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A case of a primarily extra-uterine low-grade endometrial stromal sarcoma is described. The patients clinical course, including various surgical, chemotherapeutical and hormonal therapies, is outlined and discussed. Transition from benign endometriosis to clinically malignant endometrial stromal sarcoma is illustrated by microscopy and DNA flow cytometry. A change in nuclear DNA content is demonstrated. This is possibly a useful aid in identifying the malignant potential of this tumor with such a misleading microscopic appearance.
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