A novel LRRFIP1-ALK fusion in inflammatory myofibroblastic tumor of hip and response to crizotinib.

Abstract:

:An inflammatory myofibroblastic tumor (IMT) is a rare invasive soft tissue mass with intramuscular penetration that is primarily treated via a surgical procedure. However, with unclear boundaries and a high rate of relapse, there is no standard treatment for recurrence or unresectable tumors. It is noteworthy that approximately half of IMTs harbor genetic rearrangements of the anaplastic lymphoma kinase (ALK). ALK inhibitors have been used successfully in the treatment of IMTs with a variety of ALK fusions. Here, we present a case of a 15-year-old patient with IMT around the hip. Next-generation sequencing (NGS) revealed an LRRFIP1-ALK fusion, which has not yet been reported in the literature. Crizotinib, an ALK inhibitor, was effective in the treatment of this patient, indicating that ALK inhibitors may be effective for IMT with LRRFIP1-ALK fusions. This report expands the list of gene fusions in IMTs and highlights a new target for treatment.

journal_name

Invest New Drugs

authors

Liu W,Duan Q,Gong L,Yang Y,Huang Z,Guo H,Niu X

doi

10.1007/s10637-020-00984-5

subject

Has Abstract

pub_date

2020-09-11 00:00:00

eissn

0167-6997

issn

1573-0646

pii

10.1007/s10637-020-00984-5

pub_type

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