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dc.contributor.authorFarag, S
dc.contributor.authorSomaiah, N
dc.contributor.authorChoi, H
dc.contributor.authorHeeres, B
dc.contributor.authorWang, W-L
dc.contributor.authorvan Boven, H
dc.contributor.authorNederlof, P
dc.contributor.authorBenjamin, R
dc.contributor.authorvan der Graaf, W
dc.contributor.authorGrunhagen, D
dc.contributor.authorBoonstra, PA
dc.contributor.authorReyners, AKL
dc.contributor.authorGelderblom, H
dc.contributor.authorSteeghs, N
dc.date.accessioned2017-04-21T16:52:59Z
dc.date.issued2017-05
dc.identifier.citationEuropean journal of cancer (Oxford, England : 1990), 2017, 76 pp. 76 - 83
dc.identifier.issn0959-8049
dc.identifier.urihttps://repository.icr.ac.uk/handle/internal/616
dc.identifier.eissn1879-0852
dc.identifier.doi10.1016/j.ejca.2017.02.007
dc.description.abstractPurpose Patients, platelet-derived growth factor receptor alpha (PDGFRA) D842V-mutated gastrointestinal stromal tumours (GISTs) are known for their insensitivity to imatinib. However, in clinical practice responses have been observed in some patients. We describe the natural history and treatment outcomes in a cohort of PDGFRA exon 18 mutated GIST patients.Patients and methods A retrospective cohort study was conducted in PDGFRA exon 18 mutation GIST patients treated in six expert centres in the Netherlands and the United States. Two independent radiologists assessed radiological response to imatinib according to Choi's criteria in all patients with measurable disease treated with imatinib in neo-adjuvant or palliative intent.Results Seventy-one patients with PDGFRA exon 18 mutation were identified of whom 48 patients (69%) had a D842V mutation. Twenty-two (45.8%) D842V-mutated GIST patients received imatinib treatment, 16 had measurable disease. Fourteen out of the 23 (60.9%) patients with non-D842V mutations received imatinib treatment, eight had measurable disease. Two out of 16 (12.5%) D842V-mutated GIST patients had partial response, 3 patients (18.8%) had stable disease and 9 patients (56.3%) had progressive disease as best response. Two patients did not have follow-up computed tomography scans to assess response. Six out of 8 (75%) patients with non-D842V exon 18 mutations had partial response and two (25%) had stable disease as best response.Conclusion Patients with D842V-mutated GISTs can occasionally respond to imatinib. In the absence of better therapeutic options, imatinib should therefore not be universally withheld in patients with this mutation.
dc.formatPrint-Electronic
dc.format.extent76 - 83
dc.languageeng
dc.language.isoeng
dc.rights.urihttps://www.rioxx.net/licenses/all-rights-reserved
dc.subjectHumans
dc.subjectEsophageal Neoplasms
dc.subjectStomach Neoplasms
dc.subjectGastrointestinal Stromal Tumors
dc.subjectReceptor, Platelet-Derived Growth Factor alpha
dc.subjectAntineoplastic Agents
dc.subjectPrognosis
dc.subjectTreatment Outcome
dc.subjectChemotherapy, Adjuvant
dc.subjectNeoadjuvant Therapy
dc.subjectPalliative Care
dc.subjectDigestive System Surgical Procedures
dc.subjectRetrospective Studies
dc.subjectCohort Studies
dc.subjectMutation
dc.subjectAdult
dc.subjectAged
dc.subjectAged, 80 and over
dc.subjectMiddle Aged
dc.subjectUnited States
dc.subjectNetherlands
dc.subjectFemale
dc.subjectMale
dc.subjectYoung Adult
dc.subjectImatinib Mesylate
dc.titleClinical characteristics and treatment outcome in a large multicentre observational cohort of PDGFRA exon 18 mutated gastrointestinal stromal tumour patients.
dc.typeJournal Article
dcterms.dateAccepted2017-02-05
rioxxterms.versionofrecord10.1016/j.ejca.2017.02.007
rioxxterms.licenseref.urihttps://www.rioxx.net/licenses/all-rights-reserved
rioxxterms.licenseref.startdate2017-05
rioxxterms.typeJournal Article/Review
dc.relation.isPartOfEuropean journal of cancer (Oxford, England : 1990)
pubs.notesNot known
pubs.organisational-group/ICR
pubs.organisational-group/ICR/Primary Group
pubs.organisational-group/ICR/Primary Group/ICR Divisions
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies/Clinical and Translational Sarcoma
pubs.organisational-group/ICR
pubs.organisational-group/ICR/Primary Group
pubs.organisational-group/ICR/Primary Group/ICR Divisions
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies
pubs.organisational-group/ICR/Primary Group/ICR Divisions/Clinical Studies/Clinical and Translational Sarcoma
pubs.publication-statusPublished
pubs.volume76
pubs.embargo.termsNot known
icr.researchteamClinical and Translational Sarcomaen_US
dc.contributor.icrauthorvan der Graaf, Wilhelmina


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