Pheochromocytoma

Question 1

What is pheochromocytoma

Answer 1

Catecholamine producing tumors increasing epinepheirne and norepi in system

Question 2

Cause of paroxysmal symptoms in Pheo

Answer 2

PAROXYSMAL RELEASE OF HORMONES
◦ Can last <1 hour
◦ Surgery
◦ Positional changes
◦ Exercise
◦ Pregnancy
◦ Urination
◦ Various medications

Question 3

Cause of HTN crisis and death in pheo

Answer 3

HYPERTENSIVE CRISIS & FATAL CARDIAC
ARRHYTHMIAS
◦ Spontaneously
◦ Needle biopsy or manipulation of a mass
◦ IV contrast
◦ Glucagon injections
◦ Vaginal delivery
◦ Trauma
◦ Surgery

Question 4

What are the hallmark symptoms of pheochromocytoma

Answer 4

allmark Manifestations
◦ Palpitations, sweating &headache
◦ Accompanied with hypertension
◦ Episodic or sustained
Signs & Symptoms
◦ Anxiety, pale skin, tachycardia & palpitations

Question 5

What are manifestations of pheochromocytoma in HTN crisis

Answer 5

Hypertensive Crisis
◦ Heart failure, pulmonary edema, arrhythmias &ICH
◦ Aneurysm dissection
◦ Ischemic bowel

Question 6

What is effected in pheochromocytoma crisis

Answer 6

Pheochromocytoma Multisystem Crisis
◦ Hyper- or hypotension
◦ ARDS
◦ Acute heart failure
◦ Kidney dysfunction
◦ Liver failure

Question 7

Lab testing for pheochromocytoma

Answer 7

Laboratory
◦ 24-hour urine fractionated metanephrines
◦ >2.2 mcg of total metanephrine per mg of creatinine
◦ >135 mcg total catecholamines per gram of creatinine
◦ Plasma fractionated free metanephrines
◦ 3x upper limit of normal
◦ Serum CgA
◦ Present in 85% of patients
◦ Correlates with tumor size
◦ Not specific

Question 8

What is Chlonidine testing in pheochromocytoma

Answer 8

Clonidine Suppression Testing
◦ Measure plasma fractionated free metanephrines
◦ Before the administration of Clonidine 0.3 mg &3 hours after
◦ Fall in plasma normetanephrines
◦ Rule out the presence of tumor

Question 9

Imaging for pheochromocytoma

Answer 9

Imaging
◦ CT vs MRI
◦ Similar sensitivity
◦ Performed with contrast
◦ MRI somewhat better detecting extraadrenal
◦ Tumors detected in adrenal medulla

Question 10

Medical management in pheochromocytoma

Answer 10

Alpha blockers (i.e. Phenoxybenzamine 20-40 mg PO BID/TID)
• Calcium channel blockers (i.e. Nifedipine ER 30 mg PO QD)
• **Beta blockers (i.e. Propranolol 10 mg PO TID/QID)

Question 11

Surgical management of pheochormocytoma

Answer 11

Complete tumor removal
• Ultimate therapeutic goal
• Partial or total adrenalectomy
• Preserve adrenal cortex
• Minimally invasive techniques
• Standard surgical approach
• Preoperative preparation
• Maintain blood pressure <160 / 90 mmHg
• Intraoperative
• Nipride infusion avoid hypertensive crisis
• IVF for hypotension
• Postoperative
• Check ACTH