Endometrial Stromal Sarcoma
Khin Thway, BSc, MBBS, FRCPath
Key Facts
Terminology
Malignant tumor composed of cells that resemble those of proliferative endometrium
Clinical Issues
Peak incidence in 4th-5th decades
Can present as intraabdominal soft tissue tumor
Extends from uterus or arises in endometriosis
Mass or abdominal distension
Wide differential diagnosis
Microscopic Pathology
Sheets of uniform ovoid cells
Minimal nuclear atypia
Minimal cytoplasm
Plexiform vascular pattern
Associated endometriosis in some cases
Focal smooth muscle differentiation in some
Diffuse CD10, ER, and PR expression
> 60% have t(7;17)(p15;q21) translocation
 At low power, endometrial stromal sarcoma is a multilobulated, highly cellular tumor composed of islands of ovoid cells with scant cytoplasm, some in vessels  . |
 The constituent cells are present in solid sheets. They show minimal atypia. Tumor cells are arranged around a prominent capillary network  . |
TERMINOLOGY
Abbreviations
Endometrial stromal sarcoma (ESS)
Synonyms
Low-grade endometrial stromal sarcoma
Endolymphatic stromal myosis
Definitions
Malignant mesenchymal tumor composed of cells that resemble those of proliferative endometrium
Previous division of ESS into low-grade and high-grade categories has fallen out of favor
Term ESS is applied to tumors formerly referred to as “low-grade stromal sarcoma”
High-grade/undifferentiated stromal sarcoma is now thought to be unrelated to low-grade ESS
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