Crizotinib in ALK+ inflammatory myofibroblastic tumors-Current experience and future perspectives

Pediatr Blood Cancer. 2018 Apr;65(4). doi: 10.1002/pbc.26920. Epub 2017 Dec 29.


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).

Publication types

  • Review

MeSH terms

  • Anaplastic Lymphoma Kinase / antagonists & inhibitors*
  • Crizotinib / therapeutic use*
  • Female
  • Humans
  • Inflammation / drug therapy
  • Inflammation / enzymology
  • Male
  • Neoplasm Proteins / antagonists & inhibitors*
  • Neoplasms, Muscle Tissue / drug therapy*
  • Neoplasms, Muscle Tissue / enzymology*


  • Neoplasm Proteins
  • Crizotinib
  • ALK protein, human
  • Anaplastic Lymphoma Kinase