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Crizotinib in ALK+ inflammatory myofibroblastic tumors-Current experience and future perspectives
Till-Martin Theilen1, Jan Soerensen2, Konrad Bochennek3
1Department of Pediatric Surgery and Pediatric Urology, Johann Wolfgang Goethe University, Frankfurt, Germany.
Pediatric Blood & Cancer
|December 30, 2017
Summary
Crizotinib shows promise for treating rare inflammatory myofibroblastic tumors (IMT) and epithelioid inflammatory myofibroblastic sarcomas (EIMS). This study found that 12 of 30 patients treated with crizotinib achieved remission, warranting further investigation.
Area of Science:
- Oncology
- Molecular Biology
- Pharmacology
Background:
- Inflammatory myofibroblastic tumor (IMT) and epithelioid inflammatory myofibroblastic sarcoma (EIMS) are rare soft-tissue neoplasms.
- Activating rearrangements of the anaplastic lymphoma kinase (ALK) gene are common in IMT (approx. 50%) and EIMS (100%).
- Targeted inhibition of ALK using kinase inhibitors like crizotinib represents a potential therapeutic strategy.
Purpose of the Study:
- To evaluate the efficacy of crizotinib in patients with IMT/EIMS.
- To review existing literature and analyze treatment outcomes for crizotinib in this patient population.
Main Methods:
- Literature review and analysis of data from 30 patients diagnosed with IMT/EIMS.
- Patients were treated with crizotinib, an ALK inhibitor.
Main Results:
- A total of 12 out of 30 patients (40%) achieved a complete or partial remission.
- These preliminary findings suggest a positive response rate to crizotinib in this cohort.
Conclusions:
- Crizotinib demonstrates promising preliminary efficacy in patients with ALK-positive IMT/EIMS.
- Further prospective studies are warranted to confirm these findings and establish crizotinib as a treatment option for unresectable/multifocal ALK+ IMT/EIMS.
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