A practical approach to children with phaeochromocytomas and paragangliomas
AbstractPhaeochromocytoma (PCC) and paraganglioma (PGL) are rare chromaffin cell tumours which secrete catecholamines and form part of the family of neuroendocrine tumours. They are responsible for 0.5–2% of cases of secondary hypertension in paediatrics and although rare, are potentially lethal. The presentation of hypertension in these patients is characteristic, and the treatment is definitive.1 Phaeochromocytoma and paragangliomas (PPGLs) have a reported annual incidence of two to five cases per million, of which only 10% occur in children.2
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