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Response of Carney's triad-related metastatic gastrointestinal stromal tumor to sunitinib

PRANAVAN, Ganesalingam; Goldstein, David; Yip, Desmond

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Background: Metastatic Carney's triad is a rare syndrome of gastrointestinal stromal tumour, paraganglioma and pulmonary chondroma associated with unique clinical behavior and relative resistance to imatinib Case: A 50-year-old woman was diagnosed with metastatic gastrointestinal stromal tumour, after having complete remission for almost three decades following gastrectomy for her original gastric tumour. She was c-kit-wild type and developed various paragangliomas and possible pulmonary...[Show more]

dc.contributor.authorPRANAVAN, Ganesalingam
dc.contributor.authorGoldstein, David
dc.contributor.authorYip, Desmond
dc.date.accessioned2015-12-08T22:29:24Z
dc.identifier.issn1743-7563
dc.identifier.urihttp://hdl.handle.net/1885/34084
dc.description.abstractBackground: Metastatic Carney's triad is a rare syndrome of gastrointestinal stromal tumour, paraganglioma and pulmonary chondroma associated with unique clinical behavior and relative resistance to imatinib Case: A 50-year-old woman was diagnosed with metastatic gastrointestinal stromal tumour, after having complete remission for almost three decades following gastrectomy for her original gastric tumour. She was c-kit-wild type and developed various paragangliomas and possible pulmonary chondromas during the course of her disease. Treatment with the tyrosine kinase inhibitor imatinib mesylate and subsequently the multikinase inhibitor sunitinib were associated with periods of radiological response and clinical benefit before the patient died of progressive disease. Conclusion: Identification of patients with Carney's triad tumours is important in clinical practice as they have distinct clinical behavior, mutational status and relative lack of responsiveness to the current available targeted therapy compared to the typical c-kit positive gastrointestinal stromal tumours.
dc.publisherBlackwell Publishing Ltd
dc.sourceAsia-Pacific Journal of Clinical Oncology
dc.subjectKeywords: catecholamine; CD34 antigen; chromogranin; imatinib; iron; stem cell factor receptor; su 11247; sunitinib; synaptophysin; unclassified drug; adult; article; cancer regression; Carney complex; carotid body tumor; case report; chondroma; death; disease cour Gastrointestinal stromal tumor; Paraganglioma; Proteintyrosine kinase; Proto-oncogene proteins c-kit
dc.titleResponse of Carney's triad-related metastatic gastrointestinal stromal tumor to sunitinib
dc.typeJournal article
local.description.notesImported from ARIES
local.identifier.citationvolume4
dc.date.issued2008
local.identifier.absfor111299 - Oncology and Carcinogenesis not elsewhere classified
local.identifier.ariespublicationu4241283xPUB109
local.type.statusPublished Version
local.contributor.affiliationPRANAVAN, Ganesalingam, The Canberra Hospital
local.contributor.affiliationGoldstein, David, University of New South Wales
local.contributor.affiliationYip, Desmond, College of Medicine, Biology and Environment, ANU
local.description.embargo2037-12-31
local.bibliographicCitation.startpage170
local.bibliographicCitation.lastpage174
local.identifier.doi10.1111/j.1743-7563.2008.00182.x
dc.date.updated2015-12-08T09:21:48Z
local.identifier.scopusID2-s2.0-51349144121
CollectionsANU Research Publications

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