Pleomorphic Sarcomas: The State of the Art

Surg Pathol Clin. 2019 Mar;12(1):63-105. doi: 10.1016/j.path.2018.10.004. Epub 2018 Dec 17.

Abstract

This article focuses on pleomorphic sarcomas, which are malignant mesenchymal tumors with complex genetic background at the root of their morphologic pleomorphism. They are poorly differentiated tumors that may retain different lines of differentiation, sometimes correlating with clinicopathological or prognostic features. Accurate diagnosis in this group of tumors relies on adequate sampling due to their heterogeneity and assessment with both microscopy and large panels of immunohistochemistry. Molecular analyses have a limited role in their diagnosis as opposed to translocation-related sarcomas but may provide theranostic and important prognostic information in the future.

Keywords: Dedifferentiation; Genomic instability; Leiomyosarcoma; Liposarcoma; Myxofibrosarcoma; Rhabdomyosarcoma; Sarcoma; Undifferentiated pleomorphic sarcoma.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Biomarkers, Tumor / genetics
  • Diagnosis, Differential
  • Humans
  • Immunohistochemistry
  • Prognosis
  • Sarcoma / classification
  • Sarcoma / genetics
  • Sarcoma / pathology*
  • Soft Tissue Neoplasms / classification
  • Soft Tissue Neoplasms / genetics
  • Soft Tissue Neoplasms / pathology*

Substances

  • Biomarkers, Tumor