•Endometrial stromal sarcoma (ESS) may have sex cord differentiation, usually focal.•Diagnosis of ESS is difficult when variant morphology predominates.•Prognosis differs between ESS and UTROSCT; therefore, distinction is critical.•Extensive tumor sampling is mandatory to identify neoplastic endometrial stroma.•Sex cord differentiation may be associated with endometrial hyperplasia.
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Richmond et al. (2016) studied this question.
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