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ALK+ inflammatory myofibroblastic tumor

Friday 3 September 2004

Inflammatory myofibroblastic tumour has been allocated to the intermediate (rarely metastasizing) category.

They are regarded as neoplasms which, in children, are quite often characterized by ALK-1 gene rearrangements, and which may recur or metastasize in as many as 5% of cases. Unfortunately, aggressive behaviour cannot always be predicted on morphological grounds.


- soft tissues
- urinary bladder
- uterus (#16160478#)


- neonatal case (#15991166#, #14577098#, #12574774#)
- aggressive inflammatory myofibroblastic tumour
- atypical inflammatory myofibroblastic tumour


- rare metastases (#15747099#)

  • rare pulmonary metastases (#15747099#)


- smooth muscle actin + (SMA +)
- ALK + (#11684952#)


- 2p23 rearrangement (ALK)

  • 19p13.1 (TPM4)
  • 1q22.23 (TPM3)
  • 17q23 (CLTCL2)

Molecular biology

- ALK/TPM4 fusion gene ( at 19p13.1)
- ALK/TPM3 fusion gene(at 1q22.23)
- ALK/CLTCL2 fusion gene (at 17q23)
- ALK/CARS fusion gene (#13679433#)

See also

- inflammatory pseudotumor


- Inflammatory myofibroblastic tumor versus IgG4-related sclerosing disease and inflammatory pseudotumor: a comparative clinicopathologic study. Yamamoto H, Yamaguchi H, Aishima S, Oda Y, Kohashi K, Oshiro Y, Tsuneyoshi M. Am J Surg Pathol. 2009 Sep;33(9):1330-40. PMID: #19718789#

- Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. Coffin CM, Hornick JL, Fletcher CD. Am J Surg Pathol. 2007 Apr;31(4):509-20. PMID: #17414097#

- The evolving classification of soft tissue tumours: an update based on the new WHO classification. Fletcher CD. Histopathology. 2006 Jan;48(1):3-12. PMID: #16359532#

- Mergan F, Jaubert F, Sauvat F, et al. Inflammatory myofibroblastic tumor in children: clinical review with anaplastic lymphoma kinase, Epstein-Barr virus, and human herpesvirus 8 detection analysis. J Pediatr Surg 2005;40:1581–1586.

- Yamamoto H, Oda Y, Saito T, et al. p53 Mutation and MDM2 amplification in inflammatory myofibroblastic tumours. Histopathology 2003;42:431–439.

- Identification of CARS-ALK fusion in primary and metastatic lesions of an inflammatory myofibroblastic tumor. Debelenko LV, Arthur DC, Pack SD, Helman LJ, Schrump DS, Tsokos M. Lab Invest. 2003 Sep;83(9):1255-65. PMID: #13679433#

- Cook JR, Dehner LP, Collins MH, Ma Z, Morris SW, Coffin CM, Hill DA. Anaplastic lymphoma kinase (ALK) expression in the inflammatory myofibroblastic tumor: a comparative immunohistochemical study. Am J Surg Pathol. 2001 Nov;25(11):1364-71. PMID: #11684952#

- Coffin CM, Patel A, Perkins S, et al. ALK1 and p80 expression and chromosomal rearrangements involving 2p23 in inflammatory myofibroblastic tumor. Mod Pathol 2001;14:569–576.

- Griffin CA, Hawkins AL, Dvorak C, et al. Recurrent involvement of 2p23 in inflammatory myofibroblastic tumors. Cancer Res 1999;59:2776–2780.

- Coffin CM, Watterson J, Priest JR, Dehner LP. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. Am J Surg Pathol. 1995 Aug;19(8):859-72. PMID: #7611533#

- Souid AK, Ziemba MC, Dubansky AS, et al. Inflammatory myofibroblastic tumor in children. Cancer 1993;72:2042–2048.


  • ALK immunolabelling in myofibroblastic inflammatory tumor
  • ALK immunolabelling in myofibroblastic inflammatory tumor
  • ALK immunolabelling in myofibroblastic inflammatory tumor
  • ALK immunolabelling in myofibroblastic inflammatory tumor
  • Pulmonary myofibroblastic inflammatory tumor
  • Pulmonary myofibroblastic inflammatory tumor