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Soft Tissues: Inflammatory myofibroblastic tumor

Written2003-08Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France
This article is an update of :
2001-08Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

(Note : for Links provided by Atlas : click)


ICD-Topo C470-C476,C478-C479,C490-C496,C498-C499 CONNECTIVE & SOFT TISSUE
ICD-Morpho 8825/1 Inflammatory myofibroblastic tumour
Atlas_Id 5073
Phylum Soft Tissues::Inflammatory myofibroblastic tumor
WHO/OMS Classification Soft Tissues
Other namesatypical fibromyxoid tumor
pseudosarcomatous fibromyxoid tumor
plasma cell granuloma
pseudosarcomatous myofibrotic proliferation
postoperative spindle cell nodules
inflammatory pseudotumor

Clinics and Pathology

Disease rare soft tissue tumour of controversial nosology; at the edge between benign and malignant tumours, with a possible heterogeneity
Epidemiology found in children and young adults
Clinics occurs in the soft tissue and viscera; the tumour is localized in lungs, mesentery, retroperitoneum, and pelvis.
Pathology spindle cell proliferation with myofibroblastic differenciation with a collagen stroma and an inflammatory infiltrate of lymphocytes, eosinophils, and plasma cells
Treatment surgical excision
Prognosis This tumour has an indeterminate or low malignant potential; tumour related deaths (occuring in less than 10% of cases) are due to local invasion, not to distant metastases


Note about one 50 to 60 % of the cases present with a 2p23 rearrangement involving the gene ALK; this subset of tumours should have a different genetic background, and may have a different behaviour, in terms of epidemiology (younger age?), prognosis ...


So far, t(1;2)(q25;p23), t(2;2)(p23;q13), t(2;11)(p23;p15), t(2;17)(p23;q23), and t(2;19)(p23;p13.1) have been described; the t(1;2)(q25;p23) with TPM3 involvement would be the most frequent.

Genes involved and Proteins

Note these translocations involve ALK in 2p23, and either TPM3 in 1q25, CLTC in 17q23, or TPM4 in 19p13
Gene NameALK (anaplastic lymphoma receptor tyrosine kinase)
Location 2p23.2
Protein 1620 amino acids; 177 kDa; glycoprotein (200 kDa mature protein) ; membrane associated tyrosine kinase receptor

Gene NameTPM3 (tropomyosin 3)
Location 1q21.3
Protein 284 amino acids, 33 kDa; coiled coil structure; role in Calcium dependant actin-myosin interaction

Gene NameRANBP2 (RAN binding protein 2)
Location 2q12.3
Protein 3224 amino acids, 358 kDa; nuclear pore protein involved in nuclear export

Gene NameCARS (cysteinyj-tRNA synthetase)
Location 11p15.4

Gene NameCLTC (clathrin heavy polypeptide)
Location 17q23.1
Protein 1675 amino acids, 191 kDa; mediate endocytosis of transmembrane receptors.

Gene NameTPM4 (tropomyosin)
Location 19p13.12
Protein 248 amino acids, 29 kDa; coiled coil structure

Result of the chromosomal anomaly

Hybrid Gene
Description 5' partner - 3' ALK
Fusion Protein
Description N-term amino acids from the partner gene fused to the 562 C-term amino acids from ALK (i.e. the entire cytoplasmic portion of ALK with the tyrosine kinase domain); homodimerization of the fusion protein is known or suspected.

To be noted

ALK and some of the above ALK partners, or closely related genes, are found implicated both in anaplasic large cell lymphoma and in inflammatory myofibroblastic tumours; this is a new concept, that 2 different types of tumour may result from the same chromosomal/genes


Fusion of the ALK gene to the clathrin heavy chain gene, CLTC, in inflammatory myofibroblastic tumor.
Bridge JA, Kanamori M, Ma Z, Pickering D, Hill DA, Lydiatt W, Lui MY, Colleoni GW, Antonescu CR, Ladanyi M, Morris SW
The American journal of pathology. 2001 ; 159 (2) : 411-415.
PMID 11485898
Anaplastic lymphoma kinase (ALK) expression in the inflammatory myofibroblastic tumor: a comparative immunohistochemical study.
Cook JR, Dehner LP, Collins MH, Ma Z, Morris SW, Coffin CM, Hill DA
The American journal of surgical pathology. 2001 ; 25 (11) : 1364-1371.
PMID 11684952
Identification of novel fusion partners of ALK, the anaplastic lymphoma kinase, in anaplastic large-cell lymphoma and inflammatory myofibroblastic tumor.
Cools J, Wlodarska I, Somers R, Mentens N, Pedeutour F, Maes B, De Wolf-Peeters C, Pauwels P, Hagemeijer A, Marynen P
Genes, chromosomes & cancer. 2002 ; 34 (4) : 354-362.
PMID 12112524
Recurrent involvement of 2p23 in inflammatory myofibroblastic tumors.
Griffin CA, Hawkins AL, Dvorak C, Henkle C, Ellingham T, Perlman EJ
Cancer Res. 1999.
PMID 10383129
Aberrant ALK tyrosine kinase signaling. Different cellular lineages, common oncogenic mechanisms.
Ladanyi M
The American journal of pathology. 2000 ; 157 (2) : 341-345.
PMID 10934137
TPM3-ALK and TPM4-ALK oncogenes in inflammatory myofibroblastic tumors.
Lawrence B, PerezAtayde A, Hibbard MK, Rubin BP, Dal Cin P, Pinkus JL, Pinkus GS, Xiao S, Yi ES, Fletcher CDM, Fletcher JA
Am J Pathol. 2000.
PMID 10934142
Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor.
Ma Z, Hill DA, Collins MH, Morris SW, Sumegi J, Zhou M, Zuppan C, Bridge JA
Genes, chromosomes & cancer. 2003 ; 37 (1) : 98-105.
PMID 12661011
ALK probe rearrangement in a t(2;11;2)(p23;p15;q31) translocation found in a prenatal myofibroblastic fibrous lesion: toward a molecular definition of an inflammatory myofibroblastic tumor family?
Sirvent N, Hawkins AL, Moeglin D, Coindre JM, Kurzenne JY, Michiels JF, Barcelo G, Turc-Carel C, Griffin CA, Pedeutour F
Genes, chromosomes & cancer. 2001 ; 31 (1) : 85-90.
PMID 11284039


This paper should be referenced as such :
Huret, JL
Soft tissue tumors: Inflammatory myofibroblastic tumor
Atlas Genet Cytogenet Oncol Haematol. 2003;7(4):280-281.
Free journal version : [ pdf ]   [ DOI ]
On line version :
History of this paper:
Huret, JL. Soft tissue tumors: Inflammatory myofibroblastic tumor. Atlas Genet Cytogenet Oncol Haematol. 2001;5(4):298-299.

Other genes implicated (Data extracted from papers in the Atlas) [ 9 ]


Translocations implicated (Data extracted from papers in the Atlas)

 inv(2)(p23q35) ATIC/ALK
 t(1;2)(q25;p23) TPM3/ALK
 t(2;11)(p23;p15) CARS/ALK
 t(2;12)(p23;p11) PPFIBP1/ALK
 t(2;17)(p23;q23) CLTC/ALK
 t(2;19)(p23;p13) TPM4/ALK
 t(2;2)(p23;q13) RANBP2/ALK
 t(2;4)(p23;q21) SEC31A/ALK

External links

Mitelman database inv(2)(p23q35) [CaseList]     inv(2)(p23q35) [Transloc - MCList]   ATIC/ALK Fusion - MCList]
Mitelman database t(1;2)(q25;p23) [CaseList]     t(1;2)(q25;p23) [Transloc - MCList]   TPM3/ALK Fusion - MCList]
COSMIC[ TPM3 ]   [ ALK ]
Mitelman database t(2;11)(p23;p15) [CaseList]     t(2;11)(p23;p15) [Transloc - MCList]   CARS/ALK Fusion - MCList]
Mitelman database t(2;12)(p23;p11) [CaseList]     t(2;12)(p23;p11) [Transloc - MCList]   PPFIBP1/ALK Fusion - MCList]
Mitelman database t(2;17)(p23;q23) [CaseList]     t(2;17)(p23;q23) [Transloc - MCList]   CLTC/ALK Fusion - MCList]
Mitelman database t(2;19)(p23;p13) [CaseList]     t(2;19)(p23;p13) [Transloc - MCList]   TPM4/ALK Fusion - MCList]
COSMIC[ TPM4 ]   [ ALK ]
Mitelman database t(2;2)(p23;q13) [CaseList]     t(2;2)(p23;q13) [Transloc - MCList]   RANBP2/ALK Fusion - MCList]
Mitelman database t(2;4)(p23;q21) [CaseList]     t(2;4)(p23;q21) [Transloc - MCList]   SEC31A/ALK Fusion - MCList]
COSMIC[ SEC31A ]   [ ALK ]
arrayMap Topo ( C47,C49) arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
COSMIC_fusionATIC (2q35) ALK (2p23.2)    [fusion444] [fusion445] [fusion446]
Mitelman databaseATIC/ALK[MCList]    ATIC (2q35) ALK (2p23.2)   
Mitelman databaseATIC/ALK[MCList]    ATIC (2q35) ALK (2p23.2)   inv(2)(p23q35)
TICdbATIC/ALK    ATIC (2q35) ALK (2p23.2)
COSMIC_fusionCARS (11p15.4) ALK (2p23.2)    [fusion437] [fusion438] [fusion753]
Mitelman databaseCARS/ALK[MCList]    CARS (11p15.4) ALK (2p23.2)   
Mitelman databaseCARS/ALK[MCList]    CARS (11p15.4) ALK (2p23.2)   t(2;11)(p23;p15)
TICdbCARS/ALK    CARS (11p15.4) ALK (2p23.2)
Mitelman databaseCLTC/ALK[MCList]    CLTC (17q23.1) ALK (2p23.2)   
Mitelman databaseCLTC/ALK[MCList]    CLTC (17q23.1) ALK (2p23.2)   t(2;17)(p23;q23)
TICdbCLTC/ALK    CLTC (17q23.1) ALK (2p23.2)
Mitelman databasePPFIBP1/ALK[MCList]    PPFIBP1 (12p11.23) ALK (2p23.2)   t(2;12)(p23;p11)
COSMIC_fusionRANBP2 (2q12.3) ALK (2p23.2)    [fusion415] [fusion416] [fusion417] [fusion418]
Mitelman databaseRANBP2/ALK[MCList]    RANBP2 (2q12.3) ALK (2p23.2)   inv(2)(p23q13)
TICdbRANBP2/ALK    RANBP2 (2q12.3) ALK (2p23.2)
Mitelman databaseSEC31A/ALK[MCList]    SEC31A (4q21.22) ALK (2p23.2)   
Mitelman databaseSEC31A/ALK[MCList]    SEC31A (4q21.22) ALK (2p23.2)   t(2;4)(p23;q21)
TICdbSEC31A/ALK    SEC31A (4q21.22) ALK (2p23.2)
Mitelman databaseTPM3/ALK[MCList]    TPM3 (1q21.3) ALK (2p23.2)   t(1;2)(q21;p23)
TICdbTPM3/ALK    TPM3 (1q21.3) ALK (2p23.2)
COSMIC_fusionTPM4 (19p13.12) ALK (2p23.2)    [fusion441] [fusion442]
Mitelman databaseTPM4/ALK[MCList]    TPM4 (19p13.12) ALK (2p23.2)   t(2;19)(p23;p13)
TICdbTPM4/ALK    TPM4 (19p13.12) ALK (2p23.2)
Disease databaseSoft Tissues: Inflammatory myofibroblastic tumor
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed

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