• Episodic headache, excessive sweating, palpitations, and visual blurring
• Hypertension, frequently sustained, with or without paroxysms
• Postural tachycardia and hypotension
• Elevated urinary catecholamines or their metabolites, hypermetabolism, hyperglycemia
• Less than < 0.1% of patients with hypertension
• 5% of tumors discovered incidentally on CT scan
• Most occur sporadically
• Associated with familial syndromes, such as:
• Pheochromocytomas are present in 40% of patients with MEN 2
• 90% of patients with pheochromocytoma are hypertensive
• Rule of 10s:
- 10% malignant
-10% multiple tumors
• Hypertension less common in children
• In children, 50% of patients have multiple or extra-adrenal tumors
• Extra-adrenal pheochromocytomas:
-Head and neck (3%)
• Clinical findings are variable
• Episodic or sustained hypertension
• Triad of palpitation, headache, and diaphoresis
• Anxiety, tremors
• Weight loss
• Dizziness, nausea, and vomiting
• Abdominal discomfort, constipation, diarrhea
• Visual blurring
• Tachycardia, postural hypotension
• Hypertensive retinopathy
• Elevated plasma metanephrines
• Elevated 24-hour urine metanephrines and free catecholamines
• Elevated urinary vanillylmandelic acid (VMA)
• Elevated plasma catecholamines
• Adrenal mass seen on CT or MRI
• Characteristic bright appearance on T2-weighted MRI
• Asymmetric uptake on MIBG scan. Particularly useful for extra-adrenal, multiple, or malignant pheochromocytomas. Not useful for sporadic biochemical syndrome with unilateral mass
• Avoid arteriography or fine-needle aspiration as they can precipitate a hypertensive crisis
• Early recognition during pregnancy is key because if left untreated, half of fetuses and nearly half of the mothers will die
• History and physical exam
• CT, MRI, or other scans
• Plasma and urine studies (metanephrines, catecholamines, VMA)
• Begin treatment with α-blockers
• Possible MIBG scan
• Operative excision of tumor
• -Adrenergic blocking agents, such as phenoxybenzamine
• Other agents include metyrosine, prazosin, and calcium channel blockers
• β-Adrenergic blocking agents can be used only after full blockade has been achieved
• Avoid opioids ...
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