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February 17, 2004 • Volume 1 / Number 7 E-Mail This Document  |  Download PDF  |  Bulletin Archive/Search  |  Subscribe

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Special ReportSpecial Report

Sarcoma PRG Report Calls for Aggressive Action

The final report and recommendations of the Sarcoma Progress Review Group (PRG) released late last month, calls for the formation of a Sarcoma Research Consortium (SRC) that will serve as a guide for sarcoma clinical trials and related basic and clinical research. The SRC would also enhance the network of investigators and cancer centers committed to sarcoma research.

Although the report recommends specific actions in six priority areas, the PRG decided that the formation of an SRC is paramount to success and effectively outweighs the other priorities. The need to create the SRC served as "a unifying theme" of the recommendations, the report states. "Implicit in the creation of the SRC is the notion that specialized expertise in sarcoma patient care and/or sarcoma research is required to move the field forward," the report continues. "The intent is not, however, to create a structure that is exclusive. Rather, the SRC represents an organizational umbrella that can accommodate and, indeed, welcomes participation by any investigator or center committed to sarcoma research."

The establishment of the SRC, the report states, would 1) assemble a national, multidisciplinary group of investigators to provide leadership for sarcoma research, 2) establish centers of excellence for sarcoma research, and 3) provide a common infrastructure to support and accelerate research (by establishing a centralized sarcoma and tissue repository, for example).

The PRG was co-chaired by Dr. Karen Antman of the Herbert Irving Comprehensive Cancer Center at Columbia University (see "A Conversation with..."), Dr. Todd Golub of the Dana-Farber Cancer Institute, and Dr. Lee Helman, Chief, Pediatric Oncology Branch, Center for Cancer Research, NCI.

Although sarcomas - cancers that form in connective tissues like blood vessels, bones, and cartilage - account for about 1 percent of adult malignancies, they are more common in children, representing about 15 percent of pediatric malignancies. Because of misdiagnoses, they are probably undercounted. The misdiagnosis problem has been exacerbated by the current diagnostic coding systems, the report states, which categorize cancers by their location in the body, something that does not work well for sarcomas. The report cites, for example, a form of sarcoma called gastrointestinal stromal tumor (GIST), which was once thought to be rare. A recent review of 1,500 intra-abdominal tumors for a GIST genetic marker turned up 400 cases. Of these 400, only 100 cases initially had been diagnosed as GIST, with the remainder diagnosed as other sarcoma subtypes.

In addition to misdiagnoses, improvements in treatment also have been plagued by late diagnoses, a poor understanding of risk factors for sarcomas, and limited effectiveness of currently available therapies. Sarcoma diagnosis, for instance, "is delayed in many patients by the lack of experience of primary physicians, who often attribute the initial mass to common benign lesions," the report explains. There is also no uniform standard of care for sarcomas, which has yielded "wide variations" both in treatment and outcomes.

Other priority areas identified by the PRG include:

  • Funding and fostering research in specific areas of sarcoma biology that are most likely to yield needed advances or where there are significant knowledge gaps, such as the developmental biology of mesenchymal tissues.
  • Developing sarcoma-specific animal model systems.
  • Funding and fostering comprehensive approaches to sarcoma profiling and target discovery.
  • Developing a centrally available toolkit of core reagents and access to important technology platforms (e.g., cell lines, biomarkers).
  • More strategically designed clinical trials.

As with past PRGs (11 have been convened over the past 7 years), the Sarcoma PRG solicited extensive input from the research and advocacy communities to examine NCI's research portfolio as well as research funded by other institutes and organizations. NCI will now review the recommendations - available at - and, with guidance from internal working groups, implement initiatives that respond to them.

Additional information about and reports from each of the PRGs may be found at