Overview
Neoplasms of the adrenal medulla include pheochromocytomas and paragangliomas, which are rare tumors arising from chromaffin cells. These tumors are characterized by the excessive production and secretion of catecholamines, leading to a variety of clinical manifestations.Diagnosis
Measurement of plasma or 24-hour urinary fractionated metanephrines and catecholamines is the primary diagnostic test for pheochromocytoma and paraganglioma 1.
Imaging studies such as CT or MRI of the abdomen and pelvis are used to localize the tumor 1.
123I-metaiodobenzylguanidine (MIBG) scintigraphy can be useful for detecting metastatic or extra-adrenal disease 1.Management
Surgical resection is the definitive treatment for pheochromocytoma and paraganglioma 1.
Preoperative blockade of alpha-adrenergic receptors with phenoxybenzamine or prazosin is essential to prevent hypertensive crisis during surgery 1.
Beta-adrenergic blockade is typically initiated after alpha-blockade is established to manage tachycardia 1.
Medical management with tyrosine kinase inhibitors (e.g., sunitinib) or chemotherapy may be considered for unresectable or metastatic disease 1.Special Populations
In pregnant women, diagnosis and management of pheochromocytoma are challenging and require a multidisciplinary approach 1. Alpha-blockade is initiated early in pregnancy, and surgical resection is typically performed in the second trimester 1.Key Recommendations
Measurement of plasma or 24-hour urinary fractionated metanephrines and catecholamines is the primary diagnostic test for pheochromocytoma and paraganglioma 1. (Evidence: Moderate)
Preoperative alpha-adrenergic receptor blockade is mandatory before surgical resection of pheochromocytoma or paraganglioma to prevent intraoperative hypertensive crisis 1. (Evidence: Moderate)
Surgical resection is the definitive treatment for localized pheochromocytoma and paraganglioma 1. (Evidence: Moderate)References
1 Davies B, Massey BH, Lohman TG, Williams BT. Urinary excretion of free noradrenaline and adrenaline in trained and untrained men. British journal of sports medicine 1977. link