Snapshot A 45-year-old woman presents to the emergency room from her primary care physician’s office for high blood pressure unresponsive to therapy. She has a history of neurofibromatosis type 1, though without any neurological deficits. She has multiple café-au-lait spots on her body. She is found to be hypertensive to 154/121 mmHg. Her 24-hour urine metanephrines and VMA come back elevated. Her abdominal CT comes back with an adrenal mass. Introduction Nonmalignant tumor of adrenal medulla most common adrenal tumor in adults vs neuroblastoma, which is most common in children secretes catecholamines causing episodic hypertension Rule of 10’s 10% malignant 10% bilateral 10% extra-adrenal bladder and organ of Zuckerkandl (bifurcation of aorta) 10% calcify 10% kids Epidemiology 40-50 year old ETIOLOGY Pathogenesis chromaffin cells from neural crest origin 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 therapy-resistant hypertension Physical exam ↑ BP (diastolic) skin hyperpigmentation (café-au-lait spots) may suggest NF1 Imaging Adrenal mass seen on MRI or CT MIBG scanning nuclear isotope scan to detect extra-adrenal involvement STUDIES Best initial test ↑ free metanephrine level in plasma Confirmation with 24-hour urine collection ↑ vanillyl mandelic acid (breakdown of norepinephrine and epinephrine) ↑ metanephrines (more sensitive than VMA) Histology chromaffin cells with enlarged dysmorphic nuclei Differential Diagnosis Functional adrenal tumors various different hormonal syndromes can result, such as Cushing syndrome secondary to unregulated cortisol secretion Conn syndrome secondary to unregulated aldosterone secretion male feminization secondary to unregulated estrogen secretion female virilization secondary to unregulated androgen secretion e.g., androstenedione, DHEA, and testosterone levels 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 Complications Metastases bones, lungs, and liver Transformation to malignant tumor (10%) Hypertensive crisis Prognosis Very good with resection