Tumors of Adrenal Glands Flashcards
What is the function of the adrenal medulla?
Giant presynaptic nerve ending that releases catecholamines
How common are adrenal cancers? Prognosis?
rare–most are metastatic at diagnosis
What are the ssx of hormonally active adrenal cancer?
Hirsutism
Acne
Hypokalemia
HTN
What are the ssx of NON-hormonally active adrenal cancer?
Vague ssx–a abdominal pain or fullness
True or false: most of the adrenal cancers are metastatic at diagnosis
True
What is the survival rate of localized, regional, and distant adrenal tumors?
65% localized
44% regional
7% distant
What size of an adrenal mass on CT/MRI is concerning for adrenal CA?
4 cm or larger
Are intralesional calcifications of adrenal tumors a good or bad prognostic marker?
Bad
What CT findings are particularly concerning of adrenal tumors? (2)
- High CT attenuation values (dense)
- irregular shape
What are the lab studies that should be obtained for adrenal cancers? (3)
- Cortisol level
- Metanephrines
- Androgens/estrogens
What are the treatments for adrenal tumors? (2)
Surgical is curative
Palliative is advanced ds
What size of adrenal tumors may be taken out laparoscopically? Where on the body are these incisions made?
less than 6 cm in size
Incisions made in the flank or midline
What organs are commonly involved with adrenal CA?
- Liver
- Spleen/diaphragm
- Pancreas
- Colon
What is the role of chemo/radiation with adrenal cancer?
XRT not helpful
Chemo is controversial
What is the most common cause of Cushing’s disease?
Iatrogenic–exogenous corticosteroids
What are the causes of Cushing syndrome? (3)
- Long term steroid use
- ACTH overproducing
- Benign adrenal lesions
What ectopic cancers produce ACTH classically?
Small cell lung cancer
How do you diagnose Cushing’s? (2 steps, in sequence)
Check cortisol by urine or blood
If positive, then do ACTH suppression test
What is the purpose of doing an ACTH suppression test?
Localize to adrenal or ectopic/pituitary source
What is ACTH levels with an adrenal tumor?
Low
What is the result of an ACTH suppression test with a pituitary or ectopic tumor?
High with ectopic
Lower with pituitary
What is the best imaging modality to diagnose adrenal tumors?
CT of the adrenals
If pituitary, then MRI
What is the treatment for Cushing’s tumor?
If adrenal, then surgery
What should be given to pts in the perioperative period for a cortisol producing adrenal tumor?
Supplemental steroids
Who is most commonly affected with pheochromocytoma?
20-50 y/o women
Why do you want to diagnose and treat a pheochromocytoma prior to performing any surgery?
Can potentially cause a hypertensive crisis
Who usually is affected with pheochromocytoma: hyper or hypothyroid patients?
Hyperthyroid
Is the HTN caused by a pheochromocytoma paroxysmal or constant?
Paroxysmal
What is the average weight of pheochromocytomas?
100 g
What is the most common size of pheochromocytomas?
2-4 cm in size
True or false: there is a strong correlation between pheochromocytoma size and clinical ssx
False
What percent of pts with pheochromocytomas have sustained vs intermittent HTN?
75% sustained
25% intermittent
True or false: orthostatic hypotension is uncommon with pheochromocytoma
False
What are the four associated conditions with pheochromocytomas?
- NF1
- VHL ds
- Tuberous Sclerosis
- MEN2A/2B
What is the relative sensitivity/specificity of plasma metanephrines with a pheochromocytoma?
Very sensitive, pretty specific
What is the relative sensitivity/specificity of 24 hr urine catecholamines and metanephrines with a pheochromocytoma?
Pretty sensitive, but very specific
What is the role of CTs with pheochromocytomas?
Very accurate diagnosis of ds, as well as hepatic mets and extra adrenal sites
What is the organ of Zuckerkandl?
Neuroendocrine cells that form a pheochromocytoma located at the bifurcation of the aorta
What are the 2 drugs that should be administered prior to surgery for a pheochromocytoma? Why is the order specific for this?
Alpha blockade first w/ Phenoxybenzamine 1-2 weeks prior, then beta blockers.
If give beta blockers first, then over active alpha blockers
What is the treatment for the orthostatic hypotension with pheochromocytoma?
IVFs
When are beta blockers needed prior to surgery for a pheochromocytoma?
When there is persistent tachycardia after alpha blockade
Start after phenoxybenzamine and continue until operation
What is the most common surgery modality utilized for a pheochromocytoma?
Laparoscopic
What is the key structure that, when ligated, “deactivates” a pheochromocytoma, assuming it is on the adrenal gland
Renal vein
What should you do if you discover a pheochromocytoma while performing a surgery?
Complete other surgery fast, without manipulating the tumor
How do you control the HTN that can occur with a pheochromocytoma if you find one incidentally in surgery?
Nitroprusside and alpha/beta blockers postoperatively
What is primary hyperaldosteronism?
Failure to suppress aldosterone secretion
What is the MOA of aldosterone?
Maintains intravascular volume by exchanging K for Na and H
What is Conn’s syndrome?
Primary aldosteronism
What are the lab findings of Primary aldosteronism?
Drug resistant HTN
Hypokalemia
True or false: the majority of patients with primary hyperaldosteronism are normokalemic
True
What are the ssx of Conn’s syndrome?
- Muscle weakness/cramps
- polydipsia
- Polyuria
- Nocturia
- HA
What is the three tier diagnosis strategy for primary hyperaldosteronism?
- Initial screening
- Confirmation of dx
- Determine subtype
What is the treatment for aldosterone producing adenoma?
Surgery
What is bilateral idiopathic hyperaldosteronism? Treatment?
- idiopathic excess production of aldosterone from both adrenal glands
- Medical therapy with aldosterone antagonists
What percent of primary hyperaldosteronism is from an aldosterone producing adenoma?
50-75% of cases
What percent of primary hyperaldosteronism is from IHA?
25-50%
What is the relative incidence of aldosterone producing carcinomas?
Rare
What is the primary screening study for primary aldosteronism?
Aldosterone to renin ratio (will be high)
What is the urine aldosterone test?
-24 hr urine aldosterone w/ high salt diet for 5 days
What is the saline suppression test for primary hyperaldosteronism?
Saline given should suppress aldosterone, but if still high, then suspicious for hyperaldosteronism
What is the role of CT with primary hyperaldosteronism?
Determine if unilateral or bilateral
What is the role of renal vein sampling with primary hyperaldosteronism?
Renal sampling compares the aldosterone levels to determine the laterality of adrenal lesions
-used for when CT scans are equivocal
What is the treatment for APA?
Laparoscopic surgery
What is the treatment for IHA?
Subtotal adrenalectomy
What is the drug that is used to treat primary hyperaldosteronism medically?
Spironolactone
What are the post-op results with primary hyperaldosteronism treatment?
Serum K levels revert to normal within 24-72 hours
What percent of HTN is cured with surgery for primary hyperaldosteronism?
over 90%
True or false: malignancy is extremely rare with primary hyperaldosteronism?
True
What are incidentalomas?
Unsuspected adrenal masses on imaging for some other reason
What are the three questions that should always be asked with incidentalomas?
- Is it functional
- What is the malignant potential
- Does the pt have a primary CA, and could this be a met
What are the pertinent history bits to obtain with adrenal incidentalomas?
BP Weight change Cushing's ssx Virilization/feminization Occult malignancy
What are the lab tests that should be ordered with an incidentaloma?
Hypercortisolism
Hyperaldosteronism
Pheo
What must always be checked prior to performing an FNA on an adrenal mass?
r/o pheo
What percent of adrenal cancers are hormonally active? Inactive?
60% active, 40% inactive
What are the sizes of incidentalomas that suggest CA, vs not?
Less than 4 cm is not
More than 6 is suggestive
When is an FNA helpful in the diagnosis of an adrenal mass?
To determine if there’s mets
How do you follow an incidentaloma?
Serial CT every 3-6 months. No need for additional functional testing iff there are no ssx