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

Abstract

BackgroundEndometrial stromal sarcoma (ESS) is a rare neoplasm accounting for only 0.2% of female genital tract tumors. The primary extra-uterine location of ESS is an extremely uncommon occurrence.Case presentationWe present a case of a 64-year-old woman presenting with abdominopelvic and bilateral ovarian tumors with misleading clinical presentation and diagnostic challenge. The histopathological examination of the resected specimens disclosed the diagnosis of primary extra-uterine ESS arising from ovarian endometriosis. Adjuvant therapy with an aromatase inhibitor drug was prescribed for the patient, and she is still alive with no evidence of disease 7 months after surgery.ConclusionThe awareness of the potential extra-uterine location of ESS should lead to correct diagnosis as this tumor has histopathological features and clinical behavior similar to its uterine counterpart.

Prognosis of uterine and extrauterine low-grade endometrial stromal sarcoma: an observational cohort study

“…In our study, extrauterine disease made up 5.6% of cases in the primary LGESS cohort. Many case reports have discussed LGESS originating from the vagina 8 , 13 , 14 , cervix 15 , 16 , ovaries 5 , 7 , 17 , placenta 18 , stomach 19 , ileum 20 , colorectum 21 – 23 , omentum 24 , sciatic nerves 25 , retroperitoneal cavity 26 , and nonspecific sites within the pelvic cavity 27 . The most common sites involved are the ovaries 5 .…”

Primary ovarian high-grade endometrial stromal sarcoma: a case report

“…Primary ovarian endometrial stromal sarcoma is an extremely uncommon entity with only about 100 cases reported in the literature so far. The sites of extrauterine ESS include the ovaries, Fallopian tubes, broad ligament, vagina, bladder, small bowel, colon, pelvic peritoneum, mesentery, and liver, and ovary is the most common primary site of extrauterine ESS [ 4 , 8 ]. Since the majority of ESS originates from uterine corpus, to prove primary ovarian ESS, a thorough pathologic evaluation of uterus is essential to exclude the possibility of metastasis, which was confirmed in our case [ 6 ].…”

“…The mesenchymal neoplasm occurs mainly in the uterine corpus, but it also originates from extrauterine sites such as ovary, vagina, Fallopian tube, bladder, small bowel, colon, peritoneum, etc. [ 4 , 5 ]. Although uterine endometrial stromal sarcoma is a relatively well-known disease, data regarding the management of primary ovarian ESS are scarce and limited due to its rarity.…”

A Rare Case of Intestinal Low-Grade Endometrial Stromal Sarcoma With Glandular Differentiation and Associated Endometriosis

“…ESS is a rare tumour with about 80 cases reported so far [10,11]. A case series from M D Anderson Cancer Centre, Texas, reports the largest number of such cases.…”