Perspective ARTICLE

Front. Oncol., 16 January 2012 | http://dx.doi.org/10.3389/fonc.2012.00002

The clone wars – revenge of the metastatic rogue state: the sarcoma paradigm

Holly L. Spraker1,2*, Shawn L. Price2,3, Aashi Chaturvedi2,4, Joshua D. Schiffman1,2,4, Kevin B. Jones2,3, Stephen L. Lessnick1,2,4, Mary Beckerle2,4 and R. Lor Randall2,3
  • 1 Division of Pediatric Hematology–Oncology, Primary Children’s Medical Center, University of Utah, Salt Lake City, UT, USA
  • 2 Center for Children’s Cancer Research, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT, USA
  • 3 Department of Orthopedics, Sarcoma Services, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT, USA
  • 4 Department of Oncological Sciences, University of Utah, Salt Lake City, UT, USA

Ewing sarcoma (ES) is the second most common bone tumor affecting primarily adolescents and young adults. Despite recent advances in biological understanding, intensification of chemotherapeutic treatments, and progress in local control with surgery and/or radiation therapy, patients with metastatic or recurrent ES continue to have a dismal prognosis with less than 20% overall survival. All ES is likely metastatic at diagnosis although our methods of detection and classification may not account for this. Progressive disease may arise via a combination of: (1) selection of chemotherapy-resistant clones in primary tumor, (2) signaling from bone or lung microenvironments that may attract tumor cells to distant locations, and/or (3) genetic changes within the ES cells themselves due to DNA-damaging chemotherapeutic agents or other “hits.” These possibilities and the evidence base to support them are explored.

Keywords: Ewing sarcoma, metastatic

Citation: Spraker HL, Price SL, Chaturvedi A, Schiffman JD, Jones KB, Lessnick SL, Beckerle M and Randall RL (2012) The clone wars – revenge of the metastatic rogue state: the sarcoma paradigm. Front. Oncol. 2:2. doi: 10.3389/fonc.2012.00002

Received: 27 July 2011; Paper pending published: 25 August 2011;
Accepted: 03 January 2012; Published online: 16 January 2012.

Edited by:

Douglas Hawkins, Seattle Children’s Hospital, USA

Reviewed by:

Ben Braun, University of California San Francisco, USA
Scott C. Borinstein, Vanderbilt University, USA

Copyright: © 2012 Spraker, Price, Chaturvedi, Schiffman, Jones, Lessnick, Beckerle and Randall. This is an open-access article distributed under the terms of the Creative Commons Attribution Non Commercial License, which permits non-commercial use, distribution, and reproduction in other forums, provided the original authors and source are credited.

*Correspondence: Holly L. Spraker, Division of Pediatric Hematology–Oncology, Primary Children’s Medical Center, 100 North Mario Capecchi Drive, Salt Lake City, UT 84113, USA. e-mail: holly.spraker@imail.org