The successful long-term management of an intracranial inflammatory myofibroblastic tumor with corticosteroids

Abstract

Inflammatory myofibroblastic tumors (IMFTs) are a clinically nd histologically heterogeneous group of inflammatory lesions of nknown aetiology, which are most commonly described in the ungs, but have been described in most organs including: ovary, ancreas, liver, stomach, kidney, bladder, thyroid, orbit, breast, and entral nervous system [1–3]. Intracranially located lesions are often diagnosed at biopsy of n unspecified intracranial mass or suspected meningioma [2,4]. hey are typified by an inflammatory infiltrate with fibrous tissue nd abundant polyclonal plasma cells [1,4]. Due to the low incience of disease and difficulty with nomenclature the number of revious intracranial cases has probably been underestimated [1]. f the cases reported in the literature the most common treatent has been debulking surgery with or without radiotherapy, lthough anti-metabolites and both “bolus” and long-term steroids ave been tried with varying degrees of success [2,4,5]. However, he incidence of the disease is so low, and the pathology so varied, hat there is no bank of evidence to develop an optimum treatment trategy appropriate for the histopathological appearance of the esion. Here we describe the successful long-term treatment of an ntracranial IMFT with corticosteroids alone, and refer to the availble literature to justify this method as an alternative treatment ption in cases where surgery is high-risk or impossible.