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Patient handout

Pheochromocytoma / paraganglioma crisis (catecholamine-driven episodic HTN crisis)

PRODUCTION

1. Your condition

This handout is for pheochromocytoma / paraganglioma crisis (catecholamine-driven episodic htn crisis). Your care team identified this based on: episodic htn crisis + headache + diaphoresis + palpitations + pallor (lenders lancet 2005 pmid 16125595).

Other reasons your team may use this plan: adrenal incidentaloma on ct/mri + htn — biochemical screen for pheo (endocrine society 2014 pmid 24893135); plasma free metanephrines or 24h urine fractionated metanephrines >4× upper limit — strong pheo suggestion (lenders jama 2002 pmid 12087161); men2a/2b, vhl, nf1, sdhx mutation — surveillance htn crisis (endocrine society 2014).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

MedicationStarting doseHowWhenWhat it does
phentolamine5 mg IV bolus q5-15 min titrate (max 15 mg single dose)IVPRN bolus or 0.5-1 mg/min infusionEndocrine Society 2014 PMID 24893135; Pacak NEJM 2007 PMID 17463337 — pure alpha-blocker fastest reversal of catecholamine HTN; rapid onset (1-2 min) + short half-life (~20 min)
phenoxybenzamine10 mg PO BID, increase by 10-20 mg q2-3 d to BP normal + orthostasis + nasal stuffiness, typical max 80-100 mg/dPOBIDPacak NEJM 2007 PMID 17463337 — preop alpha-blockade × 7-14 d; titrate to nasal stuffiness (alpha-1 blockade marker), supine BP normal, mild orthostasis acceptable
doxazosin2 mg PO daily, titrate to 16 mg maxPOdailyEndocrine Society 2014 — selective alpha-1 alternative to phenoxybenzamine; less reflex tachy; less postop hypotension; slightly less complete blockade
nicardipine5 mg/h IV titrate by 2.5 mg/h q5-15 min, max 15 mg/hIVcontinuousACC/AHA 2025 — predictable titration; combine with phentolamine; CCB acceptable in pheo (no unopposed-alpha concern)
nitroprusside0.25-3 mcg/kg/min IV titrateIVcontinuousAcceptable in pheo crisis (no unopposed-alpha concern); short half-life allows tight control; AVOID >24-48h or eGFR <30 (cyanide risk)
metoprololONLY after ≥24-48 h alpha-blockade established; 25 mg PO BID titratePOBIDEndocrine Society 2014 — β-blocker ONLY after alpha-blockade established; never alone; for HR control + arrhythmia prophylaxis preop
propranololONLY after ≥24-48 h alpha-blockade; 10 mg PO TID titratePOTIDHistorical preferred BB in pheo (after alpha) per Bravo NEJM 1979 PMID 481031; never alone
AVOID β-blocker monotherapyAVOIDN/AN/AEndocrine Society 2014 PMID 24893135 — β-blocker without alpha-blockade causes UNOPPOSED ALPHA vasoconstriction → SEVERE paradoxical HTN crisis; absolute contraindication
AVOID dopamine, glucagon, metoclopramide, opioidsAVOIDN/AN/AThese agents trigger catecholamine release from pheo → can precipitate crisis (Endocrine Society 2014 PMID 24893135)
IV fluids — high-salt diet preop + crystalloid intraopLiberal NaCl PO + IV NS 1-2 L preopPO + IVcontinuousPacak NEJM 2007 PMID 17463337 — chronic catecholamine venoconstriction = volume-depleted; preop volume expansion prevents postop hypotension after tumor removal

Plan: Pheochromocytoma crisis — alpha-blockade FIRST (NEVER β-blocker monotherapy); volume repletion despite HTN; preop alpha-blockade 7-14 d before surgical resection

3. When to call your provider

Contact your care team if any of the following happen:

  • Recurrent symptoms → urgent metanephrines + imaging
  • New mass on imaging → biopsy contraindicated; biochemical eval first

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

  • Pheo crisis + new severe LV apical ballooning + chest pain + minimal CAD on cath — Takotsubo precipitated by catecholamine surge(life-threatening)
  • SEVERE paradoxical HTN crisis after β-blocker administered without alpha-blockade — unopposed alpha vasoconstriction(life-threatening)
  • Pheo crisis + acute LV failure + cardiogenic shock from prolonged catecholamine exposure → catecholamine cardiomyopathy(life-threatening)
  • Intraoperative severe HTN crisis during pheo resection (despite preop alpha-blockade) — tumor manipulation releases catecholamine surge(life-threatening)
  • Severe hypotension immediately post-pheo resection — chronic volume depletion + sudden catecholamine withdrawal → vasoplegia
  • Pheo with metastatic disease (bone, lung, liver, lymph nodes) on imaging — malignant pheo (10-17% of cases, higher in SDHB)

5. Follow-up

Postop catecholamine + metanephrine repeat at 4-6 wk to confirm biochemical cure; lifelong annual surveillance metanephrines (10-15% recurrence/metastasis rate); genetic counseling + family screening if SDHx or syndromic; annual BP + glucose + cardiac assessment if catecholamine cardiomyopathy

6. Sources

Guideline: Endocrine Society 2014 Pheochromocytoma & Paraganglioma CPG (Lenders JCEM 2014 PMID 24893135) + Lenders NEJM 2014 review (PMID 25006718) + 2025 ACC/AHA HTN (Whelton)

  1. pubmed.ncbi.nlm.nih.gov/24893135
  2. pubmed.ncbi.nlm.nih.gov/25006718
  3. pubmed.ncbi.nlm.nih.gov/12087161