Introduction.Endometrial stromal tumors with sex-cord-like elements are relatively rare.We rep ort a case of this neo-plasm with prolactin as a tumor marker for recurrent dis-ease.We also report response of recu rrent disease to pro-gesterone and aromatase inhibitor.Case report.A48-year-old woman was diagnosed with St age I endometrial stroma sarcoma with sex-cord compon ent at the time of hysterectomy for presumed fibroid u terus.One and a half years later,she presented with recu rrent disease in the abdomen associated with breast tend erness,galactorrhea,and an elevated prolactin level.She received three cycles of BEP(Bleomycin,Etoposide,Cisplatin)with partial response and followed by an optimal debulking procedure.Two out of a six additional planned cy cles of BEP wereadministered with complete tumor re sponse and normalized prolactin level.Second recurrence,9months later,again presented with galactorrhea and ris ing prolactin.Disease was progressive through three cycle s of Docetaxel and Gemcitabine therapy,but had an obje ctive response to treatment with anastrozole and megestrol acetate.Prolactin level normalized.Two years later th ere is stable disease and the patient remains symptom-fre e.Discussion.En-dometrial stromal sarcoma with sex-cord stromal component may be hormonally functional.Simil arly to pure endome-trial stromal sarcomas,they may respond to hormonal treatment,and further study is warranted. Introduction. Endometrial stromal tumors with sex-cord-like elements are relatively rare. We rep ort a case of this neo-plasm with prolactin as a tumor marker for recurrent dis-ease. We also report response of recu rrent disease to pro-gesterone and aromatase inhibitor. Case report. A48-year-old woman was diagnosed with Stage I endometrial stroma sarcoma with sex-cord compon ent at the time of hysterectomy for presumed fibroid u terus. One and a half years later, she presented with recu rrent disease in the abdomen associated with breast tend erness, galactorrhea, and an elevated prolactin level. Received three cycles of BEP (Bleomycin, Etoposide, Cisplatin) with partial response and subsequently by an optimal debulking procedure. Two out of a six additional planned cy cles of BEP were administered with complete tumor re sponse and normalized prolactin level. Second recurrence, 9months later, again presented with galactorrhea and ris ing prolactin. Disease was progressive through three cycles s of Docetax el and Gemcitabine therapy, but had an obje ctive response to treatment with anastrozole and megestrol acetate. Prolactin level normalized. Two years later th ere is stable disease and the patient remained symptom-fre e. Discussion. En-dometrial stromal sarcoma with sex- cord stromal component may be hormonally functional. Simil arly to pure endome-trial stromal sarcomas, they may respond to hormonal treatment, and further study is warranted.