Patient Presentation
A 45-year-old man presents with recurrent episodes of severe headaches, palpitations, and diaphoresis. He describes episodes of high blood pressure that come and go, and he was recently diagnosed with hypertension that has been difficult to control on standard agents.
Between episodes his physical examination is unremarkable and he appears well. The paroxysmal nature of his symptoms combined with recalcitrant hypertension raises concern for a catecholamine-secreting tumor.
He has no prior surgical history. There is no documented family history of endocrine tumors at this time, and no adrenal mass has yet been identified.
What You'll Be Asked — and What a Strong Resident Discusses
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What is your differential diagnosis for this patient with paroxysmal headache, palpitations, sweating, and labile hypertension?
Expected answer
The classic triad of episodic headache, palpitations, and diaphoresis with hypertension strongly suggests pheochromocytoma. Differential includes essential/secondary hypertension, hyperthyroidism, carcinoid syndrome, panic/anxiety disorder, hypoglycemia, drug effects (stimulants, cocaine, MAOI interactions), renovascular hypertension, and hyperaldosteronism. The paroxysmal hyperadrenergic spells plus recalcitrant hypertension make pheochromocytoma the leading concern and mandate biochemical screening.
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How would you confirm the diagnosis biochemically?
Expected answer
Biochemical confirmation before any imaging. The most sensitive and specific test is 24-hour urine collection of fractionated metanephrines and catecholamines, including dopamine, with creatinine to confirm collection adequacy. Plasma free metanephrines are a useful screening test (higher false-positive rate) and are preferred in children or when reliable urine collection is difficult. I would account for confounders—acute illness, physical/emotional stress, and interfering medications (tricyclics, some antidepressants, sympathomimetics)—that can elevate levels.
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Once you have a positive biochemical diagnosis, how do you localize the tumor?
Expected answer
CT of the abdomen with and without IV contrast is first-line for localization. Pheochromocytomas are typically >3 cm, inhomogeneous with smooth edges and marked contrast enhancement, distinguishing them from lipid-rich adenomas. MRI is used when radiation should be avoided (pregnancy) and better characterizes local invasion. I-123 MIBG is useful when abdominal imaging is negative, when metastatic disease is suspected, or to characterize paragangliomas; FDG-PET helps localize SDH-mutation-associated primary and metastatic tumors. I would specifically review imaging for concerning features: local invasion, enhancing lymphadenopathy, and extra-adrenal disease.
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Would you biopsy the adrenal mass to confirm the diagnosis?
Expected answer
No—never biopsy a suspected pheochromocytoma. Biopsy and adrenal vein sampling are contraindicated because they can precipitate a life-threatening hyperadrenergic (hypertensive) crisis. The diagnosis is made biochemically and by imaging characteristics, not by needle sampling.
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Describe your preoperative preparation. Why is it essential?
Expected answer
Adequate sympathetic blockade is essential to prevent intraoperative labile hypertension and cardiovascular collapse. Start alpha-blockade (e.g., phenoxybenzamine or a selective alpha-1 blocker) typically 10–14 days before surgery and titrate until the patient becomes mildly orthostatic, indicating adequate blockade. Add a beta-blocker only AFTER alpha-blockade if reflex/rebound tachycardia occurs. Encourage a high-sodium diet and generous fluid intake to expand the contracted intravascular volume. Goals are controlled BP, resolution of spells, and volume repletion before proceeding.
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Why must alpha-blockade always precede beta-blockade?
Expected answer
Starting a beta-blocker first blocks beta-2-mediated vasodilation while leaving alpha-mediated vasoconstriction unopposed, causing a paradoxical hypertensive crisis and potential cardiac decompensation. Alpha-blockade must be established first to control vasoconstriction; beta-blockade is added afterward only to manage the reflex tachycardia that can follow.
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What operative approach do you choose, and when would you convert to or plan an open procedure?
Expected answer
Minimally invasive (laparoscopic) adrenalectomy is the preferred approach for most pheochromocytomas. Open adrenalectomy should be considered for tumors >6 cm and for any tumor with concerning features of malignancy (local invasion, lymphadenopathy) to ensure complete en-bloc resection and avoid tumor capsule disruption and seeding. Intraoperatively I would minimize tumor manipulation, ligate the adrenal vein early when feasible, and work with anesthesia anticipating rapid hemodynamic shifts.
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What intraoperative and postoperative issues do you anticipate and how do you manage them?
Expected answer
Intraoperatively expect labile hypertension with tumor manipulation and hypotension after venous ligation. This requires arterial-line monitoring, large-bore IV access, and ready short-acting agents (e.g., nitroprusside/nicardipine, esmolol/phentolamine for surges; vasopressors and fluids for post-resection hypotension). Postoperatively watch for hypotension (loss of catecholamine drive plus residual blockade—treat with fluids/pressors) and hypoglycemia (rebound hyperinsulinemia). Monitor in a high-acuity setting. Long-term, obtain follow-up biochemical testing to confirm cure and screen for recurrence/metastasis, and pursue genetic testing.
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Who should undergo genetic testing, and which syndromes are associated?
Expected answer
Genetic testing may be offered to all patients with pheochromocytoma but is mandatory when the tumor is bilateral, occurs in children or adults <45, when there are affected family members, or when it coexists with other endocrine syndromes. Associated conditions include von Hippel-Lindau disease, neurofibromatosis type 1, MEN 2, and succinate dehydrogenase subunit mutations (SDHA/B/C/D). This patient is 45, so testing should be strongly considered.
What Residents Often Miss
- Ordering CT/MRI or proceeding to surgery before establishing a biochemical diagnosis of pheochromocytoma.
- Biopsying the adrenal mass or performing adrenal vein sampling, which can precipitate a fatal hyperadrenergic crisis (automatic-fail error).
- Starting a beta-blocker before adequate alpha-blockade, causing unopposed alpha vasoconstriction and hypertensive crisis.
- Taking the patient to the OR without adequate alpha-blockade and volume expansion—leading to uncontrollable intraoperative hypertension.
- Failing to anticipate post-resection hypotension and hypoglycemia after ligation of the adrenal vein.
- Choosing laparoscopy for large (>6 cm) tumors or those with malignant features, risking incomplete resection and tumor seeding.
- Omitting genetic testing and long-term biochemical follow-up in a patient <45 with pheochromocytoma.
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