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Crizotinib in ALK+ inflammatory myofibroblastic tumors—Current experience and future perspectives |
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| Authors: | Till‐Martin Theilen Jan Soerensen Konrad Bochennek Martina Becker Dirk Schwabe Udo Rolle Thomas Klingebiel Thomas Lehrnbecher |
| Affiliation: | 1. Department of Pediatric Surgery and Pediatric Urology, Johann Wolfgang Goethe University, Frankfurt, Germany;2. Division of Pediatric Stem Cell Transplantation and Immunology, Hospital for Children and Adolescents*, Johann Wolfgang Goethe University, Frankfurt, Germany;3. Division of Pediatric Hematology and Oncology, Hospital for Children and Adolescents, Johann Wolfgang Goethe University, Frankfurt, Germany |
| 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) |