n South African Journal of Surgery - Intestinal inflammatory myofibroblastic tumour : general surgery
|Article Title||Intestinal inflammatory myofibroblastic tumour : general surgery|
|© Publisher:||Medpharm Publications|
|Journal||South African Journal of Surgery|
|Affiliations||1 University of KwaZulu-Natal, 2 University of KwaZulu-Natal, 3 University of KwaZulu-Natal, 4 University of KwaZulu-Natal, 5 University of KwaZulu-Natal, 6 University of KwaZulu-Natal and 7 University of KwaZulu-Natal|
|Publication Date||Nov 2011|
|Pages||190 - 193|
Background. Inflammatory myofibroblastic tumours (IMFTs) are rare tumours characterised by nosologic, histogenetic and aetiopathogenetic controversy and variable clinicopathological features. We report our experience with intestinal-IMFTs (I-IMFTs) that have been reported mainly as single case reports to date.
Methods. Five patients with I-IMFTs, identified between 2005 and 2008, formed the study cohort. The clinicopathological features were obtained from departmental and hospital records.
Results. The median patient age was 13 years. While 4 patients presented with symptoms and signs of intestinal obstruction, one IMFT was an incidental finding at laparotomy for trauma. Three I-IMFTs were located in the small bowel and 2 in the colon. Complete resection with end-to-end anastomoses was performed. The gross morphology included 1 polypoid myxoid tumour that served as a lead point for an intussusception, 3 multinodular whorled masses and 1 firm circumferential, infiltrative tumour.Microscopically, all tumours had typical features of IMFT with variable expression of ALK-1, a low proliferation index and tumour-free resection margins. All patients had an uneventful recovery. One patient was lost to further follow-up. Four patients were well, without local recurrence or metastases at 6 months to 3 years.
Conclusions. Surgery with tumour-free resection margins is the gold standard of care of adult and paediatric I-IMFTs. Heightened recognition of I-IMFT, albeit rare, as a cause of intestinal obstruction, including intussusception, is necessary for preoperative suspicion of I-IMFT.
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