Intestinal inflammatory myofibroblastic tumour

  • S Ntloko
  • A Gounden
  • M Naidoo
  • TE MaDIBA
  • Y Sing
  • GP Hadley

Abstract

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.

SAJS, VOL 49, NO. 4, NOVEMBER 2011

Author Biographies

S Ntloko
Department of Surgery, University of KwaZulu-Natal, Durban
A Gounden
Department of Surgery, University of KwaZulu-Natal, Durban
M Naidoo
Department of Surgery, University of KwaZulu-Natal, Durban
TE MaDIBA
Department of Surgery, University of KwaZulu-Natal, Durban
Y Sing
Department of Anatomical Pathology, University of KwaZulu-Natal
GP Hadley
Department of Paediatric Surgery, University of KwaZulu-Natal
Section
Articles

Journal Identifiers


eISSN: 2078-5151
print ISSN: 0038-2361