Introduction/Background Low grade sarcoma of endometrial stroma with no primitive uterine site is a rare diagnosis.

The diagnosis is challenging. Anatomopathological analysis could be difficult and imaging nonspecific.

We describe one case of this pathology.

Methodology/Results A 63 year old Caucasian asymptomatic female patient was referred to the gynecology department for suspicion of ovarian cancer on computed tomography (CT).

Gynecologic examination showed a mass fixed to the left pelvic wall by vaginal examination, without inguinal adenopathies or ascites.

A diagnostic laparoscopy was performed. Multiple intrapelvic adhesions and parauterine solide masses were found and biopsied. Anatomopathological analysis of the biopsies showed a leiomyoma without sign of malignity.

Secondary, a total hysterectomy and bilateral adnexectomy by laparotomy was performed. An enlarged uterus and multiple intrapelvic and intraabdominal masses were present. Peritoneal masses were found on the mesenterium of the ileum and colon, with close adhesion to the bowel. Also a large mass was found in the abdominal left upper quadrant. All the pelvic masses and most of the mesenterial masses were removed. Frozen section pathology peroperatively showed a benign leiomyoma.

Final anatomopathological analysis found a low grade sarcoma of endometrial stroma with no primitive uterine site.

There is no standard treatment protocol available for this pathology. After discussion with different Belgian experts we proposed a third operation with optimal debulking (R0) and adjuvant hormonal therapy. The patient refused the surgery and an aromatase inhibitor was started. CT scan every 3 months is planned for follow up.

Conclusion This 63-years old female was diagnosed with low grade sarcoma of endometrial stroma with an extra uterine disease only. Most masses intrapelvic and intraabdominal were removed. The patient refused a third operation for maximal debulking. Adjuvant treatment with aromatase inhibitor was started and a 3 monthly CT scan was planned.

Disclosure Nothing to disclose.