| Literature DB >> 29286567 |
Till-Martin Theilen1, Jan Soerensen2, Konrad Bochennek3, Martina Becker3, Dirk Schwabe3, Udo Rolle1, Thomas Klingebiel2,3, Thomas Lehrnbecher3.
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.Entities:
Keywords: anaplastic lymphoma kinase (ALK); child; crizotinib; epitheloid inflammatory myofibroblastic sarcoma (EIMS); inflammatory myofibroblastic tumor (IMT)
Mesh:
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Year: 2017 PMID: 29286567 DOI: 10.1002/pbc.26920
Source DB: PubMed Journal: Pediatr Blood Cancer ISSN: 1545-5009 Impact factor: 3.167