12/09/2025
❇️ Pheochromocytoma Points to Remember:
→ Tumor of chromaffin cells (neuroendocrine cells) of the adrenal medulla
→ Secretes catecholamines (mostly norepinephrine, sometimes epinephrine, rarely dopamine)
▶️ Pheochromocytoma Rule of 10s
→ 10% bilateral
→ 10% extra-adrenal (paragangliomas)
→ 10% malignant
→ 10% pediatric
→ 10% familial (but actually up to 40% are hereditary)
✔️ Associated Syndromes (Autosomal Dominant)
→ MEN 2A & 2B
→ Von Hippel-Lindau (VHL)
→ Neurofibromatosis type 1 (NF1)
✔️ Clinical Presentation
🛑 Classic triad
→ Episodic headache
→ Sweating (diaphoresis)
→ Palpitations/tachycardia
🩺 Other signs
→ Hypertension (sustained or paroxysmal)
→ Anxiety, tremors, weight loss
→ Orthostatic hypotension (due to volume depletion)
→ Hyperglycemia (catecholamines inhibit insulin)
✔️ Diagnosis
Initial test (high-yield):
→ Plasma free metanephrines or 24-hr urinary catecholamines/metanephrines
Confirmatory test:
→ Clonidine suppression test (in special cases)
Imaging:
→ CT/MRI of abdomen (for adrenal mass)
→ MIBG scan if extra-adrenal or metastatic
✔️ Treatment
Preoperative medical management:
→ α-blocker first: Phenoxybenzamine (non-selective, irreversible)
→ Then β-blocker: Propranolol or atenolol (to control tachycardia)
❗ Never give β-blocker first → unopposed α-vasoconstriction → hypertensive crisis
Definitive:
→ Surgical resection
✔️ Complications
→ Hypertensive crisis
→ Arrhythmias
→ Cardiomyopathy (catecholamine-induced)