Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content +++ Key Features ++ Uncommon; however, up to 10% of reported cases occur in pediatric patients Tumor can be located wherever chromaffin tissue (adrenal medulla, sympathetic ganglia, or carotid body) is present May be multiple, recurrent, and sometimes malignant Familial forms include pheochromocytomas associated with the dominantly inherited neurofibromatosis type 1, multiple endocrine neoplasia type 2, and von Hippel-Lindau syndromes, as well as mutations of the succinate dehydrogenase genes +++ Clinical Findings ++ Headache Sweating Tachycardia Hypertension Vasomotor instability (flushing and postural hypotension) Anxiety Dizziness, weakness Nausea, vomiting, diarrhea Dilated pupils, blurred vision Abdominal and precordial pain +++ Diagnosis ++ Serum and urine catecholamines are elevated Plasma-free metanephrine is the most sensitive and specific test, although phenoxybenzamine, tricyclic antidepressants, and β-adrenoreceptor blockers can cause false-positive results Levels three times the normal range are diagnostic Intermediate values may require additional testing with serum and urine catecholamines After demonstrating a tumor biochemically, imaging methods including CT or MRI are used to localize the tumor Nuclear medicine using functional ligands such as (123) I-MIBG, [18F]DA positron emission tomography scanning, and somatostatin receptor scintigraphy (with either [123 I]Tyr3-octreotide or [111 In] DTPA-octreotide) are useful in further diagnostic evaluation +++ Treatment ++ Laparoscopic tumor removal is the treatment of choice; however, the procedure must be undertaken with great caution and with the patient properly stabilized Oral phenoxybenzamine or intravenous phentolamine is used preoperatively Profound hypotension may occur as the tumor is removed but may be controlled with an infusion of norepinephrine, which may have to be continued for 1–2 days GET ACCESS TO THIS RESOURCE 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 Get Free Access Through Your Institution Contact your institution's library to ask if they subscribe to McGraw-Hill Medical Products. Access My Subscription GET ACCESS TO THIS RESOURCE Subscription Options Pay Per View Timed Access to all of AccessPediatrics 24 Hour $34.95 (USD) Buy Now 48 Hour $54.95 (USD) Buy Now Best Value AccessPediatrics Full Site: One-Year Individual Subscription $595 USD Buy Now View All Subscription Options