278 Ewing's Sarcoma of the Duodenum: A Rare Case Presentation and Review of Literature

Abstract

Abstract Introduction Ewing's sarcoma (ES) is a rare tumour with a tendency to metastasise early and recur. It commonly affects paediatric patients. The most common subdivision comprising 70% of all ES is Ewing's sarcoma of the bone. A review of the literature identified only 5 cases worldwide of primary ES arising from the duodenum. Case presentation We present a case of a 42-year-old man with no prior medical history who presented with right-sided abdominal pain. A computed tomography scan of the abdomen demonstrated a 7.7cm heterogeneous mass arising from the second part of the duodenum. Oesophagogastroduodenoscopy confirmed a malignant appearing duodenal lesion with biopsy showing features consistent with small cell carcinoma. The patient underwent three cycles of neoadjuvant chemotherapy followed by elective Kausch-Whipple pancreaticoduodenectomy. A combination of immunohistochemistry and molecular studies confirmed the diagnosis of a rare Ewing sarcoma tumour originating from the duodenum with invasion into the duodenal lumen. The patient recovered well from surgery and remains disease-free 18 months following resection. Discussion Literature search revealed this to be an extremely rare case. The optimal treatment strategy for patients with intestinal ES is unclear, with the results of the literature search demonstrating poor outcomes following surgical resection. The long-term prognosis of intestinal ES is unknown due to its rarity. Determinants of poor outcome include tumour size >5cm, tumours involving the pelvic site, old age, non-response to chemotherapy and presence of metastases. In the case we report, the combination of neoadjuvant chemotherapy and surgery led to a good short-term post-operative outcome.