FN1: a novel fusion partner of ALK in an inflammatory myofibroblastic tumor

Pediatr Blood Cancer. 2015 May;62(5):909-11. doi: 10.1002/pbc.25424. Epub 2015 Feb 14.

Abstract

Inflammatory myofibroblastic tumors (IMTs) are rare tumors characterized as low-to-intermediate grade sarcomas. Rearrangements of the anaplastic lymphoma kinase (ALK) gene have been reported in IMT. Here, we describe a novel fusion gene in an IMT tumor specimen. A 12-year-old male was admitted to our hospital with a bladder tumor. We identified the fibronectin 1 gene (FN1) as a fusion partner of ALK using 5'RACE. This novel fusion, FN1-ALK, resulted in ALK overexpression in the IMT. This finding should clarify the causes of IMT and facilitate development of novel therapeutics.

Keywords: ALK gene fusion; FN1; inflammatory myofibroblastic tumor.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anaplastic Lymphoma Kinase
  • Child
  • Fibronectins / genetics
  • Fibronectins / metabolism*
  • Granuloma, Plasma Cell / genetics
  • Granuloma, Plasma Cell / metabolism*
  • Granuloma, Plasma Cell / pathology
  • Humans
  • Immunoenzyme Techniques
  • Inflammation / genetics
  • Inflammation / metabolism*
  • Inflammation / pathology
  • Male
  • Myofibroblasts / pathology*
  • Neoplasms, Muscle Tissue / genetics
  • Neoplasms, Muscle Tissue / metabolism*
  • Neoplasms, Muscle Tissue / pathology
  • Oncogene Proteins, Fusion
  • Prognosis
  • RNA, Messenger / genetics
  • Real-Time Polymerase Chain Reaction
  • Receptor Protein-Tyrosine Kinases / genetics
  • Receptor Protein-Tyrosine Kinases / metabolism*
  • Reverse Transcriptase Polymerase Chain Reaction

Substances

  • FN1 protein, human
  • Fibronectins
  • Oncogene Proteins, Fusion
  • RNA, Messenger
  • ALK protein, human
  • Anaplastic Lymphoma Kinase
  • Receptor Protein-Tyrosine Kinases