Extrauterine low‐grade endometrial stromal sarcoma: Report of three cases

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Three cases of extrauterine low-grade endometrial stromal sarcoma, found in the ovary, pelvic, and abdominal cavities, presented with specific histological and immunohistochemical features and showed no recurrence after surgery.

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Abstract

Three cases of rare low-grade endometrial stromal sarcoma of the extrauterine tissue are presented. Each one occurred in the ovary, pelvic and abdominal cavities. Two were associated with endometriosis. Histologically, the tumors were characterized by an infiltrative and diffuse proliferation of uniform round or oval cells, abundant small vessels, low mitotic activity, the presence of foam cells and vascular invasion. Immunohistochemically, all tumors expressed vimentin, muscle markers (desmin, muscle-specific actin and alpha-smooth muscle actin) and progesterone receptors. Two tumors were diploid and one was aneuploid by flow cytometry. All patients were well with no evidence of disease 16-39 months after surgery. It is suggested that this neoplasm may arise with or without endometriosis under hormonal influence. This rare variant of Müllerian tumors should not be confused with adenosarcoma and soft tissue tumors, such as smooth muscle tumors and solitary fibrous tumor.

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Condition tags

endometriosis

MeSH descriptors

Endometrial Neoplasms Sarcoma, Endometrial Stromal Abdominal Neoplasms Abdominal Neoplasms Abdominal Neoplasms Abdominal Neoplasms Actins Actins Adult Biomarkers, Tumor Biomarkers, Tumor Diploidy DNA, Neoplasm DNA, Neoplasm Endometrial Neoplasms Endometrial Neoplasms Endometrial Neoplasms Female Flow Cytometry Humans

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