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Response of Carney's triad‐related metastatic gastrointestinal stromal tumor to sunitinib
Author(s) -
PRANAVAN Ganesalingam,
GOLDSTEIN David,
YIP Desmond
Publication year - 2008
Publication title -
asia‐pacific journal of clinical oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 29
eISSN - 1743-7563
pISSN - 1743-7555
DOI - 10.1111/j.1743-7563.2008.00182.x
Subject(s) - sunitinib , medicine , gist , paraganglioma , imatinib mesylate , stromal tumor , tyrosine kinase inhibitor , targeted therapy , oncology , pathology , stromal cell , imatinib , gastroenterology , cancer , myeloid leukemia
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 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.