Introduction: Endometrial stromal tumors with sex-cord-like elements are relatively rare. We report a case of this neoplasm with prolactin as a tumor marker for recurrent disease. We also report response of recurrent disease to progesterone and aromatase inhibitor.
Case report: A 48-year-old woman was diagnosed with Stage I endometrial stroma sarcoma with sex-cord component at the time of hysterectomy for presumed fibroid uterus. One and a half years later, she presented with recurrent disease in the abdomen associated with breast tenderness, 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 cycles of BEP were administered with complete tumor response and normalized prolactin level. Second recurrence, 9 months later, again presented with galactorrhea and rising prolactin. Disease was progressive through three cycles of Docetaxel and Gemcitabine therapy, but had an objective response to treatment with anastrozole and megestrol acetate. Prolactin level normalized. Two years later there is stable disease and the patient remains symptom-free.
Discussion: Endometrial stromal sarcoma with sex-cord stromal component may be hormonally functional. Similarly to pure endometrial stromal sarcomas, they may respond to hormonal treatment, and further study is warranted.