Three cases of rare low-grade endometrial stromal sarcoma of the extra
uterine tissue are presented. Each one occurred in the ovary, pelvic a
nd abdominal cavities. Two were associated with endometriosis. Histolo
gically, 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 pro
gesterone 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 vari
ant of Mullerian tumors should not be confused with adenosarcoma and s
oft tissue tumors, such as smooth muscle tumors and solitary fibrous t
umor.