随访EDSS评分≥3分）。11例患者均接受抗肿瘤治疗，4例在抗肿瘤治疗后出现NMOSD首次发作，抗肿瘤治疗后均无复发，其余7例患者仅1例NMOSD复发；9例患者接受免疫抑制剂治疗，7例NMOSD无复发，8例肿瘤病情稳定；同时接受免疫抑制剂及抗肿瘤治疗的9例患者，仅1例出现NMOSD及肿瘤复发。结论合并恶性肿瘤NMOSD患者与未合并肿瘤NMOSD在临床特点方面存在一定差异。免疫抑制剂治疗可改善NMOSD及肿瘤预后，且不增加恶性肿瘤风险；肿瘤病理类型及分期、抗肿瘤治疗方案可能影响NMOSD预后。合并肿瘤NMOSD患者可行抗肿瘤治疗及免疫抑制剂治疗。ObjectiveTo investigate the clinical features of patients involved with both malignant tumors and neuromyelitis optica spectrum disorders （NMOSD）.MethodsThe clinical data of 473 patients with NMOSD admitted into the Third Affiliated Hospital of Sun Yat-sen University and the Department of Neurology, Affiliated Hospital of Guizhou Medical University from June 2012 to March 2017 were retrospectively analyzed. Eleven NMOSD patients complicated with malignant tumors were screened out （3 with breast cancer, 2 with cervical cancer, 2 with rectal cancer, 2 with leukemia, 1 with nasopharyngeal carcinoma, 1 with thyroid cancer）. Fifty patients without NMOSD were included as controls.ResultsMost of the NMOSD patients were complicated by low-moderately differentiated squamous cell carcinoma/adenocarcinoma, mainly seen in breast, reproductive system, digestive system and hematological system. In terms of sex ratio and autoantibodies, the NMOSD patients with and without malignant tumors showed no significant difference. However, comparing to the patients without malignant tumor, the ones with malignant tumor showed a tendency of lower rate of initial brain symptoms and relapse rate, while with older onset age, higher initial EDSS score, protein content in cerebrospinal fluid （CSF）, higher rates of initial symptom resulted from the focus of posterior region of the medulla and of significant image focus. Of the 8 NMOSD patients who diagnosed as malignant tumors in our hospital, 2 with breast cancer and 1 with cervical cancer had a good prognosis （follow-up EDSS score 〈3）. All the 3 patients received aggressive surgery and chemotherapy treatment. However, the other 5 patients had poor prognosis （follow-up EDSS score ≥3 points）. All the 11 patients received anti-tumor therapy, 4 patients had first NMOSD attack after anti-tumor treatment and no relapse. Only one case from the remaining 7 patients had relapse; Among the 9 patients received immunosuppressive therapy, 7 patients had no relapse, and 8 cases mai

关 键 词：视神经脊髓炎谱系疾病 恶性肿瘤 临床特征 预后 

分 类 号：R744.52[医药卫生—神经病学与精神病学] R73[医药卫生—临床医学]