Atlas of Genetics and Cytogenetics in Oncology and Haematology

Home   Genes   Leukemias   Solid Tumors   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

X Y 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 NA

Soft Tissues: Inflammatory myofibroblastic tumor with t(2;19)(p23;p13) TPM4/ALK

Written2001-08Jean-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 5128
Phylum Soft Tissues::Inflammatory myofibroblastic tumor
WHO/OMS Classification Soft Tissues

Clinics and Pathology

Disease Inflammatory myofibroblastic tumors
Clinics rare soft tissue tumour found in children and young adults
Pathology spindle cell proliferation with myofibroblastic differenciation and an inflammatory infiltrate
Prognosis low malignant potential and good prognosis

Genes involved and Proteins

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

Result of the chromosomal anomaly

Hybrid Gene
Description 5' TPM4 - 3' ALK
Fusion Protein
Description 783 amino acids. N-term TPM4 containing all but its 27 C-term amino acids , including the coiled coil domain, fused to the 562 C-term amino acids from ALK (i.e. the entire cytoplasmic portion of ALK with the tyrosine kinase domain)
Expression Localisation predominantly cytoplasmic
Oncogenesis TPM4-ALK is contituvely activated


Recurrent involvement of 2p23 in inflammatory myofibroblastic tumors.
Griffin CA, Hawkins AL, Dvorak C, Henkle C, Ellingham T, Perlman EJ
Cancer research. 1999 ; 59 (12) : 2776-2780.
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


This paper should be referenced as such :
Huret, JL
Atlas Genet Cytogenet Oncol Haematol. 2001;5(4):307-307.
Free journal version : [ pdf ]   [ DOI ]
On line version :

Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

Genes ALK

Translocations implicated (Data extracted from papers in the Atlas)

 t(2;19)(p23;p13) TPM4/ALK

External links

Mitelman database t(2;19)(p23;p13) [CaseList]     t(2;19)(p23;p13) [Transloc - MCList]   TPM4/ALK Fusion - MCList]
COSMIC[ TPM4 ]   [ ALK ]
arrayMap Topo ( C47,C49) arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
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 with t(2;19)(p23;p13) TPM4/ALK
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed

© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Mon Dec 14 18:30:48 CET 2020

Home   Genes   Leukemias   Solid Tumors   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

For comments and suggestions or contributions, please contact us