| Identity |
| Other names | atypical 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 |
| Genetics |
| 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 ... |
| Cytogenetics |
| 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 |
| 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 |
| Bibliography |
| Recurrent involvement of 2p23 in inflammatory myofibroblastic tumors. |
| Griffin CA, Hawkins AL, Dvorak C, Henkle C, Ellingham T, Perlman EJ. |
| Cancer Res 1999, 59: 2776-2780. |
| TPM3-ALK and TPM4-ALK oncogenes in inflammatory myofibroblastic tumors. |
| Lawrence B, Perez-Atayde A, Hibbard MK, Rubin BP, Dal Cin P, Pinkus JL, Pinkus GS, Xiao S, Yi ES, Fletcher CDM, Fletcher JA. |
| Am J Pathol 2000, 157: 377-384. |
| Aberrant ALK tyrosine kinase signaling. Different cellular lineages, common oncogen,ic mechanims? |
| Ladanyi M. |
| Am J Pathol 2000; 157: 341-345. |
| ALK probe rearrangement in a t(2;11;2)(p23;p15;q31) translocation found in a prenatal mtofibroblastic 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 Chromosom Cancer 2001; 31: 85-90. |
| Fusion of the ALK Gene to the Clathrin Heavy Chain Gene, CLTC, in Inflammatory Myofibroblastic Tumor. |
| Bridge JA, Kanamori M, Ma Z, Pickering D, D. Hill A, Lydiatt W, Lui MY, Colleoni GWB, Antonescu CR, Ladanyi M, Morris SW. |
| Am J Pathol 2001; 159: 411-415. |
| Anaplastic lymphoma kinase (ALK)expression in the inflammatorymyofibroblastic tumor, a comparative immunohistochemical study.. |
| Cook JR, Dehner LP, Collins MH, Ma Z, Morris SW, Coffin CM, Hill DA. |
| Am J Surg Pathol 2001; 25: 1364-1371. |
| 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: 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: 98-105. |
| PMID 12661011 |
| REVIEW articles | automatic search in PubMed |
| Last year publications | automatic search in PubMed |
| Contributor(s) |
| Written | 08-2001 | Jean-Loup Huret |
| Updated | 08-2003 | Jean-Loup Huret |
| Citation |
| This paper should be referenced as such : |
| Huret JL . Soft tissue tumors: Inflammatory myofibroblastic tumor. Atlas Genet Cytogenet Oncol Haematol. August 2001 . URL : http://AtlasGeneticsOncology.org/Tumors/MyofibroID5073.html |
| Huret JL . Soft tissue tumors: Inflammatory myofibroblastic tumor. Atlas Genet Cytogenet Oncol Haematol. August 2003 . URL : http://AtlasGeneticsOncology.org/Tumors/MyofibroID5073.html |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Thu Apr 17 14:14:39 2008 |
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