Soft Tissues: Inflammatory myofibroblastic tumor with t(2;2)(p23;q13)ALK/RANBP2
2003-08-01 Jean-Loup Huret Affiliation1.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
Description
1430 amino acids; 6867 N-term amino acid from RANBP2, fused to the 562 C-term amino acids from ALK (i.e. the leucine-rich domain, including the leucine zipper, of RANBP2 and the entire cytoplasmic portion of ALK with the tyrosine kinase domain).
Oncogenesis
the leucine zipper of RANBP2 could mediate oligomerization of RANBP2-ALK
Highly cited references
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 29437595 | 2018 | Integrated molecular profiling of juvenile myelomonocytic leukemia. | 0 |
| 17656252 | 2007 | RANBP2 and CLTC are involved in ALK rearrangements in inflammatory myofibroblastic tumors. | 0 |
| 34919183 | 2022 | [Mesenchymal tumors and tumor-like lesions of the gastrointestinal tract: an overview]. | 0 |
| 35543076 | 2022 | Histologic characterization of paediatric mesenchymal neoplasms treated with kinase-targeted therapy. | 0 |
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 10383129 | 1999 | Recurrent involvement of 2p23 in inflammatory myofibroblastic tumors. | Griffin CA et al |
| 10934137 | 2000 | Aberrant ALK tyrosine kinase signaling. Different cellular lineages, common oncogenic mechanisms. | Ladanyi M et al |
| 12661011 | 2003 | Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor. | Ma Z et al |
| 11380391 | 2001 | Alk+ CD30+ lymphomas: a distinct molecular genetic subtype of non-Hodgkin's lymphoma. | Morris SW et al |
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/soft-tissues-inflammatory-myofibroblastic-tumor-with-t(2
