TFG-MET fusion in an infantile spindle cell sarcoma with neural features

Uta Flucke, Max M van Noesel, Marc Wijnen, Lei Zhang, Chun-Liang Chen, Yun-Shao Sung, Cristina R Antonescu

Research output: Contribution to journalArticlepeer-review

51 Citations (Scopus)

Abstract

An increasing number of congenital and infantile sarcomas displaying a primitive, monomorphic spindle cell phenotype have been characterized to harbor recurrent gene fusions, including infantile fibrosarcoma and congenital spindle cell rhabdomyosarcoma. Here, we report an unusual spindle cell sarcoma presenting as a large and infiltrative pelvic soft tissue mass in a 4-month-old girl, which revealed a novel TFG-MET gene fusion by whole transcriptome RNA sequencing. The tumor resembled the morphology of an infantile fibrosarcoma with both fascicular and patternless growth, however, it expressed strong S100 protein immunoreactivity, while lacking SOX10 staining and retaining H3K27me3 expression. Although this immunoprofile suggested partial neural/neuroectodermal differentiation, overall features were unusual and did not fit into any known tumor types (cellular schwannoma, MPNST), raising the possibility of a novel pathologic entity. The TFG-MET gene fusion expands the genetic spectrum implicated in the pathogenesis of congenital spindle cell sarcomas, with yet another example of kinase oncogenic activation through chromosomal translocation. The discovery of this new fusion is significant since the resulting MET activation can potentially be inhibited by targeted therapy, as MET inhibitors are presently available in clinical trials.

Original languageEnglish
Pages (from-to)663-667
Number of pages5
JournalGenes Chromosomes and Cancer
Volume56
Issue number9
DOIs
Publication statusPublished - Sept 2017

Keywords

  • Female
  • Histones/genetics
  • Humans
  • Infant
  • Oncogene Proteins, Fusion/genetics
  • Proteins/genetics
  • Proto-Oncogene Proteins c-met/genetics
  • SOXE Transcription Factors/genetics
  • Sarcoma/diagnostic imaging
  • Soft Tissue Neoplasms/diagnostic imaging

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