Crizotinib in ALK-rearranged inflammatory myofibroblastic tumor

Butrynski, J. E., D'Adamo, D. R., Hornick, J. L., Dal Cin, P., Antonescu, C. R., Jhanwar, S. C., Ladanyi, M., Capelletti, M., Rodig, S. J., Ramaiya, N., Kwak, E. L., Clark, J. W., Wilner, K. D., Christensen, J. G., Janne, P. A., Maki, R. G., Demetri, G. D., Shapiro, G. I. (October 2010) Crizotinib in ALK-rearranged inflammatory myofibroblastic tumor. N Engl J Med, 363 (18). pp. 1727-33. ISSN 1533-4406 (Electronic)0028-4793 (Linking)

DOI: 10.1056/NEJMoa1007056


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. (Funded by Pfizer and others; number, NCT00585195.).

Item Type: Paper
Uncontrolled Keywords: Abdominal Neoplasms/*drug therapy/genetics Adult Granuloma, Plasma Cell/*drug therapy/genetics Humans Male Mutation Neoplasms, Muscle Tissue/*drug therapy Protein Kinase Inhibitors/adverse effects/*therapeutic use Protein-Tyrosine Kinases/*antagonists & inhibitors/*genetics Proto-Oncogene Proteins c-met/antagonists & inhibitors Pyrazoles/adverse effects/*therapeutic use Pyridines/adverse effects/*therapeutic use Receptor Protein-Tyrosine Kinases Receptors, Growth Factor/antagonists & inhibitors Young Adult
Subjects: diseases & disorders > cancer
diseases & disorders > cancer > drugs and therapies
CSHL Authors:
Communities: CSHL labs > Maki lab
Depositing User: Matt Covey
Date: 28 October 2010
Date Deposited: 25 Oct 2016 15:44
Last Modified: 25 Oct 2016 15:44
PMCID: PMC3014292
Related URLs:

Actions (login required)

Administrator's edit/view item Administrator's edit/view item
CSHL HomeAbout CSHLResearchEducationNews & FeaturesCampus & Public EventsCareersGiving