Rhabdomyosarcoma

Definition:

Rhabdomyosarcomas (RMSs) are soft tissue sarcomas that are one of the most common neoplasms in children and adolescents. RMSs are presumed to be associated with the skeletal muscle lineage, although those tumors can be present in organs histologically lacking skeletal muscle, like prostate, urinary bladder or gallbladder. RMS comprises a heterogeneous into two major histologic subtypes, embryonal (ERMS) and alveolar (ARMS). Alveolar rhabdomyosarcoma (ARMS) is a pediatric sarcoma that typically occurs in older children predominantly arising in the trunk and extremities, and exhibits a worse prognosis than other types of RMSs. ARMS is associated with 2;13 or 1;13 chromosomal translocations, which generate PAX3-FOXO1A and PAX7-FOXO1A fusion products, respectively. These translocations result in altered expression, function, and subcellular localization of the fusion products relative to the wild-type proteins, and ultimately contribute to oncogenic behavior by modifying growth, differentiation, and apoptosis pathways.

References:

[1]. Elizabeth Charytonowicz, et al. Alveolar rhabdomyosarcoma: is the cell of origin a mesenchymal stem cell?. Cancer Lett. 2009 Jul 8;279(2):126-36. [Content Brief]

[2]. G D Letson, et al. Genetic and molecular abnormalities in tumors of the bone and soft tissues. Cancer Control. 2001 May-Jun;8(3):239-51. [Content Brief]

[3]. Ken Kikuchi, et al. MDM2 Amplification and PI3KCA Mutation in a Case of Sclerosing Rhabdomyosarcoma. Sarcoma. 2013;2013:520858. [Content Brief]

[4]. L Krsková, et al. Rhabdomyosarcoma: molecular diagnostics of patients classified by morphology and immunohistochemistry with emphasis on bone marrow and purged peripheral blood progenitor cells involvement. Virchows Arch. 2006 Apr;448(4):449-58. [Content Brief]

[5]. Lee J Helman, et al. Mechanisms of sarcoma development. Nat Rev Cancer. 2003 Sep;3(9):685-94. [Content Brief]

[6]. Shujuan J Xia, et al. Molecular pathogenesis of rhabdomyosarcoma. Cancer Biol Ther. 2002 Mar-Apr;1(2):97-104. [Content Brief]

[7]. Steffen Albrecht, et al. Allelic loss but absence of mutations in the polyspecific transporter gene BWR1A on 11p15.5 in hepatoblastoma. Int J Cancer. 2004 Sep 10;111(4):627-32. [Content Brief]

[8]. William D Foulkes, et al. Extending the phenotypes associated with DICER1 mutations. Hum Mutat. 2011 Dec;32(12):1381-4. [Content Brief]

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