Crizotinib in ALK-Rearranged Inflammatory Myofibroblastic Tumor

By J. Butrynski, D. D’Adamo, J. Hornick, P. Cin, C. Antonescu, S. Jhanwar, Marc Ladanyi, M. Capelletti, S. Rodig, N. Ramaiya and 8 more. New England Journal of Medicine, 2010

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SUMMARY 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.

Subfields: Epidemiology, Oncology, Rheumatology

View Paper: https://www.semanticscholar.org/paper/4b97d39d440e086a47197d84842dabb9645bc1e3

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In Epidemiology

  1. E. Ewing
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In Rheumatology

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