Treatment of Hormonally Functional Secreting Metastatic Pheochromocytoma with High Catecholamine Production
Clinical Scenario
This protocol addresses patients with a hormonally functional (secreting) metastatic pheochromocytoma or paraganglioma (mPPGL) characterised by high catecholamine production. Excess catecholamines are associated with hypertension, diaphoresis, headaches, and palpitations, as well as orthostatic hypotension, hyperglycaemia, and anxiety.
Perioperative Considerations
All patients with a hormonally functional secreting mPPGL are recommended to undergo preprocedural blockade beginning 7 to 14 days before surgery or any procedure — and before most localised and systemic therapies — to prevent periprocedural cardiovascular complications.
Treatment Approach (partial)
Management centres on alpha-adrenergic receptor blockade as the first-choice intervention, with further agents and supportive measures incorporated based on cardiovascular response and catecholamine load.
Treatment Targets
- Blood pressure <130/80 mm Hg while seated
- Systolic BP >90 mm Hg while standing
- Heart rate <90 bpm before procedures
- Heart rate 70–80 bpm for long-term control
References
DOI: 10.1097/MPA.0000000000001792
- High catecholamine production is associated with hypertension, diaphoresis, headaches, and palpitations among other symptoms and signs such as orthostatic hypotension, hyperglycemia, and anxiety.
- Given this knowledge, we recommend that all patients with a hormonally functional secreting mPPGL undergo preoperative or preprocedural blockade for 7 to 14 days before surgery/procedure and for most localized and systemic therapies to prevent periprocedural cardiovascular complications.
- There is no consensus on which agents to use; however, retrospective studies support the use of α-adrenergic receptor blockers as the first choice and calcium channel blockers as second choice.
- Independent of medication regimen, treatment should also include a high-sodium diet and increased fluid intake.
- There is no evidence from randomized controlled trials to determine the optimal target BP, but based on retrospective studies and expert experience, the goal should be a target BP of at least less than 130/80 mm Hg while seated and greater than 90 mm Hg systolic while standing with a target heart rate of less than 90 bpm before procedures and closer to 70–80 bpm, if possible, for long term control.