title: Gastrointestinal stromal tumour.
abstract: Gastrointestinal stromal tumours (GISTs) are mesenchymal neoplasms that arise in the gastrointestinal tract, usually in the stomach or the small intestine and rarely elsewhere in the abdomen. They can occur at any age, the median age being 60-65 years, and typically cause bleeding, anaemia, and pain. GISTs have variable malignant potential, ranging from small lesions with a benign behaviour to fatal sarcomas. Most tumours stain positively for the mast/stem cell growth factor receptor KIT and anoctamin 1 and harbour a kinase-activating mutation in either KIT or PDGFRA. Tumours without such mutations could have alterations in genes of the succinate dehydrogenase complex or in BRAF, or rarely RAS family genes. About 60% of patients are cured by surgery. Adjuvant treatment with imatinib is recommended for patients with a substantial risk of recurrence, if the tumour has an imatinib-sensitive mutation. Tyrosine kinase inhibitors substantially improve survival in advanced disease, but secondary drug resistance is common.
authors.affiliation: Department of Oncology, Helsinki University Central Hospital, Helsinki, Finland. heikki.joensuu@hus.fi
authors.forename: Heikki
authors.initials: H
authors.lastname: Joensuu
authors.forename: Peter
authors.initials: P
authors.lastname: Hohenberger
authors.forename: Christopher L
authors.initials: CL
authors.lastname: Corless
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides / Q000627:therapeutic use; D002812:Chondroma / Q000235:genetics; D005770:Gastrointestinal Neoplasms / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D046152:Gastrointestinal Stromal Tumors / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D006801:Humans; D000068877:Imatinib Mesylate; D007890:Leiomyosarcoma / Q000235:genetics; D008175:Lung Neoplasms / Q000235:genetics; D009154:Mutation / Q000235:genetics; D009456:Neurofibromatosis 1 / Q000235:genetics; D010236:Paraganglioma, Extra-Adrenal / Q000235:genetics; D010879:Piperazines / Q000627:therapeutic use; D011300:Preoperative Care; D019009:Proto-Oncogene Proteins c-kit / Q000235:genetics; D011743:Pyrimidines / Q000627:therapeutic use; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics; D013274:Stomach Neoplasms / Q000235:genetics
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D019009:Proto-Oncogene Proteins c-kit; D020796:Receptor, Platelet-Derived Growth Factor alpha