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

作     者：Soukaina Harrak Siham Lemsanes Sawsan Razine Salma Najem Khadija Benchekroun Saad Lannaz Hind Mrabti Hassan Errihani Soukaina Harrak;Siham Lemsanes;Sawsan Razine;Salma Najem;Khadija Benchekroun;Saad Lannaz;Hind Mrabti;Hassan Errihani

作者机构：Department of Medical Oncology National Institute of Oncology Rabat Morocco 

出 版 物：《Journal of Cancer Therapy》 (癌症治疗（英文）)

年 卷 期：2021年第12卷第7期

页      面：399-408页

学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学] 

主　　题：Ewing’s Sarcoma Primitive Neuroectodermal Tumor Small Bowel Sarcoma Extraosseous Sarcoma 

摘      要：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.