EWSR1::NR4A3 gene fusion in a cutaneous atypical myoepithelial neoplasm

J Cutan Pathol. 2023 Jul;50(7):601-605. doi: 10.1111/cup.14429. Epub 2023 Apr 14.

Abstract

Myoepithelial neoplasms of the skin and soft tissue are rare and share histopathologic features with their salivary gland counterpart. We present a case of an atypical myoepithelial neoplasm from the back of a 72-year-old female. This lesion harbored an EWSR1::NR4A3 gene fusion, a genetic signature characteristically seen in extraskeletal myxoid chondrosarcoma. To our knowledge, this is a unique case of an atypical cutaneous myoepithelial neoplasm harboring EWSR1::NR4A3 fusion.

Keywords: EWSR::NR4A3; myoepithelial carcinoma; soft tissue tumors.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Chondrosarcoma* / pathology
  • DNA-Binding Proteins / genetics
  • Female
  • Gene Fusion
  • Humans
  • Myoepithelioma*
  • Neoplasms, Connective and Soft Tissue*
  • Oncogene Proteins, Fusion / genetics
  • RNA-Binding Protein EWS / genetics
  • Receptors, Steroid*
  • Receptors, Thyroid Hormone / genetics
  • Skin Neoplasms*
  • Soft Tissue Neoplasms* / pathology

Substances

  • Oncogene Proteins, Fusion
  • RNA-Binding Protein EWS
  • NR4A3 protein, human
  • DNA-Binding Proteins
  • Receptors, Steroid
  • Receptors, Thyroid Hormone
  • EWSR1 protein, human