Pheochromocytoma
ICD-10 E27.5 · ICD-11 5A75

Treatment of Hypertensive Emergency or Urgency Due to Catecholamine Excess in Secreting Metastatic Pheochromocytoma/Paraganglioma

Secreting metastatic pheochromocytoma and paraganglioma (mPPGL) carry a risk of severe, acute cardiovascular events driven by uncontrolled catecholamine excess. Among the most critical presentations is hypertensive emergency or urgency, which requires immediate, structured pharmacological management.

This protocol applies to patients with secreting metastatic pheochromocytoma/paraganglioma who present with hypertensive emergency or urgency due to catecholamine excess. The cardiovascular manifestations of mPPGL encompass a range of serious events — including hypertensive emergency or urgency — that require urgent, targeted intervention.

Treatment Approach

Management of the acute hypertensive crisis is anchored by alpha-blockade as the primary pharmacological intervention. Depending on clinical response, additional antihypertensive agents may be incorporated. The complete regimen — including agent selection, sequencing, and clinical decision points — is detailed in the full protocol.

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References

DOI: 10.1097/MPA.0000000000001792

The hormonal manifestations of mPPGL are mainly cardiovascular and include catecholamine-induced cardiomyopathy (takotsubo), myocardial infarction, hypertensive emergency or urgency, shock, syncope, arrhythmia, dissecting aortic aneurysm, acute kidney injury, and hemorrhagic or ischemic stroke.

Treatment of hypertensive crisis is the same as for those with nonmetastatic disease and includes use of α-blockers with addition of β-blockers and calcium channel blockers as needed.

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