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.
Highlights
Endometrial stromal sarcoma (ESS) is a rare neoplasm accounting for only 0.2% of female genital tract tumors
To claim the diagnosis of a primary endometrioid stromal sarcoma (EESS), the uterus must be free of tumor as it constitutes the main primary site of ESS [2]
We report a case of a 64-year-old wowan presenting with abdominopelvic and bilateral ovarian tumors diagnosed histologically as low grade ESS arising from ovarian endometriosis
Summary
Endometrial stromal sarcoma (ESS) is a rare distinct pathologic entity accounting for only 0.2% of female genital tract tumors [1]. We report a case of a 64-year-old wowan presenting with abdominopelvic and bilateral ovarian tumors diagnosed histologically as low grade ESS arising from ovarian endometriosis. Five months later (November 2017), MRI was performed and revealed 2 latero-uterine (ovarian) solido-cystic tumors measuring 60 × 53 mm (left) and 47 × 40 mm (right), along with 2 pelvic masses (located in the recto-vaginal fascia and in the vicinity of the uterine cervix). The histological examination of the other specimens were identical to the right adnexal tumor, endometrioid glands were not noticed These histomorphologic characteristics were reminiscent of the proliferative endometrial stroma and the diagnosis of a low grade EESS arising from right ovarian endometriosis was suggested. Adjuvant therapy with an aromatase inhibitor drug (letrozole) was performed, and the patient is still alive with no evidence of disease 7 months after surgery
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