Other authors

Institut Català de la Salut

[Serrano C] Sarcoma Translational Research Group, Vall d’Hebron Institute of Oncology (VHIO), Barcelona, Spain. Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Martín-Broto J] Medical Oncology Department, Fundación Jimenez Diaz University Hospital, Madrid, Spain University Hospital General de Villalba, Madrid, Spain Instituto de investigación Sanitaria Fundación Jimenez Diaz (IIS/FJD; UAM), Madrid, Spain. [Asencio-Pascual JM] Department of General Surgery, Gregorio Marañón University Hospital, Madrid, Spain Department of Surgery, Universidad Complutense de Madrid, Madrid, Spain. [López-Guerrero JA] Laboratory of Molecular Biology, Fundación Instituto Valenciano de Oncología, Valencia, Spain. [Rubió-Casadevall J] Department of Medical Oncology, Catalan Institute of Oncology, Girona Biomedical Research Institute (IDIBGI), Girona, Spain. [Bagué S] Department of Pathology, Santa Creu i Sant Pau University Hospital, Barcelona, Spain

Vall d'Hebron Barcelona Hospital Campus

Publication date

2023-09-19T08:54:00Z

2023-09-19T08:54:00Z

2023



Abstract

Avapritinib; Gastrointestinal stromal tumor; Surgery


Avapritinib; Tumor estromal gastrointestinal; Cirurgia


Avapritinib; Tumor estromal gastrointestinal; Cirugía


Gastrointestinal stromal tumor (GIST) is the most common malignant neoplasm of mesenchymal origin. GIST spans a wide clinical spectrum that ranges from tumors with essentially no metastatic potential to malignant and life-threatening spread diseases. Gain-of-function mutations in KIT or PDGFRA receptor tyrosine kinases are the crucial drivers of most GISTs, responsible for tumor initiation and evolution throughout the entire course of the disease. The introduction of tyrosine kinase inhibitors targeting these receptors has substantially improved the outcomes in this formerly chemoresistant cancer. As of today, five agents hold regulatory approval for the treatment of GIST: imatinib, sunitinib, regorafenib, ripretinib, and avapritinib. This, in turn, represents a success for a rare neoplasm. During the past two decades, GIST has become a paradigmatic model in cancer for multidisciplinary work, given the disease-specific particularities regarding tumor biology and tumor evolution. Herein, we review currently available evidence for the management of GIST. This clinical practice guideline has been developed by a multidisciplinary expert panel (oncologist, pathologist, surgeon, molecular biologist, radiologist, and representative of patients’ advocacy groups) from the Spanish Group for Sarcoma Research, and it is conceived to provide, from a critical perspective, the standard approach for diagnosis, treatment, and follow-up.


This work was supported in part by AECC (CLSEN20004SERR) and the Fero Foundation, both to CS.

Document Type

Article


Published version

Language

English

Publisher

SAGE Publications

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Attribution-NonCommercial 4.0 International

http://creativecommons.org/licenses/by-nc/4.0/

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