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South African Journal of Surgery

On-line version ISSN 2078-5151
Print version ISSN 0038-2361

S. Afr. j. surg. vol.49 n.4 Cape Town Nov. 2011




Intestinal inflammatory myofibroblastic tumour



S. NtlokoI; A. GoundenII; M. NaidooIII; T. E. MadibaIV; Y. SingV; P. K. RamdialV; G. P. HadleyVI

IM.B. Ch.B.; Department of Surgery, University of KwaZulu-Natal, Durban
IIM.B. Ch.B., F.C.S. (s.A.); Department of Surgery, University of KwaZulu-Natal, Durban
IIIF.C.S. (s.A.); Department of Surgery, University of KwaZulu-Natal, Durban
IVM.B. Ch.B., M.Med., Ph.D., F.C.S. (s.A.), F.A.S.C.R.S.; Department of Surgery, University of KwaZulu-Natal, Durban
VF.C.Path. (anat.) S.A.; Department of Anatomical Pathology, University of KwaZulu-Natal
VIM.B. Ch.B., F.R.C.S., F.C.S. (s.A.); Department of Paediatric Surgery, University of KwaZulu-Natal




BACKGROUND: Inflammatory myofibroblastic tumours (IMFTs) are rare tumours characterised by nosologic, histogenetic and aetio-pathogenetic 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 pre-operative suspicion of I-IMFT.



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