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

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.