The treatment of recurrent or metastatic low-grade endometrial stromal sarcoma (LG-ESS) is still controversial. Recurrent disease mainly develops in the lung or in the pelvis. When the evidence of extrauterine tumor extension exists, debulking is recommended. Responses to hormonal therapy have been reported, because of the presence of estrogen and progestin receptors. Also chemotherapy has been used, but the percentage of response is low. Three patients with lung and pelvic localization of LG-ESS are reported. The first patient showed lung relapse 22 months after pelvic surgery. The second patient developed pelvic and abdominal recurrences, managed by surgery, 33 months after primary treatment and a subsequent lung recurrence 11 years later. The third patient had lung metastases at the primary diagnosis. All these patients underwent hysterectomy, bilateral salpingo-oophorectomy, and exeresis of lung recurrences. Our 3 patients were all treated with medroxyprogesterone acetate for long periods. They all presented regression or stabilization of metastatic lesions. At present, they are alive and without any evidence of disease (39, 70, and 28 months). In LG-ESS, the combined treatment of surgery and progestin therapy is effective in achieving both local and distant disease control. Metastatic lesions, especially pulmonary lesions, seem to benefit from surgical removal, followed by progestin therapy. Hormonal therapy should be maintained for an indefinite period. On account of the long period existing between primary tumor and recurrent disease, a long-term follow-up is always recommended after the primary treatment.
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