Kumar Jayant, Swati Agrawal, Rajendra Kumar Agarwal, Kalyan Dayama
Extraosseous Ewing’s sarcoma/primitive neuroectodermal tumor is a rare malignant tumor with poor outcome. It is mostly reported in the second decade of life with equal in males and females. It is an aggressive tumor with unavoidable multiple recurrences and relatively poor prognosis. These tumors can be easily misdiagnosed for different tumors due to lack of established diagnostic pathological criteria. Hence, in the cases of pancreatic tumors, it is necessary to highlight the importance of considering Ewing’s sarcoma/primitive neuroectodermal tumor in the differential diagnosis. For the better management, and improved prognosis and survival of these patients, it is necessary to understand the biological characteristics of this tumor in detail. We here present a review of 24 cases of extraosseous Ewing’s sarcoma. Chemotherapy combined with surgery showed relatively better outcome with 5 year survival rate of 80%.