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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.
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