Summary






 

Key Recommendations:

· A national tumor repository matched to a clinical data bank with an appropriate working infrastructure will be essential for the discovery and validation of new expressed and activated oncoprotein targets in soft tissue sarcomas. Studies using this resource should combine high-throughput analyses with directed molecular evaluations to identify promising targets for clinical study and intervention. Analyses would include gene expression, protein expression/activation, and exploration of cellular pathways important to oncogenesis, growth and metastasis in this diverse group of malignancies.

· There is an urgent need for a common, complete and uniform reporting of sarcoma pathology. This includes reporting histologic type, grade, size, depth, margins, and any ancillary studies performed to evaluate clinically important molecular markers. There should be a system developed for the standardization of molecular diagnostics of soft tissue sarcoma, with a set of minimum quality assurance requirements for consistency and validation.

· Accurate pathologic diagnosis and classification of the different subtypes of soft tissue sarcomas is critical to the molecular characterization and evaluation of targeted therapeutics in the context of histology-specific clinical trials. Consideration should be made for these tumors to be evaluated by a reference panel of experienced pathologists, and for patients with these rare diseases to be referred to sarcoma centers of excellence for second opinions regarding optimal therapy as well as the opportunity to participate in clinical trials.

· The accomplishment of the characterization and targeting of c-kit with imatinib in Gastrointestinal Stromal Tumors (GIST) is a model for successful molecular therapeutic intervention in patients with soft tissue sarcomas. Lessons learned from the GIST/imatinib experience should continue to guide the development of targeted systemic interventions, not only in other histologic subtypes of sarcoma but other solid tumors as well. Both appropriately chosen new targeted agents as well as combinations of targeted therapies should be explored in well-designed clinical trials for patients with these diseases. Collaborations with pharmaceutical companies should be explored and facilitated to further take advantage of this model of achievement.

· Intensity Modulated Radiotherapy (IMRT) is a promising technique for the delivery of radiation therapy that should be actively studied in well-controlled trials of patients with soft tissue sarcomas.

· In patients with retroperitoneal soft-tissue sarcomas, local recurrence is the major cause of mortality. A national trial evaluating the role of radiotherapy to enhance local control and survival is warranted and should be planned and carried out.