Case Presentation: Neuroendocrine tumors encompass a range of presentations, with pheochromocytomas (PCCs) accounting for most cases. PCCs are catecholamine-secreting tumors arising from the adrenal medulla, while paragangliomas (PGLs) represent a rarer subset (15-20%) originating from extra-adrenal chromaffin cells. Symptoms, including episodic or sustained hypertension, arise from catecholamine excess. Rarely, PGLs can present with intracerebral hemorrhage secondary to uncontrolled hypertension.We present a case of a 49-year-old male with a history of obstructive sleep apnea (OSA) and hypertension who presented with severe headache, dizziness, nausea, and vomiting. Despite compliance with a complex antihypertensive regimen, including spironolactone, hydralazine, irbesartan, propranolol, and clonidine, he had markedly elevated blood pressure on arrival. A CT scan revealed a left cerebellar hemorrhage, prompting urgent management.Initially, he was treated with a nicardipine drip for blood pressure control and received seizure prophylaxis. While neurologically stable (GCS of 14), his hypertension persisted, with systolic blood pressures (SBP) averaging in the 160s despite aggressive therapy with labetalol, hydralazine, and nicardipine. Subsequent imaging showed hemorrhage progression and new foci.After several days of intensive management with escalating antihypertensive therapy, including labetalol, losartan, clonidine, nifedipine, nicardipine drip, hydralazine, and minoxidil, the primary team investigated secondary causes of refractory hypertension. Renal artery stenosis and secondary hyperaldosteronism were ruled out. Elevated urinary metanephrines pointed to a catecholamine-secreting tumor. Imaging did not reveal an adrenal mass, but an MIBG scan confirmed a PGL near the cecum. Metyrosine was initiated to inhibit catecholamine synthesis, stabilizing the patient’s SBP to 130-140 mmHg. He was scheduled for outpatient surgical resection.
Discussion: Managing blood pressure in cases of pheochromocytoma or paraganglioma involves reducing SBP below 140 mmHg, aligning with the goals for intracerebral hemorrhage. However, medication selection becomes complex when these conditions coexist. For catecholamine excess, alpha-blockers like phentolamine are preferred, while beta-blockers must follow alpha blockade to avoid unopposed alpha stimulation. For intracerebral hemorrhage, labetalol and nicardipine are typically used, but agents like nitroprusside are avoided due to increased intracranial pressure risk.This patient’s case highlights the importance of broadening differential diagnoses when conventional treatments fail. Elevated urinary and plasma metanephrines remain the diagnostic gold standard for PCCs and PGLs. Imaging, such as MIBG scans, is critical when biochemical findings are positive but anatomical studies are inconclusive. Definitive management involves tumor resection preceded by 7-14 days of alpha blockade with agents like phenoxybenzamine, followed by beta-blockade for tachycardia.
Conclusions: Broadening the differential diagnosis is crucial, especially when standard aggressive treatments fail to control common conditions like hypertensive emergencies. While rare, catecholamine-secreting tumors such as PCCs and PGLs should remain on the radar in cases of refractory hypertension.