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Peripheral Primary Neuroectodermal Tumour of the Ileum: A Case Report and Review of the Literature

Background: Ewing’s Sarcoma (ES)/peripheral Primitive 
Neuroectodermal Tumor (pPNET) is a rare aggressive malignant small round cell 
tumour. Most of them arise in bony sites, and they represent the second 
commonest primary osseous malignancy in adolescence and young adults. The 
localization of the small bowel is very rare, to our knowledge only 35 cases of 
ES/pPNET of the small bowel have been reported in the literature. Case Presentation: We report a case of pPNET of the ileum in a 26-year-old female, presented abdominal pain with a transit disorder. The 
clinical examination was unremarkable. The histological and immunohistochemical 
study confirmed the diagnosis of peripheral primary neuroectodermal tumours. 
The patient was treated by tumor resection followed by adjuvant chemotherapy. 
The evolution was good, without recurrence with a follow-up of 12 months. Conclusion: PNET of the ileum is very rare. We report this case to enrich the database of this rare clinical entity 
and to improve the management of patients with PNET of the ileum.