Bruno Landi1, Jean-Yves Blay2, Sylvie Bonvalot3, Mathilde Brasseur4, Jean Michel Coindre5, Jean François Emile6, Vincent Hautefeuille7, Charles Honore8, Eric Lartigau9, Georges Mantion10, Marc Pracht11, Axel Le Cesne12, Michel Ducreux12, Olivier Bouche4. 1. Departments of Hepatogastroenterology and Digestive Oncology, European Georges Pompidou Hospital, Paris, France. Electronic address: bruno.landi@aphp.fr. 2. Departments of Medical Oncology, Léon Bérard Center, Lyon, France. 3. Departments of Surgery, Curie Institute, Paris, France. 4. Departments of Hepatogastroenterology and Digestive Oncology, CHU Robert Debré Hospital, Reims, France. 5. Departments of Pathology, Institut Bergonié, Bordeaux, France. 6. Departments of Pathology, Hôpital Ambroise-Paré, Boulogne-Billancourt, France. 7. Departments of Hepatogastroenterology and Digestive Oncology, CHU Amiens Picardie, Amiens, France. 8. Departments of Surgery, Gustave Roussy Cancer Campus, Villejuif, France. 9. Departments of Radiotherapy, Oscar Lambret Center, Lille, France. 10. Departments of Surgery, CHU Jean Minjoz, Besançon, France. 11. Departments of Medical Oncology, Eugène Marquis Center, Rennes, France. 12. Departments of Medical Oncology, Gustave Roussy Cancer Campus, Villejuif, France.
Abstract
BACKGROUND: This document is a summary of the French Intergroup guidelines regarding the management of gastrointestinal stromal tumours (GISTs) updated in December 2018. DESIGN: This collaborative work summarizes clinical practice recommendations (guidelines) on the management of GISTs. It is based on recent literature review, ESMO recommendations and expert opinions. RESULTS: The diagnosis of GIST is based on histological examination and immunohistochemistry with markers KIT and DOG-1. Each case must be discussed within a multidisciplinary team. Complete surgical resection tumour, avoiding peroperative perforation, is the potentially curative treatment of localized GISTs. The estimation of the recurrence risk is essential, or adjuvant treatment,and follow-up adaptation. Genotyping (KIT and PDGFRA) of all but very low-risk GISTs is recommended. The nature of mutation has a prognostic value and predictive influence on drug efficacy. Imatinib, a tyrosine-kinase inhibitor, is the standard adjuvant treatment after R0 resection of a GIST with a high risk of recurrence, and the first line therapy for advanced GISTs. Suninitib and regorafenib are respectively the second- and third-line standard treatments for advanced GISTs. CONCLUSION: Guidelines for management of GISTs are continuously evolving and need to be regularly updated. This constant progress is made possible through clinical and translational research.
BACKGROUND: This document is a summary of the French Intergroup guidelines regarding the management of gastrointestinal stromal tumours (GISTs) updated in December 2018. DESIGN: This collaborative work summarizes clinical practice recommendations (guidelines) on the management of GISTs. It is based on recent literature review, ESMO recommendations and expert opinions. RESULTS: The diagnosis of GIST is based on histological examination and immunohistochemistry with markers KIT and DOG-1. Each case must be discussed within a multidisciplinary team. Complete surgical resection tumour, avoiding peroperative perforation, is the potentially curative treatment of localized GISTs. The estimation of the recurrence risk is essential, or adjuvant treatment,and follow-up adaptation. Genotyping (KIT and PDGFRA) of all but very low-risk GISTs is recommended. The nature of mutation has a prognostic value and predictive influence on drug efficacy. Imatinib, a tyrosine-kinase inhibitor, is the standard adjuvant treatment after R0 resection of a GIST with a high risk of recurrence, and the first line therapy for advanced GISTs. Suninitib and regorafenib are respectively the second- and third-line standard treatments for advanced GISTs. CONCLUSION: Guidelines for management of GISTs are continuously evolving and need to be regularly updated. This constant progress is made possible through clinical and translational research.