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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·6,183 words·25 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.
Pheochromocytoma is a rare neuroendocrine tumor of the adrenal medulla that secretes catecholamines, causing paroxysmal or sustained hypertension and life-threatening cardiovascular crises. Although fewer than 10% of adrenal incidentalomas prove to be pheochromocytomas, early diagnosis and surgical resection are curative in most sporadic cases. Up to 40% of cases are hereditary, driven by germline mutations in genes such as RET, VHL, NF1, and SDHx, making genetic testing essential for all patients. The cornerstone of management is preoperative alpha-blockade to prevent intraoperative hypertensive crisis, followed by complete surgical resection. For metastatic disease, targeted radionuclide therapy with 177Lu-DOTATATE or high-specific-activity 131I-MIBG, and emerging agents like belzutifan, offer disease control.

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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