Developmental origins of fusion-negative rhabdomyosarcomas

Ken Kikuchi, Brian P. Rubin, Charles Keller

Research output: Book/ReportBook

14 Citations (Scopus)

Abstract

Rhabdomyosarcomas (RMS) are very heterogeneous tumors that can be divided into three major groups: alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma, and pleomorphic rhabdomyosarcoma. Concerted efforts over the past a decade have led to an understanding of the genetic underpinnings of many human tumors through genetically engineered models; however, left largely behind in this effort have been rare tumors with poorly understood chromosomal abnormalities including the vast majority of RMS lacking a pathognomonic translocation, i.e. fusion-negative RMS. In this chapter, we review the characteristic genetic abnormalities associated with human RMS and the genetically engineered animal models for these fusion-negative RMS. We explore not only how specific combinations of mutations and cell of origin give rise to different histologically and biologically distinguishable pediatric and adult RMS subtypes, but we also examine how tumor cell phenotype (and tumor "stem" cell phenotype) can vary markedly from the cell of origin.

Original languageEnglish (US)
PublisherUnknown Publisher
Number of pages24
Volume96
DOIs
StatePublished - 2011

Publication series

NameCurrent Topics in Developmental Biology
Volume96
ISSN (Print)00702153

Fingerprint

Rhabdomyosarcoma
Neoplasms
Alveolar Rhabdomyosarcoma
Embryonal Rhabdomyosarcoma
Phenotype
Genetically Modified Animals
Neoplastic Stem Cells
Chromosome Aberrations
Animal Models
Pediatrics
Mutation

Keywords

  • Embryonal Rhabdomyosarcoma
  • Muscle
  • Myogenesis
  • Rhabdomyosarcoma
  • Satellite Cell
  • Stem cell

ASJC Scopus subject areas

  • Cell Biology
  • Developmental Biology

Cite this

Kikuchi, K., Rubin, B. P., & Keller, C. (2011). Developmental origins of fusion-negative rhabdomyosarcomas. (Current Topics in Developmental Biology; Vol. 96). Unknown Publisher. https://doi.org/10.1016/B978-0-12-385940-2.00002-4

Developmental origins of fusion-negative rhabdomyosarcomas. / Kikuchi, Ken; Rubin, Brian P.; Keller, Charles.

Unknown Publisher, 2011. 24 p. (Current Topics in Developmental Biology; Vol. 96).

Research output: Book/ReportBook

Kikuchi, K, Rubin, BP & Keller, C 2011, Developmental origins of fusion-negative rhabdomyosarcomas. Current Topics in Developmental Biology, vol. 96, vol. 96, Unknown Publisher. https://doi.org/10.1016/B978-0-12-385940-2.00002-4
Kikuchi K, Rubin BP, Keller C. Developmental origins of fusion-negative rhabdomyosarcomas. Unknown Publisher, 2011. 24 p. (Current Topics in Developmental Biology). https://doi.org/10.1016/B978-0-12-385940-2.00002-4
Kikuchi, Ken ; Rubin, Brian P. ; Keller, Charles. / Developmental origins of fusion-negative rhabdomyosarcomas. Unknown Publisher, 2011. 24 p. (Current Topics in Developmental Biology).
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