- 27 yo F BIBEMS for AMS
- Progressively altered and emotionally labile over past week, per family
- No PMH/PSH/Medications
Abd: Soft, NT, ND
Studies
CBC/BMP, U/A unremarkable
bHCG neg
TSH 0.14, T4 0.85
Trop 0.180
Utox neg
EKG: Sinus tachycardia, TWIs in lateral leads, no ST deviations
NCHCT: No acute findings
CT A/P: Adrenal mass c/w pheochromocytoma
Course: Patient with likely hypertensive encephalopathy/demand ischemia due to catecholamine excess. Admitted to CCU. 24-urine with elevated metanephrines. Treated with cyclobenzamine and nicardipine; started on propranolol on HD 2.
PHEOCHROMOCYTOMA
- Catecholamine-secreting tumor
- Occur in 0.2% of patients with hypertension
- Age: 30-40s
- Associated syndromes: VHL, MEN2, NF1
- “Rule of 10’s”
- 10% bilateral
- 10% extra-adrenal
- 10% familial
- 10% malignant
- Symptoms caused by hypersecretion of one or combinations of norepinephrine/epinephrine/dopamine
- Classic triad: Episodic HA, sweating, tachycardia
- Most do not have all three
- HA occurs in 90% of symptomatic patients
- Sweating occurs in 60-70%
- Paroxysmal HTN most common sign
- Some patients may present hypotension that may be 2/2:
- Volume depletion
- Abrupt cessation of excessive catecholamine secretion
- Desensitization of adrenergic receptors
- Hypocalcemia
- Paroxysms may be triggered by:
- Micturition (urinary bladder pheochromocytoma)
- Drugs: Glucagon, IV contrast, tyramine, reglan
- Biochemical testing
- Should be performed in all patients with suspected pheochromocytoma
- Imaging
- CT A/P
- Detects almost all sporadic tumors
- Sn = 98-100%, Sp = 70%
- Consider using low-osmolar contrast as contrast may induce a paroxysm
- In ED setting, this will typically precede biochemical testing, though biochemical testing is recommended as first step in work-up of suspected pheo
- NB: 95% are within abdomen, 10-15% extra-adrenal tumors
MIBG
- May detect tumors not seen on CT/MRI
- Performed in patients with pheochromocytomas >10 cm due to increased risk of malignancy
- CT A/P
- Alpha blockade
- Phenoxybenzamine preferred agent
- Beta blockade
- Should be started only after alpha blockade initiated due to concern for unopposed alpha
- Propranolol may be used
- Calcium Channel Blockers
- Typically used as additional agent after alpha blockade
- May be used as single agent
- Nicardipine typically used
- Surgery
- Definitive treatment is lap adrenalectomy
Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet 2005;366:665-75.
Young WF, Jr. Clinical practice. The incidentally discovered adrenal mass. N Engl J Med 2007;356:601-10.
Young, Williams. “Clinical presentation and diagnosis of pheochromocytoma.” Up To Date. http://www.uptodate.com, 24 Feb. 2015. Web. 16 Mar. 2016. http://www.uptodate.com/contents/clinical-presentation-and-diagnosis-of-pheochromocytoma
Young, Williams. “Treatment of pheochromocytoma in adults.” Up To Date. http://www.uptodate.com, 27 Oct. 2015. Web. 16 Mar. 2016. http://www.uptodate.com/contents/treatment-of-pheochromocytoma-in-adults