Related Experiment Video
Updated: Mar 8, 2026
Author Spotlight: Genetically Engineered Mouse Models and Pathological Characterization of Neurofibromatosis Type 1 Associated Tumors
Published on: May 17, 2024
A distal ileum malignant peripheral nerve sheath tumor causing intussusception in a patient in China: a case report
Lin-Bo Zhu1, Peng-Fei Li2, Wei-Hua Xiao3
1Department of General Surgery, Beilun District People's Hospital, The First Affiliated Hospital, College of Medicine, Beilun Branch of Zhejiang University, No.1288, Lushan East Road, Ningbo, Zhejiang, 315800, China.
Background:
Malignant peripheral nerve sheath tumors (MPNSTs) arise from a peripheral nerve or display nerve sheath differentiation. Most MPNSTs typically originate on the trunk, extremities, head, neck, and paravertebral regions. Gastrointestinal MPNSTs are rare entities with only 10 cases reported worldwide in the literatures.
Case Presentation:
Here, we report the first Chinese case of a malignant peripheral nerve sheath tumor of the distal ileum presenting as intussusception. A 53-year-old female patient without pathological antecedent for neurofibromatosis was admitted with pain in the right lower abdomen and multiple episodes of vomiting for 1 week. Preoperative diagnosis was intussusception with a contrast-enhanced computed tomography scan (CECT) of the abdomen showing characteristic target sign. Due to difficulty reducing the ileum-colon intussusception, right hemicolectomy with ileocolostomy was performed. Histopathology was suggestive of low-grade MPNST. The patient received postoperative care and was followed up for 9 months. There is no sign of tumor recurrence and metastatic disease.
Conclusions:
This case is unique in terms of a rare tumor presenting with unusual complication.
