Today we discussed the case of a patient with the classic presentation of paroxysms of hypertension, headache (80%), palpitations (60%), and diophoresis (70%).
This is a rare diagnosis occuring in approximately 1-2 per 100,000. Approximately 10% will be extra-adrenal. About 10% of sporatic pheo will be malignant. Approximately 10% will have metastasis.
25% of patients may have a genetic predisposition to pheochromocytoma. Syndromes which include a propensity to pheochromocytoma include neurofibromatosis type 1, von hippel lindau, Multiple endocrine neoplasia types 1 and 2
A review of the condition and the diagnostic algorithm is available here.
Initially control of the BP should be achieved with alpha blockade, with agents like phenoxybenzamine (10 mg PO BID titrated every 2-3 days by 10-20mg until achieving effect), prazosin or terazosin. For long term use, prazosin or terazosin are theoretically preferred to phenoxybenzamine b/c of shorter half lives and fewer side-effects. They should be started 7-10 days pre-resection.
Beta-blockers are then added to control tachycardia and arrythmias -- but only after alpha blockade has been achieved.
Calcium channel blockers may safely be used as an adjunct for hypertension.
The goal SBP is 120 lying or sitting.
Pre-op patients should receive intravenous crystalloid to achieve a good ECF volume. This will help avoid post operative hypotension. The preferred operative route is to perform a laparascopic resection of the adrenal mass.
Catecholamine levels should return to normal by 10 days post op. Failure to decrease suggests a residual PHEO.
Post resection patients should be followed periodically for recurrence.
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