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.

Epithelioid sarcoma (ES) is a rare high-grade sarcoma subtype that constitutes less than 1% of soft tissue sarcomas (STS).There are two types: distal-type epithelioid sarcoma and proximal-type epithelioid sarcoma, based on anatomic location and the histopathological features. The clinical presentation of ES is varied and can lead to a delay in diagnosis. histopathology examination followed by immunohistochemistry will help to establish the diagnosis. The treatment of choice of Localized ES is a radical excision with microscopically radical margins and perioperative radiotherapy. systemic therapies are used in cases of locally advanced or metastatic ES. We describe a case of reoccurring proximal-type epithelioid sarcoma of the ischioanal fossa. A 56-year-old man operated two years ago for a epithelioid sarcoma of the ischioanal fossa. The patient presented with reoccurring mass at the same location, Magnetic resonance imaging (MRI) of the pelvis showed a mass of the ischioanal fossa. computed tomography (CT) of the chest, abdomen, and pelvis showed multiple pulmonary and liver metastasis. Histopathological features and immunohistochemistry were those of proximal type epithelioid sarcoma. He received intravenous doxorubicin with a partial response after 3 cycles of treatment.