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Endometrial stromal sarcoma--the new genetic paradigm
Cheng-Han Lee1, Marisa R Nucci2
1Department of Laboratory Medicine and Pathology, Royal Alexandra Hospital, University of Alberta, Edmonton, AB, Canada.
Histopathology
|October 31, 2014
Summary
Endometrial stromal sarcoma (ESS) is a uterine cancer classified into low-grade and high-grade types. This review details their distinct histological, genetic, and clinical features for accurate diagnosis.
Area of Science:
- Gynaecological Pathology
- Oncology
- Molecular Diagnostics
Background:
- Endometrial stromal sarcoma (ESS) is a rare uterine malignancy.
- The 2014 WHO classification distinguishes ESS from undifferentiated uterine sarcoma (UUS).
- ESS subtypes exhibit unique histological, genetic, and clinical profiles.
Purpose of the Study:
- To review the histological and immunophenotypic features of ESS.
- To discuss diagnostic considerations for ESS subtypes.
- To highlight the genetic distinctions between low-grade and high-grade ESS.
Main Methods:
- Review of current literature on endometrial stromal sarcoma.
- Analysis of histological classifications and diagnostic criteria.
- Examination of genetic fusions (JAZF1-SUZ12, YWHAE-NUTM2A/B) in ESS.
Main Results:
- Low-grade ESS features JAZF1-SUZ12 fusions and resembles proliferative endometrium.
- High-grade ESS is defined by YWHAE-NUTM2A/B fusions and is more aggressive than low-grade ESS.
- ESS subtypes show distinct genetic drivers and clinical behaviors compared to UUS.
Conclusions:
- Accurate classification of ESS relies on integrating histological, immunophenotypic, and genetic data.
- Understanding ESS subtypes is crucial for appropriate patient management and prognosis.
- This review provides a comprehensive overview for diagnosing and understanding ESS.
