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

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 comp...

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Bibliographic Details
Published in:Pediatric blood & cancer 2018-04, Vol.65 (4), p.n/a
Main Authors: Theilen, Till‐Martin, Soerensen, Jan, Bochennek, Konrad, Becker, Martina, Schwabe, Dirk, Rolle, Udo, Klingebiel, Thomas, Lehrnbecher, Thomas
Format: Article
Language:English
Subjects:
anaplastic lymphoma kinase (ALK)
child
crizotinib
epitheloid inflammatory myofibroblastic sarcoma (EIMS)
Hematology
Inflammation
inflammatory myofibroblastic tumor (IMT)
Literature reviews
Lymphoma
Oncology
Pediatrics
Protein-tyrosine kinase
Remission
Sarcoma
Targeted cancer therapy
Therapeutic applications
Tumors
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Summary: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.
ISSN:1545-5009
1545-5017
DOI:10.1002/pbc.26920