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

2001-08-01   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

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

TPM4 (tropomyosin)

Location

19p13.12

Protein description

248 amino acids, 29 kDa; coiled coil structure

Result of the chromosomal anomaly

Description

5 TPM4 - 3 ALK

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

Highly cited references

Pubmed IDYearTitleCitations
109341422000TPM3-ALK and TPM4-ALK oncogenes in inflammatory myofibroblastic tumors.145
176945472008Development of anaplastic lymphoma kinase (ALK) small-molecule inhibitors for cancer therapy.46
171314712006Identification of squamous cell carcinoma associated proteins by proteomics and loss of beta tropomyosin expression in esophageal cancer.46
173186152007Proteomic profiling of proteins dysregulted in Chinese esophageal squamous cell carcinoma.41
200929872010Effects of triterpenes from Ganoderma lucidum on protein expression profile of HeLa cells.21
253007972014Recurrent LRP1-SNRNP25 and KCNMB4-CCND3 fusion genes promote tumor cell motility in human osteosarcoma.14
157645782004Low-grade sarcomas with CD34-positive fibroblasts and low-grade myofibroblastic sarcomas.5
328686462020Lorlatinib for the treatment of inflammatory myofibroblastic tumour with TPM4-ALK fusion following failure of entrectinib.4
169846142006Absence of human herpesvirus-8 and Epstein-Barr virus in inflammatory myofibroblastic tumor with anaplastic large cell lymphoma kinase fusion gene.4
150803012004Assessment of t(2;5)(p23;q35) translocation and variants in pediatric ALK+ anaplastic large cell lymphoma.4
312781402019The effects of crizotinib in a transgenic Drosophila model expressing the human TPM4-ALK fusion gene or TPM4.3
351724972017Crizotinib Failure in a TPM4-ALK-Rearranged Inflammatory Myofibroblastic Tumor With an Emerging ALK Kinase Domain Mutation.1
127873122003Inflammatory myofibroblastic tumor with predominant anaplastic lymphoma kinase-positive cells lacking a myofibroblastic phenotype.1
292685612017Pulmonary inflammatory myofibroblastic tumor with TPM4-ALK translocation.0

Bibliography

Pubmed IDLast YearTitleAuthors
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

Citation

Jean-Loup Huret

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

Atlas Genet Cytogenet Oncol Haematol. 2001-08-01

Online version: http://atlasgeneticsoncology.org/solid-tumor/5128/soft-tissues-inflammatory-myofibroblastic-tumor-with-t(2;19)(p23;p13)-tpm4-alk