Soft Tissues: Inflammatory myofibroblastic tumor

2003-08-01   Jean-Loup Huret 

1.Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

Clinics and Pathology


found in children and young adults


occurs in the soft tissue and viscera; the tumour is localized in lungs, mesentery, retroperitoneum, and pelvis.


spindle cell proliferation with myofibroblastic differenciation with a collagen stroma and an inflammatory infiltrate of lymphocytes, eosinophils, and plasma cells


surgical excision


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



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 ...


Cytogenetics morphological

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


these translocations involve ALK in 2p23, and either TPM3 in 1q25, CLTC in 17q23, or TPM4 in 19p13

Gene name

ALK (anaplastic lymphoma receptor tyrosine kinase)



Protein description

1620 amino acids; 177 kDa; glycoprotein (200 kDa mature protein) ; membrane associated tyrosine kinase receptor

Gene name

TPM3 (tropomyosin 3)



Protein description

284 amino acids, 33 kDa; coiled coil structure; role in Calcium dependant actin-myosin interaction

Gene name

RANBP2 (RAN binding protein 2)



Protein description

3224 amino acids, 358 kDa; nuclear pore protein involved in nuclear export

Gene name

CARS (cysteinyj-tRNA synthetase)



Gene name

CLTC (clathrin heavy polypeptide)



Protein description

1675 amino acids, 191 kDa; mediate endocytosis of transmembrane receptors.

Gene name

TPM4 (tropomyosin)



Protein description

248 amino acids, 29 kDa; coiled coil structure

Result of the chromosomal anomaly


5 partner - 3 ALK

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


Pubmed IDLast YearTitleAuthors
114858982001Fusion of the ALK gene to the clathrin heavy chain gene, CLTC, in inflammatory myofibroblastic tumor.Bridge JA et al
116849522001Anaplastic lymphoma kinase (ALK) expression in the inflammatory myofibroblastic tumor: a comparative immunohistochemical study.Cook JR et al
121125242002Identification of novel fusion partners of ALK, the anaplastic lymphoma kinase, in anaplastic large-cell lymphoma and inflammatory myofibroblastic tumor.Cools J et al
103831291999Recurrent involvement of 2p23 in inflammatory myofibroblastic tumors.Griffin CA et al
109341372000Aberrant ALK tyrosine kinase signaling. Different cellular lineages, common oncogenic mechanisms.Ladanyi M et al
109341422000TPM3-ALK and TPM4-ALK oncogenes in inflammatory myofibroblastic tumors.Lawrence B et al
126610112003Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor.Ma Z et al
112840392001ALK 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 et al

External Links


Jean-Loup Huret

Soft Tissues: Inflammatory myofibroblastic tumor

Atlas Genet Cytogenet Oncol Haematol. 2003-08-01

Online version:

Historical Card

2001-08-01 Soft Tissues: Inflammatory myofibroblastic tumor by  Jean-Loup Huret 

Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France