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

Abstract

Objective: To investigate the clinical features of patients involved with both malignant tumors and neuromyelitis optica spectrum disorders (NMOSD). Methods: The 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. Results: Most 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 maintained stable; while, among all the 9 patients received immunosuppressive agents and anti-tumor therapy, only one case had relapse. Conclusions: There are some differences in the clinical features between the NMOSD patients with malignant tumors and the NMOSD patients without malignant tumors. Immunosuppressive therapy can improve the prognosis of patients with NMOSD and tumor, without increasing the risk of malignant tumor. The pathological type, staging and antitumor therapy may influence the prognosis of NMOSD. NMOSD patients with malignant tumor could be treated with anti-tumor and immunosuppressive agents if needed.