Figure 1: a) initial outpatient CT scan of the chest highlighting the lung mass; b) gross specimen of the lung mass; c) histological image showing spindle cell proliferation with prominent inflammatory infiltrate encroaching on adjacent alveoli; d) histological image highlighting the mixed inflammatory cells including plasma cells, lymphocytes, and eosinophils. 
The patient was referred to pediatric surgery and pediatric oncology and she was reviewed at tumor board. A video assisted thoracoscopy (VATS) with left lower lobe lobectomy was performed three months after the initial hospitalization. The left lower lobe (inclusive of the tumor) and associated axillary nodes were removed en bloc in a via thoracotomy. The tumor was sent to pathology without fixation. Postoperatively the patient recovered quickly, with removal of her chest tube and discharge home on the first postoperative day. A post-op chest x-ray demonstrated pneumothorax. She underwent chest tube placement four weeks after her initial operation due to worsening of pneumothorax following discharge from the hospital and had complete, persistent resolution of the pneumothorax.
Pathological examination showed a spindle cell lesion with associated lymphoplasmacytic and histiocytic inflammation. The final pathologic diagnosis made by an outside facility was an inflammatory myofibroblastic tumor (IMT)/inflammatory pseudotumor with microscopically negative margins and negative lymph nodes. The patient still has residual visual abnormalities in the left eye and regularly has imaging to monitor tumor recurrence in the chest and abdomen.
Neuromyelitis optic spectrum disorders and IMT are uncommon conditions, afflicting both adults and children. NMOSD is an CNS autoimmune disorder in which antibodies to the aquaporin-4 water channel are identified in one-half to two-thirds of children1. IMT are usually benign pulmonary and abdominal neoplasms that most commonly affect children and young adults. While the exact tumor pathogenesis remains uncertain, an overlap exists between IMTs and IgG-4 related disorders. NMOSD is known to be associated with other autoimmune disorders such as systemic lupus erythematosus, Sjogren syndrome, autoimmune thyroid disease, and myasthenia gravis4. However, no link between NMO and IMTs has been identified.
NMOSD often presents with visual deficits, sensory dysfunction and constitutional symptoms like fever, nausea, vomiting, and seizures. In the pediatric population, approximately 30-50% of patients experience optic neuritis during their first NMOSD episode. While NMOSD typically targets the CNS, cases of NMO with inflammatory pulmonary manifestations have been reported2. IMT symptoms are nonspecific, varying with location, such as cough and chest pain for pulmonary IMTs and gastrointestinal symptoms for abdominal IMTs5. In this patient’s case, symptoms primarily aligned with NMOSD, with finger clubbing being the sole pulmonary symptom. Given the correlation between NMOSD and other autoimmune inflammatory disorders, it’s imperative to consider autoimmune or inflammatory pathologies during initial NMOSD diagnosis, particularly in younger patients.
NMOSD is a highly relapsing disorder and therefore long-term immunotherapy is required with rituximab, mycophenolate mofetil, and azathioprine being the most commonly used long term therapies in children. Acute episodes are treated with high dose prednisolone, plasmapheresis, or IVIG1. IMTs are typically managed through complete mass resection with negative margins.
In conclusion, IMT of the lung and neuromyelitis optica spectrum disorders (NMOSD) are both rare inflammatory conditions with low incidence rates. This is the first reported case with pulmonary IMT along with NMOSD.