Neuromyelitis optica spectrum disorders (NMOSD) is a rare autoimmune demyelinating disorder primarily affecting the spinal cord and optic nerve. Pediatric patients account for 4% of all NMOSD cases, with the average age of onset at 12 years old¹. On the other hand, Inflammatory myofibroblastic tumor (IMT) is an uncommon, slow-growing neoplasm, comprising around 0.1% of all neoplasms³. IMT typically impacts the lungs in children and young adults⁵. Despite documented cases of pulmonary manifestations in NMOSD and nervous system involvement in pediatric IMT cases³, the simultaneous occurrence of NMOSD and IMT has not been recorded. In this report, we present a case of NMOSD in a 10-year-old child from central Texas, who was also subsequently diagnosed with an inflammatory myofibroblastic tumor in the left lower lung lobe.

A 10-year-old girl with a medical history of myopia corrected with lenses, visited the emergency department due to a 2–3-day history of left eye blurriness Initially manifesting as a ”line near the bottom of her eye,” her vision problems progressed to a larger spot, eventually causing loss of vision in the lower midline quadrant of her left eye. She also experienced vomiting and finger clubbing. Evaluation in the emergency department revealed bilateral papilledema and an afferent pupillary defect in the left eye. Upon further assessment, MRI of the brain, orbits and spine showed left-sided optic neuritis and signal abnormalities in the cervical and thoracic spine. Our patient subsequently had an autoimmune work up, and serum aquaporin-4 testing was the only antibody that returned positive at a titer of 1:100 leading to a diagnosis of NMSOD.

The patient underwent a high-dose methylprednisolone treatment for five days. Given the inadequate improvement in her left eye’s vision, plasmapheresis was initiated on hospital day 5. Mild improvement was observed after the third plasmapheresis session on day 8. After her fourth plasmapheresis session, the patient reported her vision in her left eye improved to where she could see outlines but not colors. Due to hypotension with her fourth and fifth plasmapheresis sessions, after her fifth session (hospital day 12), the decision was made to discontinue further plasmapheresis sessions. An intravenous immunoglobulin (IVIG) regimen, 1 g/kg per day, was initiated for two days. By hospital day 15, the patient’s left eye could clearly see nearby objects and distinguish outlines from a distance. Following discharge, the patient started rituximab therapy.

One month later, during an outpatient workup by pediatric pulmonology for bilateral finger clubbing, a chest x-ray demonstrated a left lower lobe lung mass. A contrasted CT scan of the chest was performed for further evaluation. It demonstrated a 2.7 x 3.1 x 4.1 cm mass in the left lower lung lobe and fullness of the pancreatic head.