Extraskeletal Ewing’s sarcoma/peripheral primitive neuroectodermal tumor of the small bowel presenting with gastrointestinal perforation

Abstract

Extraskeletal Ewing’s sarcoma (E-EWS)/peripheral primitive neuroectodermal tumor (pPNET) is a rare soft tissue tumor that arises from a multipotent progenitor cell and is considered to be of neuroectodermal origin. Although soft tissue E-EWS/pPNETs are common, they are exceedingly rare in the small bowel. Only 30 cases of E-EWS/pPNET of the small bowel have been reported. However, only one case of gastrointestinal perforation has been reported till today. Here, we report the second case of E- EWS/pPNET with gastrointestinal perforation.

Keywords:

• peripheral primitive neuroectodermal tumor
• immunohistochemistry
• gastrointestinal perforation
• surgery
• chemotherapy

Acknowledgments

We would like to acknowledge the help provided by Dr Bhavna Mehta, consultant histopathologist, Supratech Micropath laboratory, Ahmedabad (Gujarat, India) for providing wonderful images of histopathology and IHC.

Institutional approval

Institutional approval is neither applicable nor required for publication of this manuscript.

Disclosure

The authors report no conflicts of interest in this work.