Central nervous system inflammatory myofibroblastic tumor masquerading as chronic suppurative otitis media

Abstract

Inflammatory myofibroblastic tumor (IMT) is a rare tumor in the central nervous system (CNS), mostly being extracranial. Approximately 100 sporadic cases have been reported in the literature. The rarity of the tumor, its various histopathological characteristics, and its variable aggressive course render it difficult to diagnose and treat. IMT is generally a histological diagnosis which is rarely suspected preoperatively. It mimics other intracranial tumors such as giant cell tumor, hemangiopericytoma, anaplastic meningioma, plasmacytoma, and lymphoma. Rarely, it can present with a clinical picture which mimics a benign infective process, Rosai-Dorfman disease, or an idiopathic hypertrophic pachymeningitis. High index of suspicion is required as total resection of this lesion is mandatory to prevent recurrence. Here, we describe a case of a 10-year-old child which initially presented with clinical features mimicking chronic suppurative otitis media and radiological presentation of a small intracranial abscess. He was initially treated by an ENT surgeon who started him on intravenous antibiotics, but the patient was lost to follow up. He returned after 2 months with a large lesion at the same location. Histological examination revealed multiple spindle cells with plasma cells and lymphocytes scattered among these spindle cells. The spindle cells were immunopositive for smooth muscle actin and negative for epithelial membrane antigen, S100, and CD34.

Intracranial Inflammatory Myofibroblastic Tumor: A Literature Review and a Rare Case Misdiagnosed as Acoustic Neuroma

“…IMT is a rare junctional/low-grade tumor with the potential for recurrence and progression [1]; it is mainly composed of myofibroblast spindle cells with inflammatory cell infiltration, including plasma cells, lymphocytes, and eosinophils [2]. In 2002, the WHO officially defined it as a soft tissue tumor and named it IMT [3].…”

Cognitive evaluation following the evolution of brain myofibroblastic tumor in the adolescence: a case study report

“…The inflammatory myofibroblastic tumor (IMT) is also described as inflammatory pseudotumor or plasma cell granuloma, among the benign or low malignancy potential lesions. IMT is a rare neoplasm composed of myofibroblastic fusiform cells, usually accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils 3,4 and occurs mainly in soft tissues and the viscera of children and young adults 3,5 . CNS tumors, in general, are capable of producing signs and symptoms through local invasion, compression of adjacent structures, and increased intracranial pressure.…”