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Key Issues in the Clinical Management of Gastrointestinal Stromal Tumors: An Expert Discussion
Authors:Robert G. Maki  Jean‐Yves Blay  George D. Demetri  Jonathan A. Fletcher  Heikki Joensuu  Javier Martín‐Broto  Toshirou Nishida  Peter Reichardt  Patrick Schöffski  Jonathan C. Trent
Affiliation:1. Tisch Cancer Institute, Mount Sinai Medical Center, New York, New York, USA;2. Department of Medical Oncology, Centre Léon Bérard, Lyon, France;3. Dana‐Farber Cancer Institute, Boston, Massachusetts, USA;4. Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts, USA;5. Helsinki University Hospital and University of Helsinki, Helsinki, Finland;6. Servicio de Oncología Médica, Fundación Instituto Valenciano de Oncología, Valencia, Spain;7. National Cancer Center Hospital East, Department of Surgery, Kashiwa, Japan;8. HELIOS Klinikum Berlin‐Buch, Interdisciplinary Oncology, Berlin, Germany;9. General Medical Oncology, Leuven Cancer Institute, University Hospital Gasthuisberg, Catholic University Leuven, Leuven, Belgium;10. University of Miami Sylvester Cancer Center, Sarcoma Medical Oncology Program, Miami, Florida, USA
Abstract:After the revelation of kinase targeting with orally available small molecules,the use of imatinib in chronic myelogenous leukemia and in gastrointestinalstromal tumor (GIST) has now become commonplace and just two of many examples ofthe use of kinase inhibitors in cancer. In this article, we discuss importantpractice points that may impact upon questions of therapy of primary andmetastatic GIST, with the hope that the questions addressed in this rare solidtumor can serve as examples of what can be achieved with kinase-directed therapiesin other cancers. We present cases that highlight some of the key issues in GISTmanagement and afterward discuss both points of consensus and controversial issuesin what is now recognized as one of the most common forms of sarcoma.

Implications for Practice:

The treatment of gastrointestinal stromal tumor (GIST) has become sophisticatedwith the availability of three approved agents in many countries and 15 years ofexperience with primary and metastatic disease. Important lessons fromtyrosine-kinase inhibitors in GIST can be gleaned from this experience and willimpact implementation of similar agents for other cancers.
Keywords:Gastrointestinal stromal tumor  GIST  Imatinib  Sunitinib  Regorafenib  KIT  PDGFRA  SDH  Carney‐Stratakis syndrome
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