背景:在过去的十年中,关于肿瘤与视神经脊髓炎谱系障碍(NMOSD)之间的关联的报道越来越多。然而,肿瘤的类型和时间关系尚未得到广泛研究。
目的:报告病例并确定肿瘤与NMOSD之间的关系。
方法:对大学医院可能的副肿瘤NMOSD患者进行回顾性分析。系统检索并回顾了与“肿瘤”和“NMOSD”相关的文章。我们纳入了水通道蛋白4(AQP4)-IgG血清阳性的NMOSD患者,其NMOSD的发作和癌症诊断或复发在24个月内。时间关系,涉及的肿瘤类型,治疗,并确定了NMOSD和肿瘤的结局。亚组分析基于肿瘤组织学的AQP4表达。
结果:我们从我院224例AQP4-IgG血清阳性NMOSD的队列中确定了3例(1.3%),并从系统评价中检索了68例,共71例可能的副肿瘤NMOSD。NMOSD发病的中位年龄为55岁(IQR41-64)。80%是女性。最常见的肿瘤类型是肺和乳腺,占21.1%和18.3%,分别。其他肿瘤类型为卵巢肿瘤和血液系统恶性肿瘤,均为12.7%。最常见的组织组织学是腺癌(52.1%)。我们还报道了NMOSD患者中的第一例黑色素瘤。28名患者(39.4%)在NMOSD发病前被诊断为癌症,中位持续时间为9.5个月(范围1-24个月)。其中,8例患者在手术切除肿瘤后出现NMOSD,1例前列腺腺癌放疗后出现NMOSD。23例患者(32.4%)在癌症诊断前有NMOSD,中位时间为3个月(范围1-24个月),其余患者在同一入院期间同时诊断。3例患者在肿瘤复发前后诊断为NMOSD。82.4%的肿瘤组织表达AQP4。
结论:一小部分AQP4-IgG阳性NMOSD与恶性肿瘤相关。在没有肿瘤症状的新诊断的NMOSD患者中,应建议筛查适合年龄和风险的癌症,与一般人口相似。癌症患者发生NMOSD可能提示肿瘤复发。
BACKGROUND: An increasing number of
reports on associations between neoplasms and neuromyelitis optica spectrum disorder (NMOSD) have been published over the past decade. However, types of neoplasms and temporal relationships have not been widely studied.
OBJECTIVE: To report cases and determine the associations between neoplasms and NMOSD.
METHODS: A retrospective chart review of possible paraneoplastic NMOSD patients at a university hospital was performed. Articles related to \"neoplasm\" and \"NMOSD\" were systematically searched and reviewed. We included aquaporin-4 (AQP4)-IgG-seropositive NMOSD patients whose onset of NMOSD and cancer diagnosis or recurrence were within 24 months of one another. Temporal relationship, types of neoplasms involved, treatments, and outcomes of both NMOSD and neoplasms were determined. The subgroup analysis was based on the
AQP4 expression of neoplasm histology.
RESULTS: We identified 3 cases (1.3%) from a cohort of 224
AQP4-IgG-seropositive NMOSD at our hospital and retrieved 68 cases from a systematic review, totaling 71 cases of possible paraneoplastic NMOSD. The median age at onset of NMOSD was 55 (IQR 41-64) years. Eighty percent were female. The most frequently identified types of neoplasms were lung and breast, accounting for 21.1% and 18.3%, respectively. The other tumor types were ovarian tumors and hematologic malignancy, both at 12.7%. The most commonly identified tissue histology was adenocarcinoma (52.1%). We also reported the first
case of melanoma in an NMOSD patient. Twenty-eight patients (39.4%) were diagnosed with cancer before the onset of NMOSD with a median duration of 9.5 (range 1-24) months. Of those, eight patients had NMOSD after surgical removal of neoplasms, and one patient had NMOSD after radiotherapy of prostate adenocarcinoma. Twenty-three patients (32.4%) had NMOSD before cancer diagnosis by a median of 3 (range 1-24) months, and the rest were diagnosed concurrently during the same admission. Three cases were diagnosed with NMOSD around the time of tumor recurrence. Tumor tissue expressed
AQP4 in 82.4%.
CONCLUSIONS: A small proportion of AQP4-IgG-positive NMOSD is associated with malignancy. In newly diagnosed NMOSD patients without symptoms of neoplasms, screening for age- and risk-appropriate cancer should be recommended, similar to the general population. The occurrence of NMOSD in cancer patients might suggest tumor recurrence.