EOR endocrine part 1- pheochromocytoma, thyroid nodule, thyroid carcinoma Flashcards
What is pheochromocytoma?
Tumor of the adrenal medulla and sympathetic ganglion (from chromaffin cell lines) that produces catecholamines (NE>epi)
Which age group is most likely to be affected by pheochromocytoma?
Any age (children and adults) Average age is 40-60 yrs
Associated RFs of pheochromocytoma
MEN-II
FHx
von Recklinghausen dz
von Hippel-Lindau dz`
S/sx of pheochromocytoma
Classic triad: Palpitations HA Episodic diaphoresis Also: HTN Pallor, leading to: Flushing Anxiety Wt loss Tachycardia Hyperglycemia
What is the most common sign of pheochromocytoma?
HTN
What diagnostic tests should be performed in a pheochromocytoma?
Urine screen: VMA, metanephrine, and normetanephrine
Urine/serum epi/norepi levels
What are the other common lab findings of pheochromocytoma?
Hyperglycemia (epi increases glucose, norepi decreases insulin)
Polycythemia (resulting from intravascular volume depletion)
What is the MC site of a pheochromocytoma?
Adrenal >90%
What are the other sites of pheochromocytoma?
Organ of Zuckerkandl
Thorax (mediastinum)
Bladder
Scrotum
What are the tumor localization tests for pheochromocytoma?
CT scan MRI I-MIBG PET scan OctreoScan
How does the I-MIBG scan work?
I-MIBG is a norepi analog that colelcts in adrenergic vesicles and, thus, in pheochromocytomas
What is the role of PET scan in pheochromocytoma?
Positron Emission Tomography is helpful in localizing pheochromocytomas that do no accumulate MIBG
What is the scan for imaging adrenal cortical pheochromocytoma?
NP-59 (cholesterol analog)
What is the localizing option for pheochromocytoma if a tumor is not seen on CT, MRI, or I-MIBG?
IVC venous sampling for catecholamines (gradient will help localize the tumor)
What is the tumor site for pheochromocytoma if epinephrine is elevated?
Must be adrenal or near the adrenal gland because nonadrenal tumors lack the capability to methylate norepinephrine to epinephrine
Can histology be used to determine malignancy in pheochromocytoma?
No
Only distant metastasis or invasion can determine malignancy
What is the classic pheochromocytoma rule of 10s?
10% malignant 10% bilat 10% in children 10% multiple tumors 10% extra-adrenal
What is the preop/medical tx of pheochromocytoma?
Increase intravascular volume with alpha blockade (e.g., phenoxybenzamine or prazosin) to allow reduction in catecholamine-induced vasoconstriction and resulting volume depletion
Tx should start as soon as dx is made +/- beta blockers
What is the surgical tx of pheochromocytoma?
Tumor resection with early ligation of venous drainage and minimal manipulation
What are the possible periop complications of pheochromocytoma?
Anesthetic challenge: hypertensive crisis with manipulation (treat with nitroprusside)
Hypotension with total removal of the tumor
Cardiac dysrhythmias
In the pt with pheochromocytoma, what must be ruled out?
MEN type II (almost all cases are bilat)
What is the organ of Zuckerlandl?
Body of embryonic chromaffin cells around the abdominal aorta
Normally atrophies during childhood, but is the most common site of extra-adrenal pheochromocytoma