Inflammatory myofibroblastic tumour causing intestinal obstruction in a patient with neurofibromatosis type 1

Inflammatory myofibroblastic tumours (IMTs) are rare tumours with unpredictable biological behaviour ranging from benign to locally invasive and rarely, distant metastasis. While neurofibromatosis type 1(NF1) may manifest with gastrointestinal soft tissue tumours, this is the first report in the literature that describes an IMT occurring in a NF1 patient who presented with intestinal obstruction. Our patient presented with intestinal obstruction secondary to an obstructing terminal ileum mesenteric tumour En bloc bowel resection was performed, with histology revealing an IMT and an adjacent neurofibroma. The resection margins were clear and the patient was free of recurrence at six months.

Automated summary

IMTs are rare tumors with unpredictable behavior. This case report describes an IMT in a NF1 patient presenting with intestinal obstruction, successfully treated with surgery.