GEIS Guidelines for Gastrointestinal Sarcomas (GIST)

Abstract Gastrointestinal stromal sarcomas (GISTs) are the most common mesenchymal tumours originating in the digestive tract. They have a characteristic morphology, are generally positive for CD117 (c-kit) and are primarily caused by activating mutations in the KIT or PDGFRA genes (1) . On rare occ...

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Bibliographic Details
Published in:Cancer treatment reviews 2017-04, Vol.55, p.107-119
Main Authors: Poveda, Andrés, García del Muro, Xavier, Antonio López-Guerrero, Jose, Cubedo, Ricardo, Martínez, Virginia, Romero, Ignacio, Serrano, César, Valverde, Claudia, Martín-Broto, Javier
Format: Article
Language:English
Subjects:
Antineoplastic Agents - therapeutic use
CD117
Combined Modality Therapy
DOG1
Gastrointestinal Neoplasms - diagnosis
Gastrointestinal Neoplasms - pathology
Gastrointestinal Neoplasms - surgery
Gastrointestinal Neoplasms - therapy
Gastrointestinal Stromal Tumors - diagnosis
Gastrointestinal Stromal Tumors - secondary
Gastrointestinal Stromal Tumors - surgery
Gastrointestinal Stromal Tumors - therapy
GEIS
GIST
Hematology, Oncology and Palliative Medicine
Humans
Imatinib
Imatinib Mesylate - therapeutic use
Indoles - therapeutic use
Molecular Targeted Therapy
PDGFRA
Phenylurea Compounds - therapeutic use
Proto-Oncogene Proteins c-kit - genetics
Pyridines - therapeutic use
Pyrroles - therapeutic use
Regorafenib
Sunitinib
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Summary:Abstract Gastrointestinal stromal sarcomas (GISTs) are the most common mesenchymal tumours originating in the digestive tract. They have a characteristic morphology, are generally positive for CD117 (c-kit) and are primarily caused by activating mutations in the KIT or PDGFRA genes (1) . On rare occasions, they occur in extravisceral locations such as the omentum, mesentery, pelvis and retroperitoneum. GISTs have become a model of multidisciplinary work in oncology: the participation of several specialties (oncologists, pathologists, surgeons, molecular biologists, radiologists...) has forested advances in the understanding of this tumour and the consolidation of a targeted therapy, imatinib, as the first effective molecular treatment in solid tumours. Following its introduction, median survival of patients with advanced or metastatic GIST increased from 18 to more than 60 months. Sunitinib and Regorafenib are two targeted agents with worldwide approval for second- and third-line treatment, respectively, in metastatic GIST.
ISSN:0305-7372
1532-1967
DOI:10.1016/j.ctrv.2016.11.011