Abstract
PurposePrimary 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.ResultsThe 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.MethodsMedical records of 14 patients with primary ovarian ESS in our institution were collected and analyzed.ConclusionsThe 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.
Highlights
Endometrial stromal sarcomas are rare mesenchymal tumors accounting for approximately 0.2% of female genital tract malignancies [1, 2]
All 9 patients with low-grade endometrioid stromal sarcomas (ESS) were alive, of these, 3 (33.3%) of them developed recurrence after surgery
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
Summary
Endometrial stromal sarcomas are rare mesenchymal tumors accounting for approximately 0.2% of female genital tract malignancies [1, 2]. These mesenchymal neoplasms occur most commonly in the uterus and occasionally originate from extrauterine sites, such as the ovary, bowel wall, peritoneum, pelvis, and vagina [2, 3]. The newly released 2014 WHO classification divides these tumors into two different subtypes based on pathologic features: low-grade ESS and high-grade ESS [20]. We present the clinicopathological features of 14 primary ovarian ESS from a single institution, including 9 low-grade ESS and 5 high-grade ESS
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