Primary cutaneous biphasic sarcomatoid basal cell carcinoma with myoepithelial carcinoma differentiation: A new variant

J Cutan Pathol. 2019 Dec;46(12):949-953. doi: 10.1111/cup.13543. Epub 2019 Aug 1.

Abstract

Isolated cases of basal cell carcinoma (BCC) with partial myoepithelial component have been described. However, myoepithelial differentiation has not been described in sarcomatoid basal cell carcinomas, which usually show features resembling osteosarcoma, chondrosarcoma, or leiomyosarcoma. We report a case of an 87-year-old man with a forehead lesion that histologically showed a minor component of conventional nodular BCC in transition with a major biphasic sarcomatoid growth composed of invasive spindle-cell and epithelial-like components, the latter with a reticular pattern and scattered ductal structures. Both components showed cytological atypia and high mitotic rate (26/10HPF), with atypical mitotic figures. BER-EP4 immunostaining was exclusively found in the nodular BCC component whereas the sarcomatoid component revealed immunostaining for α-smooth muscle actin (SMA), muscle-specific actin (MSA), calponin, and p63 in both epithelial-like and spindle-cell populations. Focal immunoreactivity was observed in the epithelial component for S100 and glial fibrillary acidic protein (GFAP). Furthermore, EWSR1-PBX1 gene fusion was also detected. This is to our knowledge, the first fully documented case of biphasic sarcomatoid BCC with myoepithelial carcinoma differentiation.

Keywords: EWSR1 rearrangement; EWSR1-PBX1 gene fusion; myoepithelial carcinoma; sarcomatoid basal cell carcinoma.

Publication types

  • Case Reports

MeSH terms

  • Aged, 80 and over
  • Biomarkers, Tumor / genetics
  • Biomarkers, Tumor / metabolism
  • Carcinoma, Basal Cell / genetics
  • Carcinoma, Basal Cell / pathology*
  • Carcinoma, Basal Cell / ultrastructure
  • Cell Differentiation
  • Curettage / methods
  • Forehead / pathology
  • Gene Fusion / genetics
  • Humans
  • Male
  • Myoepithelioma / complications
  • Myoepithelioma / genetics
  • Myoepithelioma / pathology*
  • Myoepithelioma / ultrastructure
  • Pre-B-Cell Leukemia Transcription Factor 1 / genetics
  • RNA-Binding Protein EWS / genetics
  • Sarcoma / genetics
  • Sarcoma / pathology*
  • Sarcoma / ultrastructure
  • Skin Neoplasms / genetics
  • Skin Neoplasms / pathology
  • Skin Neoplasms / ultrastructure

Substances

  • Biomarkers, Tumor
  • EWSR1 protein, human
  • Pre-B-Cell Leukemia Transcription Factor 1
  • RNA-Binding Protein EWS
  • human epithelial antigen-125
  • PBX1 protein, human