SWI/SNF Complex-Deficient Soft Tissue Neoplasms: A Pattern-Based Approach to Diagnosis and Differential Diagnosis

Surg Pathol Clin. 2019 Mar;12(1):149-163. doi: 10.1016/j.path.2018.10.006.


Loss of different components of the Switch/sucrose nonfermentable (SWI/SNF) chromatin remodeling complex has been increasingly recognized as a central molecular event driving the initiation and/or dedifferentiation of mostly lethal but histogenetically diverse neoplasms in different body organs. This review summarizes and discusses the morphologic and phenotypic diversity of primary soft tissue neoplasms characterized by SWI/SNF complex deficiency with an emphasis on convergent and divergent cytoarchitectural patterns.

Keywords: Epithelioid sarcoma; Extraskeletal myxoid chondrosarcoma; INI1; Myoepithelial carcinoma; Rhabdoid tumor; SMARCA4; SMARCB1; SWI/SNF complex; Undifferentiated pediatric sarcoma.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor / genetics
  • Chromosomal Proteins, Non-Histone / deficiency*
  • Chromosomal Proteins, Non-Histone / genetics
  • DNA Helicases
  • Diagnosis, Differential
  • Humans
  • Immunohistochemistry
  • Phenotype
  • Prognosis
  • Soft Tissue Neoplasms / diagnosis*
  • Soft Tissue Neoplasms / genetics*
  • Soft Tissue Neoplasms / pathology
  • Transcription Factors / deficiency*
  • Transcription Factors / genetics


  • Biomarkers, Tumor
  • Chromosomal Proteins, Non-Histone
  • SWI-SNF-B chromatin-remodeling complex
  • Transcription Factors
  • DNA Helicases