Primary low-grade extrauterine stromal sarcoma

Endometriosis is a common benign lesion whose importance lies in the associated symptomatology and the possibility of malignant transformation. Endometrial stromal sarcoma is a rare tumor, accounting for only 0.2% of all female genital tract tumors. Primary extrauterine endometrial stromal sarcomas are even rarer, are frequently associated with endometriosis, and have been reported in the ovaries, fallopian tubes, pelvic cavity, abdominal cavity, and retroperitoneum in the absence of uterine pathology. We describe a case of a female patient who consulted for dysmenorrhea, dysuria, and dyschezia. Pelvic magnetic resonance showed a heterogeneous tumor in the left adnexa compatible with endometrioma. Histopathological findings and immunohistochemical staining were consistent with the diagnosis of primary low-grade extrauterine endometrial stromal sarcoma. The patient underwent total hysterectomy, bilateral oophorosalpingectomy, total omentectomy, and pelvic and para-aortic lymph node resection.