Primary extrauterine endometrial stromal sarcoma: Located in pelvic and abdominal tissue and arising in endometriosis.

Primary extrauterine endometrial stromal sarcoma is a rare tumor and it is infrequently associated with endometriosis. We are reporting a case of this unusual tumor in a 42-year-old female who presented with multiple nodules of tumor in the abdomen and pelvis and with metastases in para-aortic lymph nodes. The right parametrium, in addition, had a focus of endometriosis, which was contiguous with the tumor, confirming its origin.