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Endocrinology›Condition·Updated Jun 27, 2026·v1

Pheochromocytoma

Pheochromocytoma is a rare, catecholamine-secreting neuroendocrine tumor of the adrenal medulla that causes potentially fatal hypertensive crises. Diagnosis relies on plasma free metanephrines and imaging (68Ga-DOTATATE PET/CT for staging). Management centers on preoperative alpha-blockade for 7-14 days, followed by complete surgical resection (laparoscopic for tumors ≤6 cm). Up to 40% of cases are hereditary, requiring germline genetic testing in all patients. Metastatic disease is treated with targeted radionuclide therapy (177Lu-DOTATATE or 131I-MIBG) and emerging agents like belzutifan. Prognosis is excellent for localized disease (biochemical cure >95%) but guarded for metastatic disease (5-year survival 40-60%). Lifelong surveillance is essential due to risk of recurrence, especially in hereditary syndromes.

High Evidence130 references·1,789 words·8 min read·v1
pheochromocytomaparagangliomaneuroendocrine tumorsecondary hypertensionadrenal incidentalomacatecholaminesSDHBMEN2von Hippel-Lindau disease

Quick Reference

RxDrug of choicePhenoxybenzamine 10 mg PO BID, titrated to 20-40 mg TID for preoperative alpha-blockade
AltAlternativesDoxazosin 2-16 mg PO daily; for hypertensive crisis: phentolamine 5-10 mg IV bolus or nitroprusside 0.5-10 mcg/kg/min IV infusion
⊘AvoidBeta-blockers without prior alpha-blockade (risk of unopposed alpha-mediated vasoconstriction and hypertensive crisis)
DxTest of choicePlasma free metanephrines (normetanephrine, metanephrine, methoxytyramine) for diagnosis; 68Ga-DOTATATE PET/CT for staging
ScKey scorePheochromocytoma of the Adrenal Gland Scaled Score (PASS), score ≥4 indicates higher risk of metastatic behavior
→When to referRefer to endocrinology for diagnosis and preoperative management; to surgical oncology for resection; to medical oncology for metastatic disease; to genetic counselor for germline testing
Pheochromocytoma is a rare but curable cause of secondary hypertension; prompt diagnosis with plasma metanephrines, preoperative alpha-blockade, and complete surgical resection achieve cure in >95% of localized cases; all patients require germline genetic testing and lifelong surveillance.
This concise bedside reference summarizes the essential clinical facts about pheochromocytoma, a rare but curable cause of severe hypertension and cardiovascular emergencies. It covers epidemiology, risk factors, pathophysiology, clinical presentation, diagnostic workup, staging, management, and prognosis, including key drug doses and critical thresholds. Material is drawn from peer-reviewed guidelines and trials, with citation numbers preserved from the detailed wiki page.

Overview and Recommendations

Background

  • •Pheochromocytoma is a rare catecholamine-secreting neuroendocrine tumor arising from chromaffin cells of the adrenal medulla, with an incidence of 2 to 8 cases per million person-years globally, though this likely underestimates true prevalence due to frequent underdiagnosis and the fact that many tumors are discovered incidentally on cross-sectional imaging.
  • •The tumor accounts for fewer than 10% of adrenal incidentalomas in endocrine referral populations, but it is a critical diagnosis to make because undiagnosed pheochromocytoma carries a high risk of fatal hypertensive crisis, myocardial injury, and stroke; early detection and resection are curative in >95% of localized cases.
  • •Up to 40% of pheochromocytomas are hereditary, driven by germline mutations in at least 20 susceptibility genes including RET (MEN2), VHL (von Hippel-Lindau disease), NF1 (neurofibromatosis type 1), and the succinate dehydrogenase (SDHx) subunit genes, SDHB mutations confer the highest metastatic risk (30-50% lifetime), while SDHD mutations show maternal imprinting and high penetrance for head and neck paragangliomas.
  • •The classic teaching of the '10% tumor' (10% bilateral, 10% extra-adrenal, 10% malignant, 10% familial) has been replaced by a more nuanced understanding: bilaterality and familial occurrence vary substantially by genetic background, and all pheochromocytomas are now classified as having variable metastatic potential rather than being strictly benign or malignant.
  • •The molecular classification of pheochromocytomas into three clusters, pseudohypoxia (cluster 1, HIF stabilization, high metastatic risk with SDHB), kinase signaling (cluster 2, MAPK/ERK activation, lower risk), and Wnt signaling (cluster 3, intermediate risk), directly guides systemic therapy selection in metastatic disease, with cluster 1 tumors potentially responding to HIF-2α inhibitors like belzutifan and cluster 2 tumors to RET inhibitors like selpercatinib.

Evaluation

  • •Suspect pheochromocytoma in any patient with paroxysmal hypertension, resistant hypertension (uncontrolled on ≥3 agents), or the classic triad of headache, palpitations, and diaphoresis, though fewer than half present with all three symptoms, so maintain a low threshold for testing.
  • •Ask about triggers: physical activity, micturition (suggestive of bladder paraganglioma), anesthesia, surgery, contrast media, glucocorticoids (methylprednisolone can precipitate cardiac arrest), and certain medications (tricyclic antidepressants, beta-blockers that may mask symptoms).
  • •Examine for pallor, diaphoresis, tachycardia, hypertensive retinopathy (arteriolar narrowing, hemorrhages, papilledema), orthostatic hypotension (due to chronic vasoconstriction and volume contraction), and abdominal tenderness from large adrenal tumors; head and neck paragangliomas may present as a pulsatile neck mass with Horner syndrome or vocal cord palsy.
  • •Order plasma free metanephrines (normetanephrine, metanephrine, methoxytyramine) as the first-line diagnostic test, sensitivity exceeds 96%; a level >4-fold the upper reference limit is highly suggestive and warrants urgent imaging.
  • •If plasma metanephrines are equivocal (e.g., elevated normetanephrine but normal metanephrine), perform a clonidine suppression test: administer clonidine 0.3 mg orally and measure plasma normetanephrine at 3 hours; a post-clonidine level >0.71 nmol/L has 100% specificity for pheochromocytoma.
  • •Order adrenal protocol CT (noncontrast, contrast-enhanced, delayed phases) for initial localization, pheochromocytomas show precontrast attenuation >10 HU, avid enhancement, and washout <50% (unlike lipid-rich adenomas); MRI shows T2 hyperintensity (the 'light bulb' sign).
  • •For staging, perform 68Ga-DOTATATE PET/CT as the preferred functional imaging modality, per-lesion sensitivity exceeds 95%, especially in SDHB-associated metastatic disease; alternative tracers include 18F-FDG (for aggressive/metastatic tumors) and 123I-MIBG (for selecting patients for 131I-MIBG therapy).
  • •Diagnostic criteria per the Endocrine Society: biochemical evidence of catecholamine excess (elevated plasma or urinary metanephrines) plus tumor localization on imaging; histologic confirmation is reserved for cases where imaging is inconclusive or tissue is needed for molecular profiling.
  • •Also consider: rule out other causes of secondary hypertension (renal artery stenosis, hyperaldosteronism, Cushing syndrome, thyroid disease, coarctation of the aorta); in pregnancy, distinguish from preeclampsia/gestational hypertension, MRI without gadolinium is the imaging modality of choice.
  • •Perform germline genetic testing in all patients with pheochromocytoma/paraganglioma (PPGL) given the high prevalence of hereditary syndromes, test for RET, VHL, NF1, SDHA, SDHB, SDHD, SDHAF2, TMEM127, MAX; cascade testing of at-risk relatives enables early detection and prevention.

Management

  • •Initiate preoperative alpha-blockade at least 7-14 days before surgery: phenoxybenzamine (noncompetitive alpha-1 blocker) starting at 10 mg PO twice daily, titrating to 20-40 mg three times daily, or doxazosin (competitive alpha-1 blocker) 2-16 mg once daily; goal blood pressure <130/80 mmHg, heart rate 60-80 bpm, with mild orthostatic hypotension without symptoms.
  • •Add a beta-blocker (e.g., propranolol 20-40 mg PO three times daily or atenolol 25-50 mg daily) only after adequate alpha-blockade is established, never use beta-blockers first, as this can precipitate unopposed alpha-mediated vasoconstriction and hypertensive crisis.
  • •For hypertensive crisis: administer phentolamine (competitive alpha-blocker) 5-10 mg IV bolus, repeated as needed, or nitroprusside 0.5-10 mcg/kg/min IV infusion; for tachyarrhythmias, add esmolol 50-200 mcg/kg/min IV after alpha-blockade is confirmed.
  • •Perform laparoscopic adrenalectomy as the standard for tumors ≤6 cm without evidence of invasion; laparoscopic approach reduces hospital stay and blood loss compared to open surgery, with similar recurrence rates.
  • •For tumors >6 cm or with suspected invasion, proceed with open adrenalectomy to ensure complete resection and avoid tumor rupture.
  • •In hereditary syndromes (MEN2, VHL) with bilateral tumors or risk of contralateral recurrence, perform partial (cortical-sparing) adrenalectomy to preserve adrenal function, meta-analysis shows similar recurrence rates (2.1% vs 1.8%) but lower adrenal insufficiency (0% vs 100%) with partial vs total adrenalectomy.
  • •For head and neck paragangliomas, refer to a multidisciplinary team including head and neck surgery and interventional radiology; consider preoperative embolization for large, vascular tumors.
  • •Postoperatively, monitor blood glucose hourly for 24 hours, hypoglycemia occurs in 15-30% of patients due to sudden catecholamine withdrawal; treat with dextrose 50% IV if glucose <70 mg/dL, and provide continuous dextrose infusion if needed.
  • •Monitor blood pressure every 15 minutes for the first 2 hours postoperatively, then hourly for 24 hours; hypotension may require volume resuscitation with normal saline, avoid vasopressors unless refractory.
  • •Do not discharge until blood pressure is stable without vasopressors and glucose is stable; instruct patient on orthostatic precautions and follow-up with endocrinology in 2-4 weeks.
  • •For metastatic pheochromocytoma/paraganglioma, first-line therapy is targeted radionuclide therapy: 177Lu-DOTATATE (PRRT) preferred for somatostatin receptor-positive tumors, especially SDHB-mutated, with median PFS 20-30 months; alternatively, high-specific-activity 131I-MIBG (Azedra) 12 mCi/kg IV for MIBG-avid disease, with objective response rate 22% and disease control rate 68%.
  • •For progressive disease after targeted radionuclide therapy, consider belzutifan (HIF-2α inhibitor) 120 mg PO once daily, objective response rate 25%, median PFS 13.8 months; or cabozantinib 40 mg PO daily plus atezolizumab 1200 mg IV every 3 weeks, ORR 40%.
  • •Chemotherapy options: cyclophosphamide 750 mg/m² IV day 1, vincristine 1.4 mg/m² IV day 1, dacarbazine 600 mg/m² IV days 1-2, repeated every 21-28 days; OR temozolomide 150 mg/m² PO days 1-7 every 14 days plus thalidomide 50-400 mg PO daily, biochemical response ~40% but limited radiologic response.
  • •What NOT to do: do not use beta-blockers without prior alpha-blockade; do not perform percutaneous biopsy of suspected pheochromocytoma without preoperative alpha-blockade and use of an 18-gauge or smaller coaxial needle; do not use bicarbonate for intraoperative acidosis unless pH <7.1.
  • •Refer to medical oncology for metastatic disease management; refer to genetic counselor for all patients with germline mutations; refer to interventional radiology for tumor ablation or embolization of liver metastases; refer to cardiology for management of hypertensive crises and long-term blood pressure control.
  • •Discharge criteria: blood pressure <140/90 mmHg without IV medications, heart rate <100 bpm, glucose >70 mg/dL without IV dextrose, pain controlled on oral analgesics, tolerating oral diet, no evidence of wound infection or bleeding, and follow-up appointments scheduled with endocrinology and primary care.

Board Review — High Yield

  • •Zellballen, Nested clusters of polygonal chief cells surrounded by sustentacular cells on H&E staining, characteristic of pheochromocytoma.
  • •Plasma free metanephrines, First-line diagnostic test with sensitivity >96%; levels >4-fold upper reference limit are highly suggestive.
  • •Clonidine suppression test, Used when plasma normetanephrine is elevated but metanephrine is normal; post-clonidine normetanephrine >0.71 nmol/L has 100% specificity.
  • •Preoperative alpha-blockade, Mandatory for 7-14 days before surgery to prevent intraoperative hypertensive crisis; never use beta-blockers first.
  • •68Ga-DOTATATE PET/CT, Preferred functional imaging modality for staging, with sensitivity >95%, especially in SDHB-mutated disease.
  • •SDHB mutation, Strongest predictor of metastatic behavior (30-50% lifetime risk); SDHB immunohistochemistry loss is a surrogate for SDH deficiency.
  • •AJCC 8th edition TNM staging, Only validated staging system; metastasis (M1) automatically defines Stage IV and malignant disease.
  • •Belzutifan, HIF-2α inhibitor for metastatic pheochromocytoma; objective response rate 25%, median PFS 13.8 months.
  • •Pheochromocytoma in pregnancy, MRI without gadolinium for diagnosis; alpha-blockade mainstay; elective cesarean section preferred to avoid catecholamine surges during labor.
  • •Partial adrenalectomy, Recommended for bilateral tumors or hereditary syndromes (MEN2, VHL) to preserve adrenal function; similar recurrence rates to total adrenalectomy.

Deep Dive — Evidence Details

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