演題抄録

臓器別シンポジウム

開催概要
開催回
第51回・2013年・京都
 

Novel treatment strategies for musculoskeletal tumors

演題番号 : OS23-1

[筆頭演者]
Shreyaskumar R. Patel:1 

1:UT MD Anderson Cancer Center, USA

 

The field of Sarcoma has been undergoing a paradigm shift over the last few years with increasing recognition of the heterogeneity of the group of diseases it represents. There is now fairly uniform agreement that therapeutic management should to be individualized and histology specific, as opposed to lumping all sarcomas as one entity. The classic example was set forth by the identification of an activating mutation in the KIT or PDGFRA gene in patients with GIST, thus separating them from leiomyosarcomas and allowing for the advent of targeted therapies Imatinib, Sunitinib and Regorafenib, which have clearly helped extend the survival of these patients. With this example serving as an impetus, several targeted therapies have been tested in a variety of different histologies resulting in a few success stories, e.g. Imatinib in DFSP, desmoids, GCT of tendon sheath and chordomas; antiangiogenic agents for ASPS, SFT and (may be) angiosarcomas; and Denosumab in GCT of bone. Several other classes of targeted agents have shown some signal of activity, which needs to be defined further e.g. IGF1r antibodies and PARP inhibitors in Ewings; mTOR inhibitors in MPNSTs and PECOmas/ lymphangioleiomyomatosis; and death receptor agonists/proapoptic agents in synovial sarcomas and chondrosarcomas. As the knowledge base about potential targets in different histologic subsets keeps growing, there are several leads from the lab that need to be tested in the clinic, e.g. c-met inhibitors in clear cell sarcomas, CDK/MDM2 inhibitors in dedifferentiated liposarcomas, Src-inhibitors in osteosarcomas, Hedge-hog inhibitors and IDH inhibitors in chondrosarcomas etc. Lastly, as next generation sequencing becomes easily accessible, personalizing therapy for a given patient based on tumor testing and matching therapy represents the way of the future. In summary, the future of systemic therapy of sarcomas looks promising and we need to enroll patients on clinical trials to speed up the process.

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