Primary endometrioid stromal sarcomas of the ovary: a clinicopathological study of 14 cases with a review of the literature

// Weimin Xie 1 , Xiaoning Bi 1 , Dongyan Cao 1 , Jiaxin Yang 1 , Keng Shen 1 and Yan You 2 1 Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China 2 Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China Correspondence to: Jiaxin Yang, email: yangjiaxin4022@163.com Keywords: ovarian endometrioid stromal sarcoma, low-grade, high-grade, recurrence, prognosis Received: November 16, 2016      Accepted: June 02, 2017      Published: June 28, 2017 ABSTRACT Purpose: Primary endometrioid stromal sarcomas (ESS) of the ovary are rare mesenchymal tumors with scarce data on their behavior and optimal treatment. We aimed to describe the clinicopathologic features and outcome among patients with primary ovarian ESS. Results: The age of the patients ranged from 34 to 61 years (mean: 49.1 years, median: 51.5 years). The most common symptoms were abdominal distention or pain or both. Nine (64.3%) and five patients (35.7%) had low-grade and high-grade disease, respectively. The median duration of follow-up was 65 months (range, 8–311 months). All 9 patients with low-grade ESS were alive, of these, 3 (33.3%) of them developed recurrence after surgery. Only 1 patient (20%) with high-grade ESS was alive with no evidence of disease in a short-term follow-up visit; the remaining 4 (80%) developed recurrence after surgery, and 2 (40%) died of progressive disease. Methods: Medical records of 14 patients with primary ovarian ESS in our institution were collected and analyzed. Conclusions: The behavior of primary ovarian ESS is similar to that of their uterine counterparts. Low-grade ESS is an indolent tumor with a propensity for late recurrences. The prognosis of high-grade ESS is poor.