Elsevier

Gynecologic Oncology

Volume 94, Issue 2, August 2004, Pages 567-571
Gynecologic Oncology

Case Report
Recurrence of prolactin-producing endometrial stromal sarcoma with sex-cord stromal component treated with progestin and aromatase inhibitor

https://doi.org/10.1016/j.ygyno.2004.03.025Get rights and content

Abstract

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.

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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