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Alveolar rhabdomyosarcoma
| Alveolar rhabdomyosarcoma | |
|---|---|
| Specialty | Oncology  |
Alveolar rhabdomyosarcoma (ARMS) is a subtype of the rhabdomyosarcoma family of soft tissue cancers whose lineage is from mesenchymal cells and are related to skeletal muscle cells.[1] ARMS tumors resemble the alveolar tissue in the lungs.[1] Tumor location varies from patient to patient, but is commonly found in the head and neck region, male and female urogenital tracts, the torso, and extremities.[2]
Two fusion proteins can be associated with ARMS, but are not necessary: PAX3-FKHR (now known as FOXO1)[3][4] and PAX7-FKHR.[5][6] In children and adolescents ARMS accounts for about 1 percent of all malignancies, has an incidence rate of 1 per million, and most cases occur sporadically with no genetic predisposition.[1] PAX3-FOXO1 is now known to drive cancer-promoting gene expression programs through creation of distant genetic elements called super enhancers.[7]
- ^ a b c Barr, FG (2009-01-01). "Soft tissue tumors: Alveolar rhabdomyosarcoma". Atlas of Genetics and Cytogenetics in Oncology and Haematology (12). doi:10.4267/2042/44650. hdl:2042/44650. ISSN 1768-3262.
- ^ Balogh, Petra; Bánusz, Rita; Csóka, Monika; Váradi, Zsófia; Varga, Edit; Sápi, Zoltán (2016-01-01). "Primary alveolar rhabdomyosarcoma of the bone: two cases and review of the literature". Diagnostic Pathology. 11 (1): 99. doi:10.1186/s13000-016-0552-9. ISSN 1746-1596. PMC 5069778. PMID 27756397.
- ^ Fredericks WJ, Galili N, Mukhopadhyay S, et al. (March 1995). "The PAX3-FKHR fusion protein created by the t(2;13) translocation in alveolar rhabdomyosarcomas is a more potent transcriptional activator than PAX3". Mol. Cell. Biol. 15 (3): 1522–35. doi:10.1128/MCB.15.3.1522. PMC 230376. PMID 7862145.
- ^ Mercado GE, Xia SJ, Zhang C, et al. (June 2008). "Identification of PAX3-FKHR-regulated genes differentially expressed between alveolar and embryonal rhabdomyosarcoma: focus on MYCN as a biologically relevant target". Genes Chromosomes Cancer. 47 (6): 510–20. doi:10.1002/gcc.20554. PMID 18335505. S2CID 21917973.
- ^ Mercado GE, Barr FG (February 2007). "Fusions involving PAX and FOX genes in the molecular pathogenesis of alveolar rhabdomyosarcoma: recent advances". Curr. Mol. Med. 7 (1): 47–61. doi:10.2174/156652407779940440. PMID 17311532. Archived from the original on 2013-04-14.
- ^ Laé M, Ahn EH, Mercado GE, et al. (June 2007). "Global gene expression profiling of PAX-FKHR fusion-positive alveolar and PAX-FKHR fusion-negative embryonal rhabdomyosarcomas". J. Pathol. 212 (2): 143–51. doi:10.1002/path.2170. PMID 17471488. S2CID 25440633.
- ^ Gryder, Berkley E.; Yohe, Marielle E.; Chou, Hsien-Chao; Zhang, Xiaohu; Marques, Joana; Wachtel, Marco; Schaefer, Beat; Sen, Nirmalya; Song, Young (August 2017). "PAX3-FOXO1 Establishes Myogenic Super Enhancers and Confers BET Bromodomain Vulnerability". Cancer Discovery. 7 (8): 884–899. doi:10.1158/2159-8290.CD-16-1297. ISSN 2159-8290. PMC 7802885. PMID 28446439.
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