Gynecological Sarcomas: Molecular Characteristics, Behavior, and Histology-Driven Therapy

Int J Surg Pathol. 2021 Feb;29(1):4-20. doi: 10.1177/1066896920958120. Epub 2020 Sep 10.


Gynecological sarcomas represent 3% to 4% of all gynecological malignancies and 13% of all sarcomas. The uterus is the most frequent primary site (83%); less frequently sarcomas are diagnosed originating from the ovary (8%), vulva and vagina (5%), and other gynecologic organs (2%). As the classification of gynecologic sarcomas continues to diversify, so does the management. Accurate histopathologic diagnosis, utilizing appropriate ancillary immunohistochemical and molecular analysis, could lead to a more personalized approach. However, there are subtypes that require further definition, with regard to putative predictive markers and optimal management. The aim of this review is to highlight the importance of accurate diagnosis and classification of gynecologic sarcoma subtypes by the surgical pathologist in order to provide more tailored systemic treatment, and to highlight the increasing importance of close collaboration between the pathologist and the oncologist.

Keywords: BCOR; JAZF1-SUZ12; NTRK; SMARCA4; SMARCB1; YWHAE-NUTM2A/B; endometrial stromal sarcoma; gynecological sarcomas; histology-driven therapy; leiomyosarcoma; molecular characterization; smooth muscle neoplasms; systemic therapy; uterine sarcomas.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor / analysis*
  • Chemoradiotherapy, Adjuvant / methods*
  • Female
  • Genital Neoplasms, Female / diagnosis*
  • Genital Neoplasms, Female / mortality
  • Genital Neoplasms, Female / pathology
  • Genital Neoplasms, Female / therapy
  • Gynecologic Surgical Procedures / methods*
  • Humans
  • Immunohistochemistry
  • Molecular Diagnostic Techniques
  • Neoplasm Grading
  • Ovary / pathology
  • Progression-Free Survival
  • Sarcoma / diagnosis*
  • Sarcoma / mortality
  • Sarcoma / pathology
  • Sarcoma / therapy
  • Uterus / pathology
  • Vagina / pathology
  • Vulva / pathology


  • Biomarkers, Tumor