Snapshot A 40-year-old gentleman presents with episodic headaches and palpitations. Attributing it to his tendency to worry excessively, he put it off for several months until he began to sweat episodically too. In the clinic, his blood pressure is found to be elevated at 160/120 mmHg. Plasma metanephrines are elevated and an abdominal CT scan reveals an adrenal mass. Introduction Nonmalignant tumor of adrenal medulla most common adrenal tumor in adults (vs neuroblastoma, most common in children) composed of chromaffin cells that are normally stimulated by acetylcholine secretes catecholamines causing episodic hypertension Pathogenesis from chromaffin cells of neural crest origin Epidemiology 40-50 years old Rule of 10’s 10% malignant 10% bilateral 10% kids 10% extra-adrenal bladder, organ of Zuckerkandl (bifurcation of aorta) 10% calcify Associated conditions von Hippel-Lindau disease MEN 2A MEN 2B neurofibromatosis type 1 Presentation Symptoms are episodic 5 P’s Pressure (↑ BP) Pain (headache) Perspiration Palpitations (tachycardia) Pallor mediated by tumor secretion of epinephrine, norepinephrine, and dopamine Physical exam ↑ BP (diastolic and systolic increase due to alpha adrenergic stimulation resulting in vasoconstriction) Evaluation Serology ↑ free metanephrine level 24-hour urine collection for diagnostic confirmation ↑ vanillyl mandelic acid (breakdown of norepinephrine and epinephrine) ↑ metanephrines (more sensitive than VMA) Histology chromaffin cells with enlarged dysmorphic nuclei Differential Diagnosis Hyperthyroidism Carcinoid tumors Panic disorder Therapy resistant hypertension from obstructive sleep apnea Treatment In this sequence of events α-antagonist phenoxybenzamine to prevent unopposed α-action if β receptors are blocked β-blocker tumor resection Prognosis, Prevention, and Complications Prognosis very good with resection Complications metastases bones, lungs, and liver