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Taking over the Atlas
Dear Colleagues,
The Atlas, once more, is in great danger, and I will have to proceed to a collective economic lay-off of all the team involved in the Atlas before the begining of April 2015 (a foundation having suddenly withdrawn its commitment to support the Atlas). I ask you herein if any Scientific Society (a Society of Cytogenetics, of Clinical Genetics, of Hematology, or a Cancer Society, or any other...), any University and/or Hospital, any Charity, or any database would be interested in taking over the Atlas, in whole or in part. If taking charge of the whole lot is too big, a consortium of various actors could be the solution (I am myself trying to find partners). Could you please spread the information, contact the relevant authorities, and find partners.
Survival of the Atlas will be critically dependant upon your ability to find solutions (and urgently!).
Kind regards.
Jean-Loup Huret
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Soft tissue tumors: Inflammatory myofibroblastic tumor


ICD-Topo C470-C476,C478-C479,C490-C496,C498-C499 CONNECTIVE & SOFT TISSUE
ICD-Morpho 8825/1 Inflammatory myofibroblastic tumour
Phylum Soft Tissue Tumors:Inflammatory myofibroblastic tumor
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 Name ALK
Location 2p23
Protein 1620 amino acids; 177 kDa; glycoprotein (200 kDa mature protein) ; membrane associated tyrosine kinase receptor

Gene Name TPM3 (tropomyosin alpha chain)
Location 1q25
Protein 284 amino acids, 33 kDa; coiled coil structure; role in Calcium dependant actin-myosin interaction

Gene Name RANBP2
Location 2q13
Protein 3224 amino acids, 358 kDa; nuclear pore protein involved in nuclear export

Gene Name CARS
Location 11p15

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

Gene Name TPM4 (tropomyosin fibroblast, non muscle type)
Location 19p13.1
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

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


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 databaseTopo ( Soft_tissue ) - Mitelman database (CGAP - NCBI)
inv(2)(p23q35) - Mitelman database (CGAP - NCBI)
t(1;2)(q25;p23) - Mitelman database (CGAP - NCBI)
t(2;11)(p23;p15) - Mitelman database (CGAP - NCBI)
t(2;12)(p23;p11) - Mitelman database (CGAP - NCBI)
t(2;17)(p23;q23) - Mitelman database (CGAP - NCBI)
t(2;19)(p23;p13) - Mitelman database (CGAP - NCBI)
t(2;2)(p23;q13) - Mitelman database (CGAP - NCBI)
t(2;4)(p23;q21) - Mitelman database (CGAP - NCBI)
COSMICHisto = - Site = soft_tissue (COSMIC)
arrayMapTopo ( C47,C49) Morph ( 8825/1) - arrayMap (Zurich)


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
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
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
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
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
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
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed


Written08-2001Jean-Loup Huret
Updated08-2003Jean-Loup Huret


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 ]
History of this paper:
Huret, JL. Soft tissue tumors: Inflammatory myofibroblastic tumor. Atlas Genet Cytogenet Oncol Haematol. 2003;7(4):280-281.

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