Endometrial stromal tumors: Diagnostic updates and challenges

Semin Diagn Pathol. 2022 May;39(3):201-212. doi: 10.1053/j.semdp.2022.01.004. Epub 2022 Jan 14.

Abstract

Endometrial stromal tumors are rare uterine mesenchymal tumors of endometrial stromal origin. They are classified into endometrial stromal nodule, low-grade endometrial stromal sarcoma, high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma by the current (2020) WHO classification. Correct diagnosis of endometrial stromal tumors is critical for proper patient management. However, due to infrequent encounters, overlapping morphological features and immunohistochemical profiles, the differential diagnoses among endometrial stromal lesions and their morphologic mimics are often challenging. Partially with our own experience, here we review and summarize the tumor morphology, immunohistochemical phenotype, as well as molecular feature of endometrial stromal tumors and key differential diagnoses, emphasizing the newest developments and their utilization in daily practice.

Keywords: Endometrial sarcoma; Endometrial stromal sarcoma; Endometrial stromal tumor; Immunophenotype.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor / genetics
  • Diagnosis, Differential
  • Endometrial Neoplasms* / diagnosis
  • Endometrial Neoplasms* / genetics
  • Endometrial Neoplasms* / pathology
  • Endometrial Stromal Tumors* / diagnosis
  • Endometrial Stromal Tumors* / pathology
  • Female
  • Humans
  • Sarcoma, Endometrial Stromal* / diagnosis
  • Sarcoma, Endometrial Stromal* / genetics
  • Sarcoma, Endometrial Stromal* / pathology

Substances

  • Biomarkers, Tumor