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High-Grade Endometrial Stromal Sarcoma
D efinition — An intermediate to high-grade stromal sarcoma with a distinct molecular genotype.
Clinical Features
Epidemiology
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Overall endometrial stromal sarcoma (ESS) is rare; it accounts for less than 0.5% of all uterine malignancies and only 10% to 15% of mesenchymal uterine malignancies.
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This particular variant has a novel gene fusion between YWHAE and FAM22A/B harboring t(10 : 17)(q22;p13).
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Majority occur in the fifth and sixth decades, but can be seen at any decade in adulthood.
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