DeVirgilio Pt III Flashcards
What do the symptoms of hypertension, headache, perspiration, palpitation, and pallor suggest?
Five P’s of hyperadrenergic syndrome (i.e. - pheochromocytoma). Pressure, pain, perspiration, palpitation, pallor.
What genetic conditions is pheochromocytoma associated with?
MEN 2 disorders (MEN 2A: Pheo, Medullary thyroid ca, primary HPT; MEN 2B: Pheo, Medullary thyroid ca, mucosal neuromas, marfanoid habitus)
Neurofibromatosis 1: Neurofibromas, cafe au lait, lis nodules.
von Hippel Lindau: Retinal angioma, CNS hemangioblastoma, renal cell cancer, PNET (pancreatic neuroendocrine tumor), pancreatic and renal cysts
What cells are involved in a pheochromocytoma and where are they located?
Chromaffin cells
Adrenal medulla or sympathetic ganglia
What hormones does a pheochromocytoma produce?
Norepinephrine-predominant catecholamines
Epinephrine concentration is lower than Nor-epi
What activities or events can trigger a hypertensive crisis in pheochromocytoma patients?
Changes in blood flow, physical stimulation, tumor necrosis, anesthetic agents, foods containing tyramine, surgical manipulation
What feature determines the malignancy of a pheochromocytoma?
Malignancy is based on local invasion and/or metastasis, not cellular features
How is pheochromocytoma diagnosed?
Urine and/or blood levels of catecholamines and metabolites,
CT or MRI with contrast is first line imaging
What is the most sensitive test for pheochromocytoma?
Plasma free metanephrine
Very sensitive but higher false positive rate
A negative test can therefor rule out pheo (if it were there, a highly sensitive test would find it), but a positive test must be confirmed with urine testing (low specificity = lots of false positives)
What additional imaging may be ordered for pheo and when should it be ordered?
Nuclear imaging with iodine-mataiodobenzylguanidine (I-MIBG) is highly specific
Useful in cases of suspected multifocal or extra-adrenal disease
What is the treatment for pheo?
Medical conditioning with alpha blockade for at least 2 weeks
Add beta-blockade for tachycardia and/or arrhythmia
Surgical resection is curative (laparoscopic if less than 8cm and no malignant features on imaging)
What are the catecholamines?
Epinephrine
Norepinephrine
Dopamine
Patient with unexplained, severe hypertension and tachycardia intra-operatively?
Think undiagnosed pheochromocytoma
What are the breakdown products of epinephrine, norepinephrine, and dopamine?
Epinephrine: Metanephrine and vanillylmandelic acid (VMA)
Norepinephrine: Normetanephrine and VMA
Dopamine: Homovanillic acid (HVA)
What are the surgically correctible causes of HTN?
95% of HTN is primary. 5% may be corrected surgically and result from:
pheo
adrenal adenoma producing cortisol or aldosterone
renal artery fibromuscular dysplasia
aortic coarctation
What are the normal and abnormal functions of a1 and a2 receptors?
a1: smooth muscle contraction, gluconeogenesis, glycogenolysis - abnormal fxn: hypertension and hyperglycemia
a2: Smooth muscle contraction, platelet aggregation - abnormal fxn: Pallor
What are the normal and abnormal functions of b1 and b1 receptors?
b1: Chronotropic, inotropic, sweat glands - abnormal fxn: tachycardia, sweating
b2: smooth muscle relaxation - abnormal fxn: hypotension
Why do pheos rarely produce dopamine and what happens when they do?
Specific catecholamine ratios are dependent on the location of the pheo, which can develop anywhere along the sympathetic nervous system. Dopamine secreting tumors are usually found in extra-adrenal (paragangliomas) which do not occur very often. Dopamine tumors do not produce hypertension, but do have watery diarrhea due to dopamine’s effect on the GI system
What to suspect in a patient with severe hypotension or hypoglycemia after bilateral adrenalectomy? Test?
Addisonian crisis.
Check plasma ACTH