Inflammatory myofibroblastic tumor of the uterus (uIMT) is rare. However, it has been increasingly recognized in recent years, largely due to more awareness of its occurrence in the gynecologic tract and the characterization of features that help distinguish it from more common lesions in the differential diagnosis, particularly smooth muscle neoplasms. One of these features is expression of anaplastic lymphoma kinase (ALK, also known as CD246), which has been documented in most uIMTs described in the literature. This review concentrates on the role of ALK testing in the diagnosis and management of uIMT. In terms of immunohistochemistry, an emphasis on antibody selection, sensitivity/specificity, interpretation and quality control is given. Regarding molecular analysis for ALK alterations, this review appraises fluorescence in situ hybridization and RNA sequencing technologies. Lastly, the role of tyrosine kinase inhibitor therapy in patients with uIMT is discussed, highlighting the importance of a correct diagnosis of this entity.
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