Abstract
We report a rare case of paraneoplastic neuromyelitis optica spectrum disorder (NMOSD) in a 38 year-old woman with anaplastic ependymoma. She was diagnosed with right parieto-occipital ependymoma after a gross total resection at the age of 16 years. A few months after initial resection, she underwent a second resection with intraoperative radiation for tumor recurrence. At the age of 18 years, she had tumor recurrence for the third time for which she underwent re-resection of anaplastic ependymoma. Yearly surveillance imaging showed stability of the tumor up until age 38 years. Recurrence at age 38 years was diagnosed following work-up of new onset left eye optic neuritis. MRI brain also revealing a new 1.5 cm enhancing nodule along the inferolateral margin of the right occipital resection cavity as well as new FLAIR hyperintensity in the left insular and postrema. Resection of enhancing tumor revealed recurrent anaplastic ependymoma. Serum was positive for neuromyelitis optica immunoglobulin G antibody anti-aquaporin-4. Simultaneous occurrence of ependymoma recurrence and first clinical NMO flare, supports the consideration of paraneoplastic neuromyelitis optica from ependymoma. While most NMOSD cases are idiopathic, it can rarely be paraneoplastic in etiology. Various specific malignancies have been found to be associated with NMOSD including breast carcinoma, lung, nasopharynx, cervical, bladder, prostate, lymphoma, thymoma, and pituitary adenoma. More rare cases have described paraneoplastic NMOSD in serous ovarian carcinoma and adrenocortical carcinoma. No cases have been reported associating paraneoplastic NMOSD with an ependymoma. Paraneoplastic syndrome is an extremely rare complication of ependymomas. We present the first case of paraneoplastic NMOSD associated with an ependymoma. This case shows that paraneoplastic NMOSD can occur with ependymoma and highlights the importance of testing for paraneoplastic etiology in ependymoma patients with clinical and radiographic features of NMOSD.