Endometrial stromal sarcoma presenting as large bleeding left upper quadrant mass

Endometrial stromal sarcoma (ESS) represents only 0.2% of all uterine malignancies. Even fewer cases have been reported on patients presenting with symptomatic disease. We report the case of young lady presenting with a large bleeding abdominal mass. At the time of surgery, an en bloc excision of the mass with a partial pancreatectomy, splenectomy, transverse colectomy, left nephrectomy, left adrenalectomy and resection of the diaphragm was performed. The final pathology revealed a malignant spindle and epithelial cell neoplasm with features favoring a variant of ESS. ESS can present in an atypical fashion as an intra-abdominal mass; a multi-disciplinary treatment approach for patients with this disease is crucial.