| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
Priority Reports |
Departments of 1 Pediatrics, 2 Pharmacology and Cancer Biology, 3 Pathology, and 4 Radiation Oncology, Duke University Medical Center, Durham, North Carolina; and 5 Center for Childhood Cancer, Columbus Children's Research Institute and Children's Hospital, Columbus, Ohio
Requests for reprints: Christopher M. Counter, Duke University Medical Center, Box 3813, Durham, NC 27710. Phone: 919-684-9890; Fax: 919-684-8958; E-mail: count004{at}mc.duke.edu.
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.
This article has been cited by other articles:
|
|
 |
|
  S. J. Xia, D. D. Holder, B. R. Pawel, C. Zhang, and F. G. Barr High Expression of the PAX3-FKHR Oncoprotein Is Required to Promote Tumorigenesis of Human Myoblasts Am. J. Pathol., December 1, 2009; 175(6): 2600 - 2608. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. M. O'Hayer, D. C. Brady, and C. M. Counter ELR+ CXC chemokines and oncogenic Ras-mediated tumorigenesis Carcinogenesis, November 1, 2009; 30(11): 1841 - 1847. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S. Naini, K. T. Etheridge, S. J. Adam, S. J. Qualman, R. C. Bentley, C. M. Counter, and C. M. Linardic Defining the Cooperative Genetic Changes That Temporally Drive Alveolar Rhabdomyosarcoma Cancer Res., December 1, 2008; 68(23): 9583 - 9588. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Y.-X. Ren, F. G. Finckenstein, D. A. Abdueva, V. Shahbazian, B. Chung, K. I. Weinberg, T. J. Triche, H. Shimada, and M. J. Anderson Mouse Mesenchymal Stem Cells Expressing PAX-FKHR Form Alveolar Rhabdomyosarcomas by Cooperating with Secondary Mutations Cancer Res., August 15, 2008; 68(16): 6587 - 6597. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. M. Linardic, S. Naini, J. E. Herndon II, C. Kesserwan, S. J. Qualman, and C. M. Counter The PAX3-FKHR Fusion Gene of Rhabdomyosarcoma Cooperates with Loss of p16INK4A to Promote Bypass of Cellular Senescence Cancer Res., July 15, 2007; 67(14): 6691 - 6699. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Davicioni, F. Graf Finckenstein, V. Shahbazian, J. D. Buckley, T. J. Triche, and M. J. Anderson Identification of a PAX-FKHR Gene Expression Signature that Defines Molecular Classes and Determines the Prognosis of Alveolar Rhabdomyosarcomas. Cancer Res., July 15, 2006; 66(14): 6936 - 6946. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| Cancer Research | Clinical Cancer Research |
| Cancer Epidemiology Biomarkers & Prevention | Molecular Cancer Therapeutics |
| Molecular Cancer Research | Cancer Prevention Research |
| Cancer Prevention Journals Portal | Cancer Reviews Online |
| Annual Meeting Education Book | Meeting Abstracts Online |
