Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + • 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 +++ Epidemiology + • 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: -Multiple endocrine neoplasia type 2A (MEN 2A)-MEN 2B-Recklinghausen disease-von Hippel-Lindau disease• Pheochromocytomas are present in 40% of patients with MEN 2• 90% of patients with pheochromocytoma are hypertensive• Rule of 10s:- 10% malignant-10% familial-10% bilateral-10% multiple tumors-10% extra-adrenal• Hypertension less common in children• In children, 50% of patients have multiple or extra-adrenal tumors• Extra-adrenal pheochromocytomas:-Abdomen (75%)-Bladder (10%)-Chest (10%)-Pelvis (2%)-Head and neck (3%) +++ Symptoms and Signs + • 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 +++ Laboratory Findings + • Hyperglycemia• Elevated plasma metanephrines• Elevated 24-hour urine metanephrines and free catecholamines• Elevated urinary vanillylmandelic acid (VMA)• Elevated plasma catecholamines +++ Imaging Findings + • 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 +++ Rule Out + • Other causes of hypertension• Hyperthyroidism• Anxiety disorder• Carcinoid syndrome + • History and physical exam-Suspect pheochromocytoma based on symptoms• CT, MRI, or other scans• Plasma and urine studies (metanephrines, catecholamines, VMA)• Begin treatment with α-blockers• Possible MIBG scan• Operative excision of tumor +++ When to Admit + • Hypertensive crisis (can develop multisystem organ failure, mimicking severe sepsis) + • α-Adrenergic blocking agents should be started as soon as the biochemical diagnosis is established to restore blood volume, to prevent a severe crisis, and to allow recovery from the cardiomyopathy +++ Surgery +++ Indications + • All pheochromocytomas should be excised +++ Contraindications + • Metastatic disease• Inadequate medical preparation ( blockade) +++ Medications + • -Adrenergic blocking agents, such as phenoxybenzamine• Other agents include metyrosine, prazosin, and calcium channel blockers• ... Your MyAccess profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth