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Genetic modeling of human rhabdomyosarcoma.

Publication ,  Journal Article
Linardic, CM; Downie, DL; Qualman, S; Bentley, RC; Counter, CM
Published in: Cancer Res
June 1, 2005

Rhabdomyosarcoma, a malignancy showing features of skeletal muscle differentiation, is the most common soft tissue sarcoma of childhood. The identification of distinct clinical presentation patterns, histologic tumor types, and risk groups suggests that rhabdomyosarcoma is a collection of highly related sarcomas rather than a single entity. In an effort to understand this seemingly heterogeneous malignancy, we constructed a genetically defined but malleable model of rhabdomyosarcoma by converting less differentiated human skeletal muscle cell precursors (SkMC) and committed human skeletal muscle myoblasts (HSMM) into their malignant counterparts by targeting pathways altered in rhabdomyosarcoma. Whereas the two cell types were both tumorigenic, SkMCs gave rise to highly heterogeneous tumors occasionally displaying features of rhabdomyosarcoma, whereas HSMMs formed rhabdomyosarcoma-like tumors with an embryonal morphology, capable of invasion and metastasis. Thus, despite introducing the same panel of genetic changes, altering the skeletal muscle cell of origin led to different tumor morphologies, suggesting that cell of origin may dictate rhabdomyosarcoma tumor histology. The ability to now genetically induce human rhabdomyosarcoma-like tumors provides a representative model to dissect the molecular mechanisms underlying this cancer.

Duke Scholars

Published In

Cancer Res

DOI

ISSN

0008-5472

Publication Date

June 1, 2005

Volume

65

Issue

11

Start / End Page

4490 / 4495

Location

United States

Related Subject Headings

  • Transplantation, Heterologous
  • Telomerase
  • Rhabdomyosarcoma
  • Oncology & Carcinogenesis
  • Neoplasm Transplantation
  • Muscle, Skeletal
  • Mice, SCID
  • Mice
  • Mesenchymal Stem Cells
  • Humans
 

Citation

APA
Chicago
ICMJE
MLA
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Linardic, C. M., Downie, D. L., Qualman, S., Bentley, R. C., & Counter, C. M. (2005). Genetic modeling of human rhabdomyosarcoma. Cancer Res, 65(11), 4490–4495. https://doi.org/10.1158/0008-5472.CAN-04-3194
Linardic, Corinne M., Diane L. Downie, Stephen Qualman, Rex C. Bentley, and Christopher M. Counter. “Genetic modeling of human rhabdomyosarcoma.Cancer Res 65, no. 11 (June 1, 2005): 4490–95. https://doi.org/10.1158/0008-5472.CAN-04-3194.
Linardic CM, Downie DL, Qualman S, Bentley RC, Counter CM. Genetic modeling of human rhabdomyosarcoma. Cancer Res. 2005 Jun 1;65(11):4490–5.
Linardic, Corinne M., et al. “Genetic modeling of human rhabdomyosarcoma.Cancer Res, vol. 65, no. 11, June 2005, pp. 4490–95. Pubmed, doi:10.1158/0008-5472.CAN-04-3194.
Linardic CM, Downie DL, Qualman S, Bentley RC, Counter CM. Genetic modeling of human rhabdomyosarcoma. Cancer Res. 2005 Jun 1;65(11):4490–4495.

Published In

Cancer Res

DOI

ISSN

0008-5472

Publication Date

June 1, 2005

Volume

65

Issue

11

Start / End Page

4490 / 4495

Location

United States

Related Subject Headings

  • Transplantation, Heterologous
  • Telomerase
  • Rhabdomyosarcoma
  • Oncology & Carcinogenesis
  • Neoplasm Transplantation
  • Muscle, Skeletal
  • Mice, SCID
  • Mice
  • Mesenchymal Stem Cells
  • Humans