1.2mg/d. Can see falsely elevated levels if patient is on labetolol or buspirone (metabolite of buspirone mistakenly read as metanephrine).6# Plasma catecholamines: Must be drawn under strict guidelines including patient fasting, supine for 30 minutes, and needle inserted at least 20 minutes prior to blood draw. Norepinephrine >2000pg/ml or epinephrine >200pg/ml is 95% specific and 85% sensitive. Metanephrine and normetanephrine plasma measurement are probably the most sensitive because these metabolites released directly from adrenals (>90% and 24-40%, respectively).# Clonidine suppression test: indicated if urine and plasma studies non-diagnostic and history still suggestive. Biopsy: contraindicated. 6Radiologic tests:# CT scan: 87% sensitive because it may miss tumors < 1cm which means in MEN-2 can miss up to 25%.# MRI: 98-100% sensitive, bonus ability to differentiate an adrenal pheo from other adrenal masses by T2 weighted images showing hyperintensity of pheos.6# I131-MIBG scan: scan detect tumors missed by CT or MRI and is excellent for evaluation of metastasis. Patients should not be on phenoxybenzamine prior to scan.6# DOPA-PET scan: can detect tumors in unusual locations most sensitively and specifically. 6# Adrenal Vein sampling; only needed when work up is inconclusive .Rx:# Surgical removal is Rx of choice and are now being successfully removed laparoscopically.# Pre-operative Rx: Treat hypertension and volume contraction.# Hypertensive Rx:- Alpha blockade with phenoxybenzamine, a long acting alpha pre and post-synaptic adrenergic antagonist. 10mg BID and increase to 0.5mg/kg daily in divided doses as needed to control HTN for 2-3weeks prior to surgery. Hold the morning of surgery.Prazosin may be used but requires more frequent dosing. Phenoxybenzamine can be used as definitive therapy for inoperable and malignant tumours.- Beta blockade can be added to treat tachycardia after adequate alpha blockade has been started. Labetolol best with its alpha 1 and beta-adrenergic antagonist activity.- Metyrosine (alpha-methyl-para-tyrosine) inhibits catecholamine synthesis.- Calcium channel blockers may be used if beta-blocker not tolerated.# Highest risk periods are during anesthesia induction and when tumor is manipulated. Nitroprusside should be available. Post-operatively hypotension and shock may occur. Hypoglycemia may develop in 10-15% secondary to release of suppression of insulin by excess catecholamines. Catecholamine secretion should return to normal in about one week.Prognosis:# Can be cured with surgery, although recurrence rate 14% in one series, with half of those being malignant. Risk of recurrence highest in familial disorders. Pain from metastatic bone lesions can be relieved with radiation therapy +/- chemotherapy. Disorders of Adrenal MedullaPheochromoclytoma# Neoplasm of neural crest-derived chromaffin cells that secrete catecholamines (usually norepinephrine) - hypertension# Highest incidence in children and adults age 30-50 - Clinical: paroxysmal or constant hypertension is most classic symptom; also, sweating, headache, arrhythmias, palpitations# Lab values: elevated urinary homovanillic acid (HVA) and vanillylmandelic acid (VMA)# The Rule of 10s for pheochromocytoma:- 10% extra-adrenal- 10% bilateral- 10% malignant- 10% affect children- 10% familialNeuroblastoma# Most common malignant extracranial solid tumor of childhood# Occurs most frequently in the adrenal medulla, but may arise in sympathetic chain# Amplification of the N-myc oncogene--more copies=more aggressive# Clinical: tumors grow rapidly, metastasize widely (especially to bone); prognosis in younger patients (less than 1 year old) better than for older children# Pathology: lobulated with areas of necrosis, hemorrhage, calcification# Microscopic appearance: rosette pattern of small cells">