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Pheochromocytoma and paraganglioma: Diagnosis, genetics, management, and treatment

2014; Elsevier BV; Volume: 38; Issue: 1 Linguagem: Inglês

10.1016/j.currproblcancer.2014.01.001

1535-6345

Victoria Martucci, Karel Pacák,

Hormonal Regulation and Hypertension

Paragangliomas are neuroendocrine tumours of the sympathetic and parasympathetic nervous systems originating from neural crest cells. Less than 1% of paragangliomas in the heart originate from intrinsic cardiac ganglia cells in the posterior wall of the atria, atrioventricular groove, and along the root of the great vessels. We describe the tumour characteristics, patient demographics, presentation, means of diagnosis, pathology correlation, management, and outcome in 11 patients with intrapericardial paragangliomas. To the authors' knowledge, this is the largest case series reported, with emphasis on multimodality imaging findings.