See article, p. 889.
Inflammatory myofibroblastic tumor (IMT) is a rare soft-tissue tumor that predominantly affects children and young adults. IMT is usually treated by surgical resection, but there is no standard therapy for patients with unresectable or advanced IMT. Activating ALK gene fusions occur in approximately half of IMTs, and a patient with ALK-positive IMT was recently shown to have a partial response to the ALK inhibitor crizotinib. However, no actionable genetic alterations have been reported in ALK-negative IMT. Lovly and colleagues performed targeted next-generation–based genomic profiling on archival tissue from an 8-year-old patient with unresectable, treatment-refractory ALK-negative IMT and identified a TFG–ROS1 fusion. As crizotinib also inhibits ROS1, the patient was compassionately treated with crizotinib and subsequently experienced a dramatic response. Evaluation of a larger cohort of samples revealed that 8 of 11 IMTs classified as ALK-negative by immunohistochemistry harbored a kinase fusion; in addition...
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