Case ReportRecurrence of prolactin-producing endometrial stromal sarcoma with sex-cord stromal component treated with progestin and aromatase inhibitor
Introduction
Endometrial stromal sarcomas with sex-cord stromal elements are relatively rare tumors. Behavior of the tumor, like that of a pure endometrial stromal sarcoma is thought to depend on the cytological characteristics and stage [1]. The role of chemotherapy, radiation therapy, and hormonal therapy is poorly defined. We report a tumor of this type with hyperprolactinemia acting as a tumor marker for clinical disease behavior. We also report recurrent tumor responsive to treatment with aromatase inhibitor and progestin.
Section snippets
Case report
A 48-year-old woman with active ulcerative colitis presented with a 2-year history of intermittent vaginal bleeding. Pelvic ultrasound demonstrated an irregular fibroid uterus measuring 18 × 11 ×15 cm. The patient underwent an exploratory laparotomy, total abdominal hysterectomy and bilateral salpingo-oophorectomy. Intraoperative frozen section of the uterus showed cellular mesenchymal tumor favoring sarcoma. Pelvic and paraaortic lymph node dissection was performed. Final pathology
Discussion
A series of uterine tumors resembling ovarian sex-cord tumors (UTROSCT) was described by Clement and Scully [2]. They divided these tumors into two groups based on the relative proportion of sex-cord-like elements and endometrial stromal cells. Type I tumors (also known as ESTSCLE) appear histologically as classic endometrial stromal cell sarcomas with focal areas of sex-cord-like elements that resemble granulosa cell or Sertoli cell neoplasms. The sex-cord stromal component involves 10–40% of
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