Pediatric Non-Myofibroblastic Primitive Spindle Cell Tumors with ALK Gene Rearrangements and Response to Crizotinib

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Abstract

We describe two poorly differentiated, non-myofibroblastic (SMA−, S100+, CD34±), spindle cell neoplasms with immunohistochemical positivity for ALK and with ALK gene rearrangements leading to PLEKHH2::ALK and CLTC::ALK fusions, respectively. ALK protein overexpression and/or gene fusions should be evaluated in poorly differentiated spindle cell neoplasms, even when there is an absence of a myofibroblastic phenotype. A positive ALK evaluation has therapeutic implications as both tumors responded to single-agent treatment with the tyrosine kinase inhibitor crizotinib.

Original languageEnglish (US)
JournalInternational Journal of Surgical Pathology
DOIs
StateAccepted/In press - 2022

Keywords

  • ALK
  • CLTC
  • PLEKHH2
  • RNA sequencing
  • crizotinib
  • gene fusion
  • pediatric
  • spindle cell tumor

ASJC Scopus subject areas

  • Surgery
  • Anatomy
  • Pathology and Forensic Medicine

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