Crizotinib in ALK+ inflammatory myofibroblastic tumors-Current experience and future perspectives

Abstract

Inflammatory myofibroblastic tumor (IMT) and its subtype epithelioid inflammatory myofibroblastic sarcoma (EIMS) are rare soft-tissue tumors. As about 50% of IMT and 100% of EIMS contain activating rearrangements of the anaplastic lymphoma kinase (ALK) gene, targeted kinase inhibition of ALK by compounds such as crizotinib is a potential treatment option. We performed a literature review and analyzed a total of 30 patients with IMT/EIMS treated with crizotinib. A total of 12 patients achieved complete or partial remission. As preliminary data are promising, a prospective study evaluating crizotinib treatment in patients with unresectable/multifocal ALK⁺ IMT/EIMS is warranted.

Keywords: anaplastic lymphoma kinase (ALK); child; crizotinib; epitheloid inflammatory myofibroblastic sarcoma (EIMS); inflammatory myofibroblastic tumor (IMT).