Myoepithelial Tumors of Bone

Surg Pathol Clin. 2017 Sep;10(3):657-674. doi: 10.1016/j.path.2017.04.010. Epub 2017 Jun 29.

Abstract

Myoepithelial tumors (METs) of bone (BMETs) are a rare but distinct tumor entity. METs that are cytologically benign are termed myoepitheliomas; METs with malignant histologic features are called myoepithelial carcinomas. BMETs have a wide age range, may involve any part of the skeleton, and have a variable spindle cell and epithelioid morphology. Bone tumors to be considered in the differential diagnosis are discussed. Additional techniques are indispensable to correctly diagnose BMETs. By immunohistochemistry, BMETs often express cytokeratins and/or EMA together with S100, GFAP, or calponin. Half of BMETs harbor EWSR1 (or rare FUS) gene rearrangements with different gene partners.

Keywords: Bone; EWSR1; FUS; Fusion gene; Immunohistochemistry; Myoepithelial carcinoma; Myoepithelioma; Pathology.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor / metabolism
  • Bone Neoplasms / genetics
  • Bone Neoplasms / metabolism
  • Bone Neoplasms / pathology*
  • Calcium-Binding Proteins / metabolism
  • Calponins
  • Diagnosis, Differential
  • Gene Rearrangement
  • Glial Fibrillary Acidic Protein / metabolism
  • Humans
  • Immunohistochemistry
  • Keratins / metabolism
  • Microfilament Proteins / metabolism
  • Myoepithelioma / genetics
  • Myoepithelioma / metabolism
  • Myoepithelioma / pathology*
  • RNA-Binding Protein EWS / genetics
  • rab GTP-Binding Proteins / metabolism

Substances

  • Biomarkers, Tumor
  • Calcium-Binding Proteins
  • EWSR1 protein, human
  • Glial Fibrillary Acidic Protein
  • Microfilament Proteins
  • RAB33A protein, human
  • RNA-Binding Protein EWS
  • Keratins
  • rab GTP-Binding Proteins