Soft Tissues: Inflammatory myofibroblastic tumor with t(2;11)(p23;p15) CARS/ALK

2003-08-01   Jean-Loup Huret  

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

Clinics and Pathology

Clinics

rare soft tissue tumour found in children and young adults

Pathology

spindle cell proliferation with myofibroblastic differenciation and an inflammatory infiltrate

Cytogenetics

The translocation was found complex: t(2;11;2)(p23;p15;q31)

Prognosis

low malignant potential and good prognosis

Genes Involved and Proteins

Gene name

ALK (anaplastic lymphoma receptor tyrosine kinase)

Location

2p23.2

Protein description

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

Gene name

CARS (cysteinyj-tRNA synthetase)

Location

11p15.4

Protein description

748 amino acids, 85 kDa. Forms homodimers

Result of the chromosomal anomaly

Description

5 CARS - 3 ALK

Description

606 N-term amino acid from CARS, fused to the 562 C-term amino acids from ALK (i.e. the entire cytoplasmic portion of ALK with the tyrosine kinase domain).

Highly cited references

Pubmed IDYearTitleCitations
136794332003Identification of CARS-ALK fusion in primary and metastatic lesions of an inflammatory myofibroblastic tumor.32
340016952021Clinicopathologic and Genomic Characterization of Inflammatory Myofibroblastic Tumors of the Head and Neck: Highlighting a Novel Fusion and Potential Diagnostic Pitfall.3
329974362021Metastatic Low-Grade Sarcoma with CARS-ALK Fusion Dramatically Responded to Multiple ALK Tyrosine Kinase Inhibitors: A Case Report with Comprehensive Genomic Analysis.2
355603682022Cutaneous inflammatory myofibroblastic tumor with CARS-ALK fusion: Case report and literature review.0

Article Bibliography

Pubmed IDLast YearTitleAuthors
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
126610112003Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor.Ma Z et al

Citation

Jean-Loup Huret

Soft Tissues: Inflammatory myofibroblastic tumor with t(2;11)(p23;p15) CARS/ALK

Atlas Genet Cytogenet Oncol Haematol. 2003-08-01

Online version: http://atlasgeneticsoncology.org/solid-tumor/5213/js/favicon/teaching-explorer/