Symptomdx.

Get Started

Pheochromocytoma (Crisis)MANAGEMENT

ENDOCRINE AND METABOLIC PRESENTATIONS · 12 SECTIONS

⚲

784 items

☷ Legend

⚲

Cardiovascular Presentations71▾

Respiratory Presentations52▾

Neurological Presentations83▾

Gastrointestinal Presentations74▾

Genitourinary and Reproductive Presentations48▾

Infectious Disease Presentations148▾

Trauma Presentations89▾

Toxicologic Presentations80▾

Psychiatric Presentations26▾

Musculoskeletal and Rheumatologic Presentations96▾

Dermatology Presentations23▾

Environmental and Exposure Presentations27▾

Hematologic and Oncologic Presentations36▾

Pediatric-Specific Presentations19▾

Endocrine and Metabolic Presentations45▾

Acute Porphyria Adrenal crisis Adrenal Insufficiency (Addisonian Crisis)Dehydration Diabetes Insipidus Diabetic Ketoacidosis (DKA)Euglycemic DKA (SGLT2 inhibitor)Galactosemia GLP-1 receptor agonist complications (retained gastric contents, aspiration risk)Homocystinuria Hypercalcemia Hyperkalemia Hypermagnesemia Hypernatremia Hyperosmolar Hyperglycemic State (HHS)Hypertensive emergency Hypertensive urgency Hypocalcemia Hypoglycemia Hypogonadism (Acute Presentation)Hypokalemia Hypomagnesemia Hyponatremia Hypoparathyroidism (Tetany)Insulin pump failure Maple Syrup Urine Disease (Crisis)Medium-Chain Acyl-CoA Dehydrogenase Deficiency (MCAD)Metabolic Acidosis Metabolic Alkalosis Myasthenia gravis crisis Myxedema coma Pellagra Pheochromocytoma (Crisis)Pituitary Apoplexy Pompe Disease (GSD II)Respiratory Acidosis Respiratory Alkalosis Scurvy SIADH Starvation Ketosis Tarui Disease (GSD VII)Thyroid storm Tyrosinemia Vitamin D Deficiency (Severe-Rickets)Von Gierke Disease (GSD I)

ENT and Maxillofacial Presentations27▾

Ophthalmologic Presentations19▾

Obstetric Presentations23▾

Systemic and Miscellaneous Presentations17▾

01Approach to the Critical Patient/attcp57

Immediate stabilization

›Airway and oxygenation

›High-flow oxygen for hypoxemia or pulmonary edema

›SpO2 target >94%

›Intubation if airway not protected or refractory hypoxemia

›Escalate to resuscitation bay for flash pulmonary edema or cardiogenic shock

›Bilateral crackles with severe hypertension

›Worsening respiratory failure despite oxygen

›Hemodynamic crisis recognition

›Hypertensive emergency pattern

›SBP >180 mmHg with end-organ damage

›Goal: reduce SBP by ~25% in first hour then gradually to <140 mmHg over 24-48 hours

›Cardiogenic shock pattern

›Hypotension with evidence of low output

›Mean LVEF 30% in ICU cohort

›Hemodynamic lability pattern

›Rapidly alternating hypertension and hypotension

›Mortality rate 14-27% overall

Critical early actions

›IV access and monitoring

›Large-bore IV access x2

›Arterial line for continuous BP monitoring

›Central access if vasopressors or ECMO likely

›Continuous cardiac monitoring

›12-lead ECG immediately

›Arrhythmia detection: VT/VF most lethal

›Foley catheter for urine output monitoring

›Target urine output >0.5 mL/kg/hour

02History/hx98

Classic presentation

›Classic triad (present in only ~24% of patients)

›Severe headache

›Most common symptom in 200-case review (39.5%)

›Typically paroxysmal and severe

›Palpitations

›Often accompanied by tremor and anxiety

›Tachycardia or awareness of forceful heartbeat

›Diaphoresis

›Generalized profuse sweating

›Pallor, not flushing — catecholamine-mediated vasoconstriction

›Paroxysmal features

›Episodes lasting minutes to hours

›Sudden onset without clear trigger in some cases

›May occur at rest or with trivial exertion

›Nausea and vomiting

›Significantly associated with higher mortality

›Abdominal pain with nausea common

›Anxiety and tremor

›Adrenergic surge sensation

›Prior episodes dismissed as panic attacks

Alarm features

›End-organ damage indicators

›BP ≥180/120 mmHg with focal neurologic signs or AMS

›Hypertensive encephalopathy risk

›Posterior reversible encephalopathy syndrome possible

›Chest pain with troponin elevation

›Catecholamine cardiomyopathy or ACS mimic

›Young patient without traditional cardiovascular risk factors is key clue

›Acute pulmonary edema or flash pulmonary edema

›Catecholamine-induced heart failure

›May occur with normal or supranormal BP

›Pheochromocytoma multisystem crisis (PMC) pattern

›Temperature >40°C with encephalopathy

›Present in 19% of all crisis cases

›Highest mortality subtype

›Multi-organ failure with labile BP

›Lactic acidosis

›Unexplained hyperglycemia

Triggers and precipitants

›Pharmacologic triggers

›Metoclopramide and dopamine antagonists

›D2 receptor blockade triggers catecholamine release

›Antiemetics commonly given before diagnosis is known

›Beta-blockers without alpha-blockade

›Can precipitate or worsen crisis

›Labetalol insufficient alpha blockade

›Glucagon, corticosteroids, tricyclic antidepressants

›All can trigger catecholamine surge

›General anesthetics and neuromuscular blockers also implicated

›Physical and physiologic triggers

›Surgery or anesthesia induction

›Undiagnosed PPGL undergoing any procedure highest risk

›Tumor manipulation causes massive catecholamine release

›Trauma or tumor hemorrhage/infarction

›Spontaneous or traumatic

›Can cause sudden onset crisis

›Pregnancy (third trimester and peripartum)

›Mechanical compression of tumor

›Dramatically increased maternal and fetal mortality if unrecognized

›Physical exertion

›Valsalva maneuver, heavy lifting

›Bladder distension triggers in pelvic paraganglioma

Risk factors and family history

›Hereditary genetic syndromes (25-40% of all PPGL)

›MEN2 (RET mutation)

›PCC in up to 50% of MEN2 patients

›Bilateral tumors common

›Von Hippel-Lindau (VHL)

›PCC/PGL in 10-20% of VHL patients

›Earliest median age at diagnosis of hereditary syndromes

›SDHx mutations (SDHB, SDHD, SDHC, SDHA)

›Highest metastatic risk especially SDHB

›Extra-adrenal paraganglioma more common

›Neurofibromatosis type 1 (NF1)

›PCC in ~3% of NF1 patients

›Cafe-au-lait spots and neurofibromas on exam

›TMEM127 and MAX mutations

›Less common but recognized hereditary forms

›High-risk features

›Up to 40% of PPGL harbor germline pathogenic variants — highest rate of any solid tumor

›Genetic testing recommended for ALL patients per NCCN guidelines

›Young age at presentation (<45 years)

›Hereditary syndrome most likely

›Bilateral or extra-adrenal tumors

›Known adrenal incidentaloma

Past medical and medication history

›Prior episodes and diagnoses

›Recurrent "spells" of hypertension, palpitations, sweating

›Often dismissed as panic attacks or anxiety

›Prior anesthetic complications or intraoperative hypertensive crises

›Known adrenal mass or prior pheochromocytoma resection

›Recurrence surveillance compliance

›Residual or metastatic disease possible

›Chronic hypertension refractory to standard three-drug therapy

›Secondary hypertension workup context

›Current medication review

›Beta-blockers without alpha-blockade

›Contraindicated in active pheochromocytoma

›MAO inhibitors

›Severe hypertensive crisis risk with tyramine-rich foods

›Current alpha-blocker use

›Phenoxybenzamine or doxazosin suggests prior diagnosis

03Physical Exam/exam69

Vital signs

›Blood pressure assessment

›Severe hypertension often >200/120 mmHg

›Bilateral arm measurement for asymmetry

›Repeat frequently given labile nature

›Hemodynamic lability pattern

›Alternating hypertension and hypotension

›Type B crisis: sustained hypotension with shock

›Heart rate and temperature

›Sinus tachycardia most common rhythm abnormality

›Rate may exceed 150/min in epinephrine-secreting tumors

›Hyperthermia >40°C in PMC

›Catecholamine-induced hyperthermia distinct from fever

›Paradoxical bradycardia (less common)

›Baroreceptor reflex response to severe hypertension

Cardiovascular exam

›Cardiac auscultation

›S3 gallop indicating acute heart failure

›Dilated cardiomyopathy pattern

›Catecholamine cardiomyopathy pattern

›Pulmonary crackles

›Flash pulmonary edema

›Bilateral crackles with severe hypertension

›New murmur

›Wall motion abnormality complication

›Mitral regurgitation from papillary muscle dysfunction

›Peripheral perfusion

›Pallor (not flushing — classic catecholamine-mediated vasoconstriction)

04Differential Diagnosis/ddx57

Immediate life threats

›Cardiovascular emergencies

›Acute coronary syndrome / STEMI

›ICD-10 I21.9

›Pheo mimics ACS with ST changes, troponin elevation, wall motion abnormalities

›Normal coronary arteries on angiography distinguishes catecholamine cardiomyopathy

›Aortic dissection

›ICD-10 I71.00

›Severe hypertension with tearing chest and back pain

›CT angiography to exclude

›Hypertensive emergency from other cause

›ICD-10 I16.1

›Renal artery stenosis, primary hyperaldosteronism, malignant hypertension

Catecholamine-like syndromes

›Sympathomimetic toxicology

›Cocaine toxicity

›ICD-10 T40.5X1A

›History of illicit drug use

›Urine drug screen positive

›Amphetamine toxicity

›Similar hemodynamic crisis

›Screen for MDMA, methamphetamine

›Serotonin syndrome

›ICD-10 T43.1X5A

›Clonus, hyperreflexia, hyperthermia, agitation

›Medication history: SSRIs, MAOIs, triptans

›Neuroleptic malignant syndrome

›ICD-10 G21.0

05Laboratory Tests/lt54

Biochemical confirmation labs

›Plasma free metanephrines

›Preferred test in acute crisis setting

›Sensitivity >96% for pheochromocytoma

›Collected at bedside during hemodynamic crisis

›Levels in crisis typically 23x upper limit of normal

›Interpretation

›Epinephrine-secreting: adrenal pheo more likely

›Norepinephrine-only: extra-adrenal paraganglioma or adrenal pheo

›24-hour urine fractionated metanephrines and catecholamines

›After stabilization when acute collection feasible

›Complement to plasma testing

›False positive risk with severe illness; plasma preferred in crisis

›Urine fractionated metanephrines sensitivity ~97%

›Specificity lower than plasma free metanephrines

End-organ damage assessment

›Cardiac biomarkers

›Troponin (high-sensitivity preferred)

›Catecholamine-induced myocardial injury

›Demand ischemia from tachycardia and hypertension

›Elevation does not confirm ACS — normal coronaries in pheo cardiomyopathy

›BNP or NT-proBNP

›Acute heart failure severity marker

›Guide diuresis decision in pulmonary edema pattern

›Lactate

›Tissue hypoperfusion marker in cardiogenic shock

›Elevated lactate with normal or high BP suggests obstructive cardiomyopathy

›Renal and metabolic function

06Diagnostic Tests/dt73

Scoring Systems

›Whitelaw crisis severity classification

›Type A crisis

›Hemodynamic instability without sustained hypotension

›4 times more common than Type B

›Lower mortality than Type B

›Type B crisis

›Sustained hypotension, shock, and multi-organ dysfunction

›Higher mortality and ICU resource utilization

›Pheochromocytoma Multisystem Crisis (PMC)

›Hyperthermia + multi-organ failure + encephalopathy + labile BP

›Present in 19% of all crisis cases

›Highest mortality subtype

›SOFA score for ICU severity stratification

›Sequential Organ Failure Assessment

›Respiratory, coagulation, liver, cardiovascular, CNS, renal components

›Score ≥2 reflects organ dysfunction

›SAPS II

›Validated prognostic tool for ICU populations

›Used in published pheochromocytoma crisis cohort studies

MRI

›MRI adrenal/abdomen

›Preferred imaging modality for tumor characterization

›No ionizing radiation — preferred in younger patients and pregnancy

›T2-weighted: bright "light bulb" appearance characteristic of pheochromocytoma

›MRI protocol specifics

›Chemical shift imaging for lipid-poor adrenal lesions

›Gadolinium contrast enhancement pattern

›90-94% sensitivity for adrenal pheochromocytoma

›Limitations in acute crisis

›Monitoring difficulty in unstable patient

›CT preferred in hemodynamically unstable crisis

›MRI brain

›Posterior reversible encephalopathy syndrome (PRES)

›T2/FLAIR hyperintensity in posterior parieto-occipital regions

›Associated with severe hypertension

›Usually reversible with BP control

›CT adrenal / abdomen-pelvis

›First-line imaging in acute hemodynamic crisis

›Rapid acquisition in unstable patient

›Unenhanced CT: Hounsfield units >10 suggests functioning adrenal mass

›Sensitivity ~85-90% for adrenal pheochromocytoma

›CT protocol and interpretation

›Washout protocol: absolute washout >60% or relative >40% suggests benign adenoma

›Large tumors (>5 cm), irregular borders, local invasion suggest malignancy

›Extra-adrenal paraganglioma detection: scan from neck to pelvis

›CT angiography chest

›Exclude aortic dissection as mimic

›Pulmonary embolism exclusion in hemodynamically unstable patient

›CT head

›If hypertensive stroke or PRES suspected

›Hemorrhagic stroke: hypertensive hemorrhage

›Ischemic stroke: severe hypertension with vessel spasm

›Non-contrast CT first for hemorrhage exclusion

›CT angiography head/neck if dissection or vascular abnormality suspected

Ultrasound

›Bedside echocardiography

›Critical in pheochromocytoma crisis management

›Median LVEF 30% reported in ICU cohort

›Assess Takotsubo pattern: apical ballooning or inverted/basal pattern more common in PPGL-related TTS

›Dilated cardiomyopathy pattern from catecholamine toxicity

›Key echo parameters

›LVEF and regional wall motion abnormalities

›RV function and pulmonary pressures

›Pericardial effusion exclusion

›IVC size and respirophasic variation for volume status

›Serial echocardiography

›Recovery of LV function with treatment

›Guide mechanical circulatory support decisions

›Adrenal ultrasound

›Limited sensitivity for pheochromocytoma (~30-60%)

›Operator-dependent

›Bowel gas interference common

›Point-of-care use in pregnant patient

›Avoids radiation in first trimester

›Positive findings guide urgent MRI

07Disposition/dispo35

ICU admission — all crisis cases

›ICU admission criteria

›All confirmed or suspected pheochromocytoma crisis require ICU

›Continuous arterial BP monitoring

›Continuous cardiac monitoring and telemetry

›Ability to manage mechanical circulatory support if needed

›End-organ damage requiring ICU

›Cardiogenic shock

›Acute pulmonary edema

›Hypertensive encephalopathy

›Multi-organ dysfunction

›Ventricular arrhythmias

Transfer criteria

›Transfer to tertiary center indications

›Refractory cardiogenic shock requiring VA-ECMO

›Transfer to center with ECMO capability

›Early transfer before further deterioration

›Survival rate ~93.5% in published VA-ECMO series with full LVEF recovery in survivors

›Planned adrenalectomy

›Center with experienced endocrine or urologic surgery

›Anesthesia team familiar with pheochromocytoma management

›Complex hereditary syndrome management

›Multidisciplinary team (endocrinology, genetics, oncology)

Surgical timing

›Definitive surgical resection timing

›Minimum 7-14 days of medical stabilization before elective surgery

›Alpha-blockade adequacy: orthostatic hypotension and nasal congestion confirm adequate blockade

›Volume repletion with high-sodium diet and generous fluid intake

08Treatment/t111

Alpha-blockade — first-line and mandatory

›Phentolamine IV (preferred in acute crisis)

›Dosing protocol

›5 mg IV bolus

›Repeat every 10 minutes as needed

›Maximum 50 mg per 24 hours

›Mechanism and indication

›Competitive alpha-1 and alpha-2 receptor antagonist

›Immediate onset — ideal for crisis

›2025 AHA/ACC guideline: first-line for catecholamine excess

›Nicardipine IV (widely available alternative)

›Dosing protocol

›5 mg/h IV infusion

›Titrate by 2.5 mg/h every 5 minutes

›Maximum 15 mg/h

›Mechanism

›Dihydropyridine calcium channel blocker

›Arterial vasodilator without reflex tachycardia augmentation

›Does not require prior alpha-blockade

›Nitroprusside IV (refractory hypertension)

›Dosing protocol

›Starting dose 0.3-0.5 mcg/kg/min IV infusion

›Titrate to BP target

›Maximum 10 mcg/kg/min

›Cyanide toxicity risk with prolonged high-dose use (>72 hours)

›Indication

›Refractory hypertension not controlled by phentolamine or nicardipine

›Clevidipine IV (per 2025 AHA/ACC guidelines)

›Dosing protocol

›1-2 mg/h IV infusion

›Double dose every 90 seconds to target

›Maximum 32 mg/h

›Advantage

›Ultra-short acting; metabolism by blood esterases

›Rapidly titratable

Blood pressure targets

›Acute phase BP targets

›Reduce SBP by approximately 25% in first hour

›Avoid aggressive lowering to <120 mmHg in first hour (unlike aortic dissection)

›Gradual normalization to <140 mmHg over 24-48 hours

›Avoid over-treatment

›Catecholamine-mediated vasoconstriction masks true intravascular volume depletion

›Aggressive lowering may unmask severe hypotension

Beta-blockade — only after adequate alpha-blockade

›Esmolol IV (preferred — short acting and titratable)

›Indication

›Tachyarrhythmias after adequate alpha-blockade established

›Rate control for atrial fibrillation or flutter

›Ventricular arrhythmia adjunct

›Dosing protocol

›Loading dose 500 mcg/kg IV over 1 minute

›Maintenance infusion 50 mcg/kg/min

›Titrate by 25-50 mcg/kg/min every 5-15 minutes

›Maximum 300 mcg/kg/min

›Critical constraint

›ONLY after sufficient alpha-blockade confirmed

›Premature beta-blockade causes unopposed alpha stimulation and worsening hypertension

›Labetalol — AVOID in pheochromocytoma crisis

›Alpha-blocking effect insufficient relative to beta-blockade

›Can paradoxically worsen hypertensive crisis

›Contraindicated per consensus guidelines

Arrhythmia management

›Ventricular arrhythmias

›Esmolol as primary agent after alpha-blockade

›Short-acting, easily titratable

›Preferred for sustained VT

›Lidocaine IV for refractory ventricular arrhythmias

›1-1.5 mg/kg IV bolus

›Maintenance infusion 1-4 mg/min

›Magnesium sulfate IV adjunct

›2 g IV over 10-20 minutes for torsades de pointes risk

›Adjunctive BP and arrhythmia control

›QTc prolongation is common in pheochromocytoma crisis

›Atrial arrhythmias

›Rate control with esmolol after alpha-blockade

›Avoid digoxin — pro-arrhythmic in catecholamine excess state

›Cardioversion if hemodynamically unstable

Cardiogenic shock management

›Initial support

›IV fluid challenge cautiously in volume-depleted patient

›Guided by echocardiography

›Avoid aggressive diuresis unless overt pulmonary edema

›Vasopressors

›Norepinephrine for refractory hypotension

›Avoid pure alpha agonists — may worsen catecholamine excess state

›Mechanical circulatory support

›VA-ECMO as bridge to recovery in refractory cardiogenic shock

›Survival rate ~93.5% in published series

›Full LVEF recovery in survivors

›Early ECMO consideration — do not wait for cardiac arrest

›Impella or IABP as bridge or alternative

›Center capability dependent

›Impella preferred in hemodynamically unstable patients as bridge to ECMO

›Continuous renal replacement therapy (CRRT)

›Indications

›Acute kidney injury with fluid overload

›Catecholamine clearance adjunct

›Multi-organ dysfunction in PMC

Preoperative preparation (medical stabilization phase)

›Oral alpha-blockade for surgical preparation

›Phenoxybenzamine (irreversible, non-selective)

›Starting dose 10 mg PO twice daily

›Titrate to 20-30 mg three times daily

›Target orthostatic hypotension and nasal congestion as markers of adequate blockade

›Doxazosin (selective alpha-1, reversible — preferred in many centers)

›Starting dose 1 mg PO daily

›Titrate to 8-16 mg daily in divided doses

›Maximum 16 mg daily

›Duration: minimum 7-14 days before elective surgery

›Volume expansion protocol

›High-sodium diet (~5000 mg/day) during alpha-blockade

›Counteracts catecholamine-induced intravascular volume depletion

›Reduces intraoperative hemodynamic instability

›Generous fluid intake (~2.5 L/day)

›Beta-blockade added only after adequate alpha-blockade for reflex tachycardia

›Propranolol or atenolol PO

›Never first-line

09Special Populations/sp76

Pregnancy

›Unique risks in pregnancy

›Pheochromocytoma in pregnancy carries dramatically increased maternal and fetal mortality if unrecognized

›Risk highest in third trimester and peripartum

›Mechanical compression of tumor by gravid uterus triggers crisis

›Misdiagnosis as preeclampsia or HELLP syndrome

›Severe hypertension with headache and proteinuria overlap

›Plasma metanephrines appropriate in pregnancy — safe and highly sensitive

›Imaging in pregnancy

›MRI abdomen without gadolinium preferred

›Avoids ionizing radiation

›Gadolinium crosses placenta — avoid in first trimester

›Ultrasound as initial localization tool

›Limited sensitivity but no radiation risk

›Positive finding guides MRI

›Treatment modifications

›Phenoxybenzamine — limited data but most experience; fetal neonatal hypotension reported

›Monitor neonate for hypoglycemia and hypotension

›Nicardipine — reasonable alternative for BP control in pregnancy

›Calcium channel blockers used in pregnancy for other indications

›Beta-blockers with caution

›Risk of fetal bradycardia and IUGR

›Delivery timing

›If diagnosed before 24 weeks: laparoscopic adrenalectomy with concurrent pregnancy

›If diagnosed after 24 weeks: alpha-blockade until fetal maturity then cesarean section with concurrent adrenalectomy

›Vaginal delivery avoided — contractions trigger catecholamine surges

Geriatric

›Altered presentation patterns

›Atypical symptoms more common

›Weight loss and fatigue may predominate over classic triad

›Sustained hypertension more common than paroxysmal in older patients

›Higher comorbidity burden

›Pre-existing cardiovascular disease increases crisis mortality

›Baseline CKD limits aggressive IV fluid resuscitation

›Pharmacologic considerations

›Phentolamine — titrate carefully with arterial line monitoring

›Older patients prone to severe orthostatic hypotension

›Reduced starting doses prudent

›Volume resuscitation — caution in reduced cardiac reserve

›Echocardiography-guided fluid administration

›Pulmonary edema risk higher with aggressive resuscitation

›Drug interactions more common

›Polypharmacy review essential

›Tricyclic antidepressants, MAO inhibitors in older patients

›Postoperative risks

›Higher incidence of prolonged hypotension after resection

›ICU monitoring for 24-48 hours post-adrenalectomy

›IV fluid support and vasopressors as needed

›Increased delirium risk post-operatively

›Delirium prevention bundles

›Avoid anticholinergic medications

Pediatrics

›Epidemiology and presentation

›Rare in pediatric population — 10% of all PPGL cases

›Hereditary syndrome more likely in children and adolescents

›Genetic testing priority even higher in pediatric PPGL

›Extra-adrenal location more common in children (~30%)

›Paraganglioma along sympathetic chain

›Multisite disease possible

›Sustained hypertension more common than paroxysmal in children

›Often presents as refractory childhood hypertension

›Headache and visual changes common presenting symptoms

›Diagnostic testing

›Plasma free metanephrines — preferred test

›Sensitivity comparable to adults

›Reference ranges age-adjusted

›MRI preferred over CT to avoid radiation in developing children

›Gadolinium contrast use with caution

›Treatment in pediatric crisis

›Phentolamine weight-based dosing

›0.1 mg/kg IV bolus (maximum 5 mg) every 10 minutes

›Nicardipine infusion

›0.5-3 mcg/kg/min IV — titrate to response

›Esmolol weight-based after alpha-blockade

›100-500 mcg/kg IV loading dose

›25-200 mcg/kg/min infusion

›Preoperative alpha-blockade

›Phenoxybenzamine 0.2-1 mg/kg/day divided twice daily

›Doxazosin 0.05 mg/kg/day titrated up

10Background/b72

Epidemiology

›Incidence and prevalence

›Overall pheochromocytoma/paraganglioma (PPGL) incidence: 2-8 cases per million per year

›Pheochromocytoma accounts for 80-85% (adrenal), paraganglioma 15-20% (extra-adrenal)

›Crisis occurs in 7-18% of PPGL patients

›Age and sex distribution

›Peak incidence 30-50 years but can occur at any age

›Equal sex distribution overall

›Young patients (<45 years) more likely to have hereditary syndrome

›Hereditary burden

›Up to 40% of PPGL harbor germline pathogenic variants — highest rate of any solid tumor

›Previously thought to be rare hereditary disease (<10%)

›Over 20 susceptibility genes identified

›Hereditary syndromes account for 25-40% of all cases

›MEN2, VHL, NF1, SDHx most common

›Mortality and outcomes

›Pheochromocytoma crisis overall mortality: 14-27%

›PMC (pheochromocytoma multisystem crisis): highest mortality subtype

›Heart most commonly damaged organ (99% in crisis series)

›Lungs (44%) and kidneys (21.5%) also commonly affected

Pathophysiology

›Catecholamine excess mechanism

›Tumor origin

›Arise from chromaffin cells of adrenal medulla (pheo) or extra-adrenal sympathetic ganglia (paraganglioma)

›Secrete epinephrine, norepinephrine, and/or dopamine

›Stored in dense-core granules; released in bursts

›Hemodynamic effects of catecholamine excess

›Alpha-1 stimulation: intense vasoconstriction, hypertension, decreased organ perfusion

›Beta-1 stimulation: tachycardia, increased contractility, arrhythmias

›Epinephrine-secreting phenotype: Takotsubo and tachyarrhythmias predominate

›Norepinephrine phenotype: atherosclerotic complications and flash pulmonary edema predominate

›Cardiac injury mechanisms

›Catecholamine cardiomyopathy

›Direct myocardial toxicity via oxidative stress and mitochondrial dysfunction

›Catecholamine-mediated myocarditis — focal and diffuse myocardial fibrosis

›MRI-confirmed fibrosis in a significant proportion of PPGL patients

›Takotsubo syndrome (stress cardiomyopathy)

›PPGL-related TTS: higher cardiogenic shock rate (34%) vs non-PPGL TTS (4%)

›Apical sparing pattern (inverted/basal) more common in PPGL than typical TTS

›Catecholamine receptor downregulation in apical myocardium proposed mechanism

›Coronary vasospasm

›Alpha-mediated vasoconstriction of coronary arteries

›STEMI pattern with normal coronaries on angiography

›Volume depletion mechanism

›Chronic catecholamine-mediated vasoconstriction

›Reduces venous capacitance and plasma volume

›True hypovolemia despite hypertension

›Explains post-resection hypotension

Therapeutic Considerations

›Alpha-before-beta principle

›Fundamental rule of pheochromocytoma pharmacotherapy

›Beta-blockade before alpha-blockade causes unopposed alpha stimulation

›Can precipitate or dramatically worsen hypertensive crisis

›Even short-acting beta-blockers dangerous without prior alpha-blockade

›Mechanism of worsening

›Beta-2 blockade removes vasodilatory beta-mediated relaxation

›Net alpha stimulation unopposed: profound vasoconstriction

›Tyramine avoidance

›Tyramine-rich foods displace catecholamines from vesicular storage

›Aged cheeses, cured meats, fermented foods, red wine

›Can precipitate crisis in active or uncontrolled PPGL

›MAO inhibitors — absolute contraindication in PPGL

›Markedly amplify catecholamine excess through reduced degradation

›Surgical cure vs medical management

›Surgical resection is the only curative treatment

›Laparoscopic adrenalectomy standard for adrenal PPGL <10 cm

›Open adrenalectomy for large or invasive tumors

›Intraoperative anesthetic precautions

›Invasive arterial monitoring mandatory

›Phentolamine and nitroprusside immediately available

›Esmolol for intraoperative tachyarrhythmias

›Anticipate hypotension after ligation of tumor blood supply — have norepinephrine ready

›Biochemical cure confirmation

›Plasma/urine metanephrines at 2-6 weeks post-resection

›Annual lifelong surveillance given recurrence risk

11Patient Discharge Instructions/pdi52

copy discharge instructions

›Diagnosis and condition explanation

›You were treated for a pheochromocytoma crisis

›A pheochromocytoma is a tumor of the adrenal gland that releases excessive adrenaline (epinephrine and norepinephrine)

›This caused dangerously high blood pressure and effects on your heart and other organs

›This is a serious condition that required intensive monitoring and treatment

›Medications — critical instructions

›Take all blood pressure medications exactly as prescribed

›Do NOT stop your alpha-blocker (phenoxybenzamine or doxazosin) without speaking to your doctor

›Taking a beta-blocker without your alpha-blocker can be life-threatening

›Medications to avoid

›Metoclopramide (Reglan) — can trigger a crisis

›Over-the-counter decongestants (pseudoephedrine, phenylephrine)

›Epinephrine-containing dental anesthetics — inform your dentist of your diagnosis

›Foods to avoid

›Aged cheeses, cured or smoked meats, fermented foods, red wine

›These foods can trigger a blood pressure surge

›Diet and fluid instructions

›High-salt diet if on preoperative alpha-blockade

›Your doctor may recommend extra salt to keep blood pressure stable on your medications

›Drink plenty of fluids — aim for approximately 2.5 litres daily

›This helps prevent dehydration and light-headedness

›Activity restrictions

›Avoid heavy lifting, straining, or vigorous exertion until cleared by your surgeon

›Physical strain can trigger a release of adrenaline from the tumor

›Avoid Valsalva maneuvers (bearing down)

›Increased abdominal pressure can squeeze the tumor

›Genetic testing and family notification

12References/r30

Guidelines and key sources

›Ando Y, et al. Clinical Characteristics and Outcomes of Pheochromocytoma Crisis: A Literature Review of 200 Cases. Journal of Endocrinological Investigation. 2022. PMID 35857218

›200-case literature review; headache 39.5%; nausea/vomiting associated with higher mortality

›Heart most commonly damaged organ (99%), lungs (44%), kidneys (21.5%)

›Sauneuf B, et al. Pheochromocytoma Crisis in the ICU: A French Multicenter Cohort Study. Critical Care Medicine. 2017. PMID 28403121

›Median LVEF 30% in ICU cohort

›VA-ECMO survival ~93.5% with full LV recovery in survivors

›Whitelaw BC, et al. Phaeochromocytoma crisis. Clinical Endocrinology. 2014.

›Type A and Type B crisis classification

›PMC defined: hyperthermia + encephalopathy + labile BP + multi-organ failure (19% of cases)

›Nazari MA, et al. Catecholamine-Induced Hypertensive Crises: Current Insights and Management. Lancet Diabetes Endocrinology. 2023. PMID 37944546

›Comprehensive review of pathophysiology and management strategies

›BP target: reduce SBP 25% in first hour; <140 mmHg over 24-48 hours

›Jones DW, et al. 2025 AHA/ACC/AANP Guideline for the Prevention, Detection, Evaluation, and Management of High Blood Pressure in Adults. JACC. 2025.

›Phentolamine and clevidipine recommended for catecholamine excess states

›First-line IV alpha-blockade before beta-blockade

›Nazari MA, Rosenblum JS, Haigney MC, Pacak K. Pathophysiology and Acute Management of Tachyarrhythmias in Pheochromocytoma. JACC. 2020. PMID 32703516

›Comprehensive arrhythmia management; esmolol preferred for rate control after alpha-blockade

›Epinephrine phenotype: Takotsubo and tachyarrhythmia risk; norepinephrine phenotype: atherosclerosis and flash pulmonary edema

›Neumann HPH, Young WF, Eng C. Pheochromocytoma and Paraganglioma. NEJM. 2019. PMID: 31875526

›Landmark review of genetics, diagnosis, and management

›Up to 40% germline pathogenic variants; genetic testing recommended for all

›NCCN Clinical Practice Guidelines in Oncology: Neuroendocrine and Adrenal Tumors. Updated 2026.

›Genetic testing recommended for ALL PPGL patients

›Comprehensive surveillance and management algorithms

›Giraud R, et al. Pheochromocytoma Multisystem Crisis Requiring Temporary Mechanical Circulatory Support: A Narrative Review. JCM. 2025. PMID 40142715

›PMC management; early mechanical circulatory support recommendation

›Ferreira VM, et al. Pheochromocytoma Is Characterized By Catecholamine-Mediated Myocarditis, Focal and Diffuse Myocardial Fibrosis. JACC. 2016. PMID 27199060

Learn More: Pheochromocytoma (Crisis)→

Comprehensive Clinical Review

Structured, evidence-informed, long-form analysis for deeper understanding.

Evidence & Review

Reviewed by SymptomDx Medical Team · Last reviewed August 18, 2026

SymptomDx is an educational tool for medical professionals. It does not replace clinical judgment. Verify all clinical data and drug dosages with authoritative sources.