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Pheochromocytomas

Pheochromocytomas. Laura Grese, M4 UT College of Medicine. Introduction. Rare, catecholamine secreting tumors Most secrete epinephrine or norepinephrine Some secrete dopamine Derived from chromaffin cells Often associated with hereditary syndromes

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Pheochromocytomas

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  1. Pheochromocytomas Laura Grese, M4 UT College of Medicine

  2. Introduction • Rare, catecholamine secreting tumors • Most secrete epinephrine or norepinephrine • Some secrete dopamine • Derived from chromaffin cells • Often associated with hereditary syndromes • Multiple Endocrine Neoplasia (types 2a and 2b) • Von Hippel-Lindau syndrome • Neurofibromatosis type 1

  3. Clinical Presentation • Classically present with intermittent: • Headaches • Diaphoresis • Palpiations • Severe hypertension

  4. Clinical Presentation • Other symptoms include: • Tremor • Nausea • Weakness • Anxiety • Chest pain

  5. Clinical Presentation • Physical exam findings include: • Tachycardia • Hypertension (paroxysmal in 50% of cases) • Postural hypotension • Weight loss • Tremor • Fever

  6. “Rule of 10’s” • 10% Malignant • 10% Multiple • 10% Bilateral • 10% Extra-adrenal • 10% Familial • 10% Children

  7. Laboratory Diagnostic Tests • Plasma metanephrines • 96% sensitivity, 85% specificity • Test of choice for high risk patients • 24 hour urine collection of catecholamines and metanephrines • 87.5% sensitivity, 99.7% specificity • Test of choice for low risk patients

  8. Other Diagnostic Tests • Imaging Studies (to localize the tumor) • Abdominal CT • MIBG Scintigraphy • Genetic Testing

  9. Therapeutic Approach • Surgical resection is the treatment of choice • Preferred approach is laparoscopic adrenalectomy • Requires pharmacologic control of blood pressure preoperatively to prevent intraoperative crisis

  10. Preoperative Medical Management • Alpha blockade with phenoxybenzamine is initiated 10-14 days prior to surgery • Beta blockade is begun only after adequate alpha blockade (usually 2 days) • If initiated prematurely, unopposed alpha stimulation could precipitate a hypertensive crisis

  11. Follow-Up Post-Op • Plasma metanephrine testing • 2 weeks post-op • If within reference range, resection is complete • Yearly for 10 years if patient does not have a genetic syndrome (otherwise lifetime follow-up)

  12. Quiz • A 42 year old male presents with a severe headache. He states he has been suffering from severe headaches for months and they seem to be worsening. The headaches are self-limited and accompanied by palpitations and excessive sweating. His past medical history is significant for hypertension, which has been difficult to control. His blood pressure today is 180/110 and his heart rate is 110. • After appropriate tests to confirm your diagnosis, what is the next best step in management?

  13. Quiz • Laparoscopic adrenalectomy • Open adrenalectomy • Phenoxybenzamine • Propanolol • Sumatriptan

  14. References • http://0-www.uptodate.com.opac.uthsc.edu/contents/treatment-of-pheochromocytoma-in-adults?source=search_result&search=pheochromocytoma&selectedTitle=3%7E150 • http://emedicine.medscape.com/article/124059-treatment#aw2aab6b6b2

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