Soft Tissues: Inflammatory myofibroblastic tumor with t(2;2)(p23;q13)ALK/RANBP2

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; staining of ALK-positive cases, representing 60% of IMT cases, is usually confined to the cytoplasm, but it showed a nuclear membran pattern in one of the two t(2;2) available cases

Prognosis

Inflammatory myofibroblastic tumors have a low malignant potential and good prognosis; however, the 2 cases with a t(2;2) showed (relatively) aggressive clinical course with tumor recurrence and round cell transformation

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

RANBP2 (RAN binding protein 2)

Location

2q12.3

Protein description

3224 amino acids; nuclear pore protein involved in nuclear export

Result of the chromosomal anomaly

Description

5 RANBP2 - 3 ALK

Oncogenesis

the leucine zipper of RANBP2 could mediate oligomerization of RANBP2-ALK

Highly cited references

Pubmed IDYearTitleCitations
176562522007RANBP2 and CLTC are involved in ALK rearrangements in inflammatory myofibroblastic tumors.15

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
126610112003Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor.Ma Z et al
113803912001Alk+ CD30+ lymphomas: a distinct molecular genetic subtype of non-Hodgkin's lymphoma.Morris SW et al

External Links

Citation

Jean-Loup Huret

Soft Tissues: Inflammatory myofibroblastic tumor with t(2;2)(p23;q13)ALK/RANBP2

Atlas Genet Cytogenet Oncol Haematol. 2003-08-01

Online version: http://atlasgeneticsoncology.org/solid-tumor/5214/t0202p23q13id5214