4.8 Article

Crizotinib in ALK-Rearranged Inflammatory Myofibroblastic Tumor

Journal

NEW ENGLAND JOURNAL OF MEDICINE
Volume 363, Issue 18, Pages 1727-1733

Publisher

MASSACHUSETTS MEDICAL SOC
DOI: 10.1056/NEJMoa1007056

Keywords

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Funding

  1. National Institutes of Health [R01-CA136851, P01-CA47179, RC2-CA148260]
  2. National Cancer Institute-American Society of Clinical Oncology Cancer Foundation
  3. Cycle for Survival

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Inflammatory myofibroblastic tumor (IMT) is a distinctive mesenchymal neoplasm characterized by a spindle-cell proliferation with an inflammatory infiltrate. Approximately half of IMTs carry rearrangements of the anaplastic lymphoma kinase (ALK) locus on chromosome 2p23, causing aberrant ALK expression. We report a sustained partial response to the ALK inhibitor crizotinib (PF-02341066, Pfizer) in a patient with ALK-translocated IMT, as compared with no observed activity in another patient without the ALK translocation. These results support the dependence of ALK-rearranged tumors on ALK-mediated signaling and suggest a therapeutic strategy for genomically identified patients with the aggressive form of this soft-tissue tumor.

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