A 38-year-old woman from Mumbai presents with episodic severe headaches, profuse diaphoresis, and palpitations for 3 months. Her blood pressure during an episode was 180/110 mmHg, and between episodes it normalizes to 130/80 mmHg. She reports anxiety during attacks and occasional chest pain. Urine metanephrines are elevated at 1.8 times the upper limit of normal. CT abdomen shows a 3 cm left adrenal mass. She has no family history of hypertension or endocrine tumors. What is the most appropriate next step in management?

Explanation

## Pheochromocytoma: Preoperative Preparation Protocol **Key Point:** The cardinal rule of pheochromocytoma management is **alpha-blockade first, then beta-blockade**. Reversing this order or omitting alpha-blockade risks hypertensive crisis and arrhythmias. ### Why This Patient Needs Preoperative Blockade This 38-year-old has: - Classic triad: episodic headache, diaphoresis, palpitations - Paroxysmal hypertension (180/110 mmHg during attacks, normotensive between) - Biochemical confirmation: elevated urine metanephrines (1.8× ULN) - Imaging: 3 cm left adrenal mass (consistent with pheochromocytoma) ### Preoperative Preparation Sequence 1. **Alpha-blockade (10–14 days):** Phenoxybenzamine 10 mg daily, titrate to 20–40 mg BD - Prevents catecholamine-induced vasoconstriction and hypertensive surge during tumor manipulation - Allows peripheral vasodilation and blood volume expansion - Reduces perioperative morbidity and mortality 2. **Beta-blockade (after alpha-blockade established):** Propranolol 20–40 mg TDS or atenolol 50–100 mg daily - NEVER start beta-blockers first (unopposed alpha-adrenergic effects → hypertensive crisis) - Controls tachycardia and arrhythmias 3. **Surgical resection:** After 10–14 days of dual blockade **High-Yield:** The mnemonic **"Alpha before Beta"** is the golden rule. Phentolamine is a short-acting agent used only for acute hypertensive crisis *during* surgery, not preoperative preparation. ### Why Genetic Testing Is Not First-Line Here Genetic counseling and testing (RET, VHL, NF1) are important for syndromic pheochromocytoma (MEN 2, VHL disease, neurofibromatosis), but they do NOT change immediate preoperative management. Testing is done *after* biochemical and imaging confirmation, and does not delay surgery. **Clinical Pearl:** ~30–40% of pheochromocytomas are hereditary. Genetic testing is indicated in all patients <50 years or with family history, but it is a *parallel* workup, not a prerequisite for surgery. ### Clonidine Is Contraindicated Alpha-2 agonists (clonidine) suppress catecholamine release but cause rebound hypertension on withdrawal and are not standard therapy for pheochromocytoma. [cite:Harrison 21e Ch 405]