Background: Ewing's sarcoma usually presents in paediatric patients with its primary location being bone tissue. Nevertheless, we present such an adult case which arises from the small intestine. We registered thirty one cases of such origin published so far excluding ours.
Case presentation: We report a case of 30 year old female who was admitted due to the persistent anaemia. Whole body computed tomography scan revealed abdominal mass in her left upper abdominal compartment. Surgery on the mass originating from jejunum was performed, although due to extremely complicated postoperative period and rapid dissemination no additional therapy had been performed. The tumour was positive for CD99, ERG, CD56, Synaptophysin, PanCK, Cam5.2.
Conclusion: Extraosseus Ewing's sarcoma is extremely rare entity, with poor prognosis.
Keywords: ERG-FUS translocation; Ewing’s sarcoma; Extraosseus sarcoma; Primitive neuroectodermal tumor; Small bowel sarcoma.