Abstract
Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm that is characterized by a proliferation of myofibroblastic and fibroblastic spindle cells, accompanied by an inflammatory infiltrate that is abundant in plasma cells, lymphocytes, and eosinophils. IMT can arise in various anatomical locations but most commonly occurs in the abdominal cavity, retroperitoneum, and lung, particularly in children and young adults. IMT typically demonstrates local invasion or recurrence, whereas metastasis is rare. IMTs pose a diagnostic challenge because of their overlapping morphological characteristics with a heterogeneous group of nonneoplastic and neoplastic lesions. Precise diagnosis is crucial for optimal management and accurate prognostication. Despite recent advancements in IMT diagnosis and treatment, its biological complexity and clinical management remain challenging due to significant histological heterogeneity and molecular genetic diversity. This review provides comprehensive updates on the clinical, molecular, and pathological characteristics of IMT, highlighting the diagnostic approaches and key differential diagnoses.