Paraneoplastic Neuromyelitis Optica Spectrum Disorder Associated with Atypical Thymic Carcinoid: A Case Report

Ann Thorac Cardiovasc Surg. 2022 Oct 20;28(5):362-365. doi: 10.5761/atcs.cr.20-00354. Epub 2021 Apr 27.

Abstract

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disease, occasionally accompanied by malignant tumors. Immunosuppressive therapy is the mainstay treatment for idiopathic NMOSD; no guidelines have been published for paraneoplastic NMOSD because it is rarely reported in the literature. We report a rare case of a 67-year-old man with paraneoplastic NMOSD associated with thymic carcinoid whose cells expressed aquaporin-4 antibody. After surgical resection, the patient's symptoms improved, and serum aquaporin-4 autoantibody turned negative. We believe that radiographic examination for mediastinal tumors in patients with NMOSD is necessary because thymic epithelial tumors could have a role in the pathogenesis of paraneoplastic NMOSD. After mediastinal tumor has been detected, they should be surgically resected to improve neurological symptoms.

Keywords: paraneoplastic syndrome; thymic cancer; thymothymectomy.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Aquaporin 4
  • Autoantibodies
  • Carcinoid Tumor* / complications
  • Carcinoid Tumor* / diagnostic imaging
  • Carcinoid Tumor* / surgery
  • Humans
  • Male
  • Neuromyelitis Optica* / complications
  • Neuromyelitis Optica* / diagnostic imaging
  • Neuromyelitis Optica* / surgery
  • Treatment Outcome

Substances

  • Aquaporin 4
  • Autoantibodies