Inflammatory myofibroblastic tumors of the colon in pediatrics: clinical presentation, management, and outcomes—A case report and systematic review of literature

Abstract

PURPOSE: Inflammatory myofibroblastic tumors (IMTs) of the colon represent an exceptionally rare entity in the pediatric population. This systematic review aims to comprehensively analyze the clinical presentation, diagnostic workup, management strategies, and outcomes of colorectal IMTs in children. METHODS: A systematic literature review was conducted across multiple electronic databases (inception to January 2025), including MEDLINE (via PubMed), Embase, Cochrane, Web of Science, and Google Scholar. Two independent reviewers screened abstracts, reviewed studies, and extracted data on all reported cases of colorectal IMTs in the pediatric population, including one previously unreported case from our institution. RESULTS: Including our case, 53 pediatric patients with colorectal IMTs were identified from 39 studies. The mean age at diagnosis was 7 years (range: 5 months-17 years) with a slight female preponderance. The IMTs comprised a wide range of anatomic locations with rectum (27%) and ascending colon (24%) being the most common. Abdominal pain (54%), gastrointestinal bleeding (29%), and fever (21%) were the predominant symptoms. Anemia was the most common laboratory abnormality (62%). Surgical resection was the primary treatment modality in 98% of cases. After a mean follow-up of 38 months ( range: 3-181 months), the local recurrence rate was 11%, with no distant metastases reported. CONCLUSION: Colorectal IMTs in children present diagnostic and therapeutic challenges. While complete surgical resection remains the gold standard treatment, emerging therapies such as ALK inhibitors and NSAIDs warrant further investigation. The potential for late recurrence mandates long term follow-up.