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Internal Medicine Residents Rounds

Internal Medicine Residents Rounds. Juan Rivera, MD. Pheochromocytomas . Rare tumors Less than 0.2% of HTN 2 / million / year More frequent in autopsy series (250 – 1300 / million) Aldosteronism is much more common Pheos should be suspected, confirmed, localized, and resected because:

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Internal Medicine Residents Rounds

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  1. Internal Medicine Residents Rounds Juan Rivera, MD

  2. Pheochromocytomas • Rare tumors • Less than 0.2% of HTN • 2 / million / year • More frequent in autopsy series (250 – 1300 / million) • Aldosteronism is much more common • Pheos should be suspected, confirmed, localized, and resected because: • HTN is curable • Paroxysms can be lethal • 10% are malignant • Occur in both sexes and at any age, but more in the 4th – 5th decades of life

  3. Who should be screened? • Episodic or very labile HTN • Shock or severe pressor responses with: • induction of anesthesia, intubation, surgery, parturition, invasive procedures and antihypertensive drugs • Adverse CV response to certain drugs: • Anesthetics, glucagon, TRH, ACTH, naloxone, beta blockers, TCA, histamine.

  4. Who should be screened? • Spells of: headache, tachycardia, diaphoresis, anxiety attacks (c/s HTN) • Occurring during exercise, twisting of the torso, straining (Valsalva), coitus, or micturition. • Family history of pheochromocytoma or MTC • Hypertensive patients with: • Unexplained weight loss, diaphoresis, constipation, orthostatic hypotension, seizures, cold hands and feet • Incidental adrenal or abdominal mass

  5. Perspiration Palpitation Pain Pallor Pressure Plethora of manif: tremor, anxiety, N&V, weakness, wt loss, dyspnea, warmth, visual disturb, dizziness, orthostatism, constipatn, paresthesias, BG, painless hematuria. Rarely: flushing (not a typical feature) Triggers: postural change, anxiety,exercise. Duration: 10-60 min Flushing Abdo pain: Intermittent intestinal obstruction or ischemia Diarrhea Bronchospam Hypotensionor rarely HTN Nasal congestion & periocular edema Symptoms ofpulmonic stenosis or tricuspid insufficiency Triggers:excitement, alcohol ingestion, catecholamines, chocolates. Duration: 2 – 5 min Spells Phenotypes Pheo Carcinoid Mastocytosis • Flushing and facial warmth • Pallor (if abrupt  BP) • Palpitations • Lightheadedness or syncope • Dyspnea and c/p • N,V,D • Pruritus • Fatigue and profound lethargy after the spell • Triggers: heat, exertion, emotional upset, ethanol, narcotics, anticholinergics, radiocontrast agents. • Typical duration: 15-30mi • Frequency: daily to 3 times annually

  6. Endocrine: Thyrotoxicosis Menopausal syndrome Medullary thyroid ca Hypoglycemia Cardiovascular: Labile essential HTN Renovascular disease Angina Psychiatric: Anxiety & panic attacks Somatization disorders Miscellaneous: Polycytemia Vera Pharmacologic Antidepressants (SSRI) Sildenafil Niacin CCB Opiates Tamoxifen, bromocriptin Cocaine, LSD Vancomycin EtOH + metronidazol / Ketoconazol / chlorpropamide /cephalosporines Neurologic POTS Autonomic neuropathy Migraine headache Seizure disorder Other causes of spells

  7. Non-classical Symptoms • Abdominal pain • Vomiting • Dyspnea • Heart failure • Hypotension • Sudden death • Fever (IL-6) • Cushings sd

  8. Other findings • Leucocytosis • Hyperglycemia (CA) • Hypercalcemia (PTHrp) • Hypokalemia (CA) • Erythrocytosis (Erythropoietin)

  9. What is the best screening test? • Options: • Resting plasma catecholamines (CA) • Plasma Metanephrines (MN) • 24h urine CA + MN • We use 24h-urine CA + MN

  10. From indwelling cath, supine x 30min Total CA (N<4.2 nmol/L) > 12  dx 6-12 highly sugg False positive are frequent Medications, food, stress Acidified container If omitted, keep cold, process immediate Urine MN: Least susceptible to interference(labetalol, buspirone) > 6,500 nmol/d  highly suggest ~ 90% specific VMA: unacceptably high false negative rate (41%) Screening Tests Plasma CA Urine CA + MN Plasma MN • Very sensitive (97-99%) • Specificity is low (82%) • except in hereditary syndromes (96%)

  11. A 72 y.o. male • Admitted because of colon ca • Abdomen CT shows a 12cm mass on the right adrenal • Endo sees the patient and concludes that this is most likely metastasis. Cosyntropin test normal. • 24h-urine CA + MN: normal except for DA = 32,000 (N < 2,630) • What’s going on?

  12. A 72 y.o. male with Colon ca and an adrenal mass • Patient on treatment with Sinemet for Parkinson

  13. Interference • DRUGS • Acetaminofen • Benzodiazepines • Buspirone • Diuretics • Levodopa • Sympathomimetics • TCA • Vasodilators • Tetracycline and quinidine • Ethanol • FOODS AND OTHERS • Bananas (NE) • Caffeine • Nicotine • Exercise Westphal: Am J Med Sci, Volume 329(1).January 2005.18-21

  14. An 82 y.o. male • While on vacation in Florida consults because on dyspnea • A chest CT notes a 3 cm mass in the right adrenal • Patient otherwise healthy, “never sick”, completely asymptomatic, BP 100/60, physical exam unremarkable • 24h-urine CA + MN: • E  25 (-136); NE  3678 (-591) • MN  645 (281-1841); NMN 21740(502-2531)

  15. Why are some PHEO patients normotensive despite high circulating cathecolamines? • Chronically hypovolemics • Increase production of vasodilator agents (DOPA acting in the CNS; dopamine acting on mesenteric and renal vessels; prostaglandins) • Adrenergic desensitization: down-regulation of alpha-1 receptors • Polymorphism in beta-2 receptors that allows continued b2-mediated vasodilation

  16. Can radiology help?

  17. MRI CT

  18. Transverse CT scans obtained in 49-year-old woman with adrenal pheochromocytoma. (a) Nonenhanced scan obtained at level of middle portion of left adrenal gland shows well-defined mass (arrows) with isoattenuation relative to liver parenchyma. (b, c) Contrast-enhanced scans obtained at same level as in a. The tumor (arrows) has heterogeneous enhancement on (b) the 1-minute scan and homogeneous enhancement on (c) the 10-minute scan. Tumor attenuation is 56 HU in a, 107 HU in b, and 94 HU in c. Thus, the absolute percentage of enhancement loss in this tumor is 25%, and the relative percentage of enhancement loss is 12%.

  19. Pre-op • A 39-year-old woman comes to your office complaining of episodic anxiety, headache, and palpitations. She states that without dieting she has lost 15 pounds over the past 6 months. Physical examination is normal except for a blood pressure of 200/100 mm Hg and a resting pulse rate of 110 bpm. Chart review shows that prior blood pressures have always been normal, including one 6 months ago. The diagnosis of pheochromocytoma is confirmed, and a 3 cm mass is visualized on the right adrenal by MRI. • Question: • How do you prepare her for OR?

  20. General Measures • High PO fluid intake at home • Generous hydration 24-48h pre-op once admitted • Teach self-monitoring of BP (BID & PRN)

  21. Medical Therapy • CCB: • Nicardipine, Amlodipine, Nifedipine or verapamil • Advantages: better tolerated, less perioperative fluids, fast and effective during paroxysms, in vitro nifedipine reduces proliferation of pheo cells • Alpha-adrenergic blockade • Phenoxybenzamine: start 10 mg/day, increase q3d to 30-60 mg/d • Problems: takes longer to control BP, worse post-op hypotension, SE (dry mouth, h/a, diplopia, nasal congestion), not during pregnancy (accumulates in the fetus). • Selective alpha-1: Doxazosin, prazosin • Advantages: short acting (prazosin< doxazosin), less reflex tachy, less post-op hypotension

  22. Medical Therapy • ACE inhibitors and ARBs • CA  ^ renin  etc • Pheos have ACE binding sites • Should not be use as sole agent • Beta blockers • Only after alpha-blockade or CC blockade • Metoprolol, labetalol, propranolol • Metyrosine • Inhibits tyrosine hydroxylase • Used as adjuvant when uncontrolled HTN prior to surgery • Reported to reduce intraop HTN and arrhythmias • Postop hypotension may be more severe for several days • SE:sedation, psychiatric, nightmares, diarrhea, galactorrhea, extrapyramidal sx, crystalluria and urolithiasis

  23. That’s itThanks

  24. Sorry… THAT’S NOT THE CORRECT ANSWER

  25. YOUR ANSWER IS CORRECT!!!

  26. Pathophysiologic & Clinical Manifestations of CA Excess1 Target Tissue Physiologic Effect Pathophysiologic Manifestations Clinical Manifestations Heart Increased heart rate Tachycardia Palpitations Tachyarrhythmia Angina pectoris Increased contractility Increased myocardial O2 consumption Angina pectoris Myocarditis Congestive heart failure Cardiomyopathy Blood vessels Arteriolar constriction Hypertension Headache Congestive heart failure Angina pectoris Venoconstriction Decreased plasma volume Dizziness Orthostatic hypotension Circulatory collapse Gut Intestinal relaxation Impaired intestinal motility Ileus Obstipation Pancreas (B cells) Suppression of insulin release Carbohydrate intolerance Hyperglycemia Glucosuria Liver Increased glucose output Carbohydrate intolerance Hyperglycemia Glucosuria Adipose Lipolysis Increased free fatty acids Weight loss Skin (apocrine glands) Stimulation Sweating Diaphoresis Bladder neck Contraction Elevated urethral pressures Urinary retention Most tissues Increased basal metabolic rate Increased heat production Heat intolerance Sweating Weight loss 1Modified, with permission, from Werbel SS, Ober KP: Pheochromocytoma: Update on diagnosis, localization, and management. Med Clin North Am 1995;79:131.

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