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

Soukaina Harrak,Saad Lannaz,Siham Lemsanes,Salma Najem,Hind Mrabti,Khadija Benchekroun,Sawsan Razine,Hassan Errihani

doi:10.4236/jct.2021.127035

Abstract

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.

Highlights

Peripheral primitive neuroectodermal tumors are small round cell neoplasms with neuroectodermal differentiation that develop outside the central and sympathetic nervous systems
Case Presentation: We report a case of pPNET of the ileum in a 26-year-old female, presented abdominal pain with a transit disorder
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

Summary

Introduction

Peripheral primitive neuroectodermal tumors are small round cell neoplasms with neuroectodermal differentiation that develop outside the central and sympathetic nervous systems. ES/PNET are primarily seen in bone and soft tissue in children and young adults, primitive visceral sites have been reported such as the kidney, uterus, ovary, gallbladder, parotid gland, pancreas, lung, adrenal gland, esophagus and testicle [2] [3] [4] [5] [6]. It has been reported previously in this location [7] [8], ES/PNET is extremely rare in the small bowel. The present study reports a case of pPNET in the ileum in a 26-year-old woman, and describes the presenting symptoms, imaging findings, anatomopathological features of these tumors

Case Report

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Discussion

Conclusions