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

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.

Novel MXD4–NUTM1 fusion transcript identified in primary ovarian undifferentiated small round cell sarcoma

“…Ovarian sarcomas are rare mesenchymal tumors, and various histological types—such as carcinosarcoma,1 leiomyosarcoma,2 rhabdomyosarcoma,3, 4 endometrial stromal sarcoma5 and fibrosarcoma6—are pathologically defined. Small round cell sarcoma is a heterogeneous group of tumors arising from various sites, and the prognosis of this disease is poor 7, 8.…”

Extra-uterine low grade endometrioid stromal sarcoma arising from ovarian endometriosis: a case report and review of the literature

“…Extra-uterine endometrioid stromal sarcoma (EESS) is an extremely uncommon entity as the current literature offers only some case reports and short series [1, 2, 4, 10]. In 2013, Masand et al reported the largest series of EESS with 63 cases, ovarian involvement was found in 25 patients [1].…”

“…In 2013, Masand et al reported the largest series of EESS with 63 cases, ovarian involvement was found in 25 patients [1]. Ovaries are the most common site of EESS, the patients’ age ranged from 34 to 76 years with a median age around 50 years [2, 10]. Foci of endometriosis are frequently found embedded within EESS and these tumors are thought to arise from endometriosis [1, 4].…”

“…The clinical presentation of EESS is not specific, it is often related to the tumor location and size. Reported cases of patients with ovarian ESS presented with wide clinical symptoms, from asymptomatic to abdominal distension [2, 10]. Mostly, ovarian ESS was diagnosed in advanced stages with tumor extension beyond the ovaries, especially in the pelvis or abdomen [2, 4, 10].…”

“…To claim the diagnosis of a primary EESS, the uterus must be free of tumor as it constitutes the main primary site of ESS [2]. Most reported cases of ovarian ESS were of low grade type, however high grade ovarian ESS have been reported [10].…”