Inflammatory myofibroblastic tumours: where are we now?

Abstract

Inflammatory pseudotumor is a generic term applied to a variety of neoplastic and non-neoplastic entities that share a common histologic appearance, namely a cytologically bland spindle cell proliferation with a prominent, usually chronic inflammatory infiltrate. Over the last two decades, inflammatory myofibroblastic tumor (IMT) has emerged from within the broad category of inflammatory pseudotumor, with distinctive clinical, pathologic and molecular features. IMT shows a predilection for the visceral soft tissues of children and adolescents and has a tendency for local recurrence but only a small risk of distant metastasis. Characteristic histologic patterns include the fasciitis-like, compact spindle cell and hypocellular fibrous patterns, which are often seen in combination within the same tumor. Chromosomal translocations leading to activation of the ALK tyrosine kinase can be detected in approximately 50% of IMTs, particularly those arising in young patients. This review will examine the clinical, pathologic, and molecular genetic features of IMT and discuss an approach to diagnosis and differential diagnosis.