Abstract
A 33-year-old man who became aware of dizziness and palpitations on effort visited a neighboring hospital, when the Hb level was 7.3 g/dl, revealing anemia. A type 2 tumor was demonstrated in the third part of duodenum. Pylorus-preserving pancreaticoduodenectomy was performed in our hospital with a diagnosis of duodenal cancer. The histopathology revealed a growth of small round cells. Immunostaining of the cells revealed positive for MIC2 and NSE, but negative for myogenic and epithelial markers. Furthermore we confirmed the same translocation of chromosome as Ewing sarcoma. Peripheral primitive neuroectodermal tumor (pPNET) was thus diagnosed. Adjuvant chemotherapy was conducted, and the patient has been alive without having recurrence, as of 4 years after the operation.
These pPNETs are tumors of soft tissue arising in the extremities and in the chest wall. Recently these tumors have been clarified to have the common translocation of chromosome to that of Ewing sarcoma, and thus they are collectively called Ewing sarcoma family of tumors. Since the Ewing sarcoma family of tumors extremely rarely affects the intestine, this case is presented here, together with a review of the literature.
