What is the management plan for a 25-year-old male with a nodular mass on the adrenal gland, hypertension (HTN), and episodes of paroxysmal hypertension with headache and sweats, suggestive of pheochromocytoma?

Management of Suspected Pheochromocytoma in a Young Male

This 25-year-old male with an adrenal mass, hypertension, and classic paroxysmal symptoms (headache and sweats) requires immediate biochemical confirmation with plasma free metanephrines or 24-hour urinary fractionated metanephrines, followed by urgent alpha-blockade initiation and surgical planning. 1

Immediate Diagnostic Workup

Biochemical Testing (First Priority)

• Obtain plasma free metanephrines (sensitivity 96-100%, specificity 89-98%) OR 24-hour urinary fractionated metanephrines (sensitivity 86-97%, specificity 86-95%) to confirm catecholamine excess 1
• Plasma testing should ideally be collected from an indwelling venous catheter after 30 minutes supine to minimize false positives 1
• The classic triad of headache, sweating, and palpitations has 90% diagnostic specificity for pheochromocytoma 1
• Avoid interfering medications and foods prior to testing, though common antihypertensives do not affect LC-MS/MS analysis 1

Imaging Studies

• CT or MRI of abdomen/pelvis is indicated to localize the tumor and assess for bilateral disease, extra-adrenal paragangliomas, or metastases 2, 3
• Do NOT perform fine needle biopsy—this is absolutely contraindicated due to risk of fatal hypertensive crisis 2, 3
• MRI is preferred over CT due to risk of hypertensive crisis with IV contrast 1

Interpretation of Biochemical Results

If metanephrines are ≥4 times upper limit of normal:

• This confirms biochemically active pheochromocytoma 1, 3
• Proceed immediately to alpha-blockade and surgical planning 3

If metanephrines are 2-4 times upper limit of normal:

• Repeat testing in 2 months 1
• Consider clonidine suppression test (100% specificity, 96% sensitivity) if results remain equivocal 1

If metanephrines are marginally elevated:

• Repeat in 6 months and consider clonidine suppression test 1

Medical Management Prior to Surgery

Alpha-Blockade (Critical First Step)

• Initiate alpha-adrenergic blockade within 24-48 hours of biochemical confirmation 3
• Phenoxybenzamine is FDA-indicated for pheochromocytoma to control hypertension and sweating episodes 4
• Continue alpha-blockade for 10-14 days before any surgical intervention 3
• Target blood pressure: <130/80 mmHg supine AND systolic >90 mmHg standing 3

Beta-Blockade (Only After Alpha-Blockade)

• Add beta-blocker only if tachycardia is excessive, and only AFTER adequate alpha-blockade is established 4
• Never initiate beta-blockade before alpha-blockade—this can precipitate unopposed alpha-stimulation and hypertensive crisis 5, 6

Surgical Management

Definitive Treatment

• Complete surgical extirpation (R0 resection) is the only curative treatment and mainstay of therapy 2, 3
• Laparoscopic adrenalectomy is the preferred approach for most adrenal pheochromocytomas 3
• Meticulous perioperative management of blood pressure, glucose, electrolytes, and cardiac abnormalities is critical 2

Intraoperative Considerations

• Treat intraoperative hypertension with magnesium sulfate, phentolamine, calcium antagonists, nitroprusside, or nitroglycerin 3
• Anticipate and aggressively treat postoperative hypotension with fluid resuscitation 3
• Monitor glucose levels closely—hypoglycemia commonly occurs after catecholamine levels drop 3

Postoperative Follow-Up

Immediate Postoperative Assessment

• Measure plasma or urine metanephrines at 2-8 weeks postoperatively to confirm complete tumor removal 3

Long-Term Surveillance (Mandatory)

• All patients require lifelong surveillance due to 10-15% recurrence risk 3
• Clinical monitoring (blood pressure, adrenergic symptoms) every 3-4 months for first 2-3 years, then every 6 months 3
• Biochemical testing (plasma/urine metanephrines, chromogranin A) every 3-4 months for 2-3 years, then every 6 months 3
• Continue follow-up for at least 10 years, with lifelong surveillance favored 2

Genetic Testing Considerations

Screening Recommendations

• Approximately one-third of pheochromocytomas are hereditary, making genetic screening important 1
• All first-degree relatives should undergo biochemical testing 7
• Consider molecular genetic testing, especially in young patients (<30 years) 1, 8
• SDHB mutations are associated with higher risk of malignancy and require more intensive surveillance 1

Critical Pitfalls to Avoid

• Never biopsy a suspected pheochromocytoma—this can cause fatal hypertensive crisis 2, 3
• Never start beta-blockers before alpha-blockade—this causes unopposed alpha-stimulation 5, 6
• Never proceed to surgery without adequate preoperative alpha-blockade (10-14 days minimum) 3
• Do not assume benign disease—10-15% of pheochromocytomas are malignant, defined by presence of metastases 2
• Do not discontinue surveillance after successful surgery—lifelong follow-up is mandatory 3