GENERAL SURGERY

 

Intestinal inflammatory myofibroblastic tumour

 

 

S. Ntloko^I; A. Gounden^II; M. Naidoo^III; T. E. Madiba^IV; Y. Sing^V; P. K. Ramdial^V; G. P. Hadley^VI

^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

 

 

---

ABSTRACT

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.

---

 

 

“Full text available only in PDF format”

 

REFERENCES

1. Karnak I, Senocak ME, Ciftci AO, et al. Inflammatory myofibroblasts tumour in children: Diagnosis and treatment. J Pediatr Surg 2001;36:908-912.         [ Links ]

2. Coffin CM, Dehner Lp, Meis-Kindblom JM, et al. Inflammatory myofibroblastic tumour, inflammatory fibrosarcoma and related lesions: A historical review with differential diagnostic considerations. Sem Diag Pathol 1998;15:102-110.         [ Links ]

3. Coffin CM, Fletcher JA. Inflammatory myofibroblastic tumour. In: Fletcher CDM, Unni KK, Mertens F, eds. Pathology and Genetics of Soft Tissue and Bone. World Health Organization Classification of Tumours. Lyon: IARC Press;2002:91-93.         [ Links ]

4. Coffin CM, Hornick JL, Fletcher CD. Inflammatory myofibroblastic tumour: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. Am J Surg Pathol 2007;31:509-520.         [ Links ]

5. DiFiore JW, Goldblum JR. Inflammatory myofibroblastic tumour of the small intestine. J Am Coll Surg 2002;194:502-506.         [ Links ]

6. Makhlouf HR, Sobin LH. Inflammatory Myofibroblastic tumours (inflammatory pseudotumour) of the gastrointestinal tract: How closely are they related to inflammatory fibroid polyps. Hum Pathol 2002;33:307-315.         [ Links ]

7. Milne ANA, Sweeney KJ, O'Riodain DS, et al. Inflammatory myofibroblastic tumour with ALK/TPM3 fusion presenting as ileocolic intussusception: An usual presentation of an unusual neoplasm. Hum Pathol 2006;37:112-116.         [ Links ]

8. Park S-H, Kim J-H, Min BW, et al. Exophytic inflammatory myofibroblastic tumour of the stomach in an adult woman: a rare cause of hemoperitoneum. World J Gastroenterol 2008;14:136-139.         [ Links ]

9. Pratap A, Tiwari A, Agarwal B, et al. Inflammatory myofibroblastic tumour of the abdominal wall simulating rhabdomyosarcoma: report of a case. Surg Today 2007;37:352-355.         [ Links ]

10. Bajaj P, Harris L, Vermillion JM, et al. An uncommon presentation of inflammatory myofibroblastic tumour. Hosp Physician 2007;43:27-30.         [ Links ]

11. Demirkan NC, Akalin T, Yilmaz F, et al. Inflammatory myofibroblastic tumour of the small bowel in childhood: Report of a case and a review of the literature. Pathol Int 2001;51:47-49.         [ Links ]

12. Sanders BM, West KW. Inflammatory pseudotumour of the alimentary tract: Clinical and surgical experience. J Pediatr Surg 2001;36:169-173.         [ Links ]

13. Ciftci AO, Caglar M, Tanyel FC, et al. Diagnostic aids in inflammatory pseudotumour of the liver. Pediatr Surg Int 1995;10:161-164.         [ Links ]

14. Apa H, Diniz G, Saritas T, et al. Abdominal inflammatory myofibroblastic tumour: review of literature by means of case report. Turk J Cancer 2002;32:28-31.         [ Links ]

15. Pettinato G, Manivel JC, De Rosa N, et al. Inflammatory myofibroblastic tumour (plasma cell granuloma). Clinicopathologic study of 20 cases with immunohistochemical and ultrastructural observations. Am J Clin Pathol 1990;94:538-546.         [ Links ]

16. Meis-Kindblom JM, Kjellstrom C, Kindblom LG, et al. Inflammatory fibrosarcoma: Update, reappraisal and perspective on its place in the spectrum of inflammatory myofibroblastic tumours. Sem Diag Pathol 1998;15:133-143.         [ Links ]

17. Atilli SVS, Chandra CR, Hemant D, et al. Retroperitoneal inflammatory myofibroblastic tumour- a case report. World J Surg Oncol 2005;3:66.         [ Links ]

18. Ciftci AO, Akcoren Z, Tanyel FC, et al. Inflammatory pseudotumour causing intestinal obstruction: Diagnostic and therapeutic aspects. J Pediatr Surg 1998;33:1843-1845.         [ Links ]

19. Germanidis G, Xanthakis I, Tsitouridis I, et al. Regression of inflammatory myofibroblastic tumour of the gastrointestinal tract under infliximab treatment. Dig Dis Sci 2005;50:262-265.         [ Links ]

20. Pungpapong S, Geiger XJ, Raimondo M. Inflammatory myofibroblastic tumour presenting as a pancreatic mass: A case report and reiew of literature. J Pancreas 2004;5:360-367.         [ Links ]

21. Souid AK, Ziemba MC, Dubansky AS, et al. Inflammatory myofibroblastic tumour in children. Cancer 1993;72:2042-2043.         [ Links ]

22. Khoddami M, Sanae S, Nikkhoo B, et al. Rectal and appendiceal inflammatory myofibroblastic tumours. Arch Iran Med 2006;9:277-281.         [ Links ]

23. Tsuzuki T, Magi-Galluzzi C, Epstein JL. ALK-1 expression in inflammatory myofibroblastic tumour of the urinary bladder. Am J Surg Pathol 2004;28:1609-1614.         [ Links ]

24. Bonnet JP, Basset T, Dijoux D. Abdominal Inflammatory myofibroblastic tumour in children: Report of an appendiceal case and review of the literature. J Pediatr Surg 1996;31:1311-1314.         [ Links ]

25. Miettinen M, Sobin LH, Sarlomo-Rikala M. Immunohistochemical spectrum of GISTs at different sites and their differential diagnosis with CD117 (KIT). Modern Pathol 2000;13:1134-1142.         [ Links ]

26. Shima Y, Ikegami E, Migita M, et al. Congenital fibrosarcoma of the jejunum in a premature infant with meconium peritonitis. Eur J Pediatr Surg 2003;13:134-136.         [ Links ]

27. Yousem.S.A., Shaw H, Cieply K. Involvement of 2p53 in pulmonary inflammatory pseudotumors. Hum Pathol 2001;32:428-433.         [ Links ]

28. 28. Coffin CM, Watterson J, Priest JR, et al. Extrapulmonary inflammatory myofibroblastic tumour (inflammatory psuedotumour): A clinicopathological and immunohistochemical study of 84 cases. Am J Surg Pathol 1995;19:859-872.         [ Links ]

29. Fletcher CDM. Soft Tissue Tumours. In: Fletcher CDM, ed. Diagnostic Histopathology of Tumours. Philadelphia, USA: Churchill Livingstone, 2007:1527-1592.         [ Links ]