CHAPTER 21- ADRENAL

1. Whats the blood supply to the superior adrenal?

inferior phrenic artery

2. Whats the blood supply to the middle adrenal?

aorta

3. Whats the blood supply to the inferior adrenal?

renal artery

4. Where do the right/left adrenal veins drain to?

o right adrenal vein drains to - inferior vena cava
o left adrenal vein drains to- left renal vein
5. Asymptomatic adrenal mass:

1) 1-2% of abdominal CT scans show incidentaloma (5% are metastases or

primary adrenal tumors)

2) Benign adenomas are common

3) adrenals are also common sites for metastases

o 4) surgery is indicated if mass has:

o 1- ominous characteristics (nonhomogenous)
o 2- >4-6 cm
o 3- functioning
o 4- enlarging

5) need to follow every 3 months for 1 year, then yearly

o 6) Dx:
o 1- serum K
o 2- urine metanephrines/VMA/catecholamines
o 3- urinary hydroxycorticosteroids
o 4- plasma renin + aldosterone levels if HTN or decreased K
o 5- CXR
o 6- stool guiac and colonoscopy
 o 7- mamogram

7) anterior approach for adrenal cancer resection

6. Common metastases to adrenal:

o 1) lung cancer (#1)
o 2) breast cancer
o 3) melanoma
o 4) renal Ca

Cancer history with asymptomatic adrenal mass- needs biopsy

7. Adrenal cortex:
o 1) from mesoderm
o 2)
o Glomerulosa: aldosterone
o Fasciculata- glucocorticoids
o Reticularis- androgens/estrogens
o 3) Cholesterol--> progesterone--> androgens/cortisol/aldosterone
o 4) all zones have 21 and 11-beta hydroxylase
8. What is the innervation to the adrenal cortex + medulla:
o 1) no innervation to the cortex
o 2) medulla receives innervation from the splanchnic nerves
9. What is the lymphatic drainage of the adrenal cortex:

1) lymphatics drain to subdiaphragmatic and renal lymph nodes

10. Cortisol pathway:

o 1) corticotropin-releasing hormone (CRH) is released from the
hypothalamus and goes to anterior pituitary gland
o 2) ACTH is released from the anterior pituitary gland and causes the
release of cortisol
o 3) cortisol has a diurnal peak at 4-6am
11. Aldosterone pathway:
o 1) Aldosterone stimulates:
o 1- renal sodium resorption
o 2- secretion of K, H, and ammonia
 o 2) Aldosterone secretion is stimulated by:
o 1- angiotensin II
o 2- hyperkalemia
o 3- to some extent ACTH
12. What should you be suspicious of with excess androgens and estrogens by
adrenals?

almost always cancer

13. Congenital adrenal hyperplasia

Enzyme defect in cortisol synthesis

o 1) 21-Hydroxylase deficiency:
o 1- 90%, most common
o 2- precocious puberty in males, virilization in females
o 3- increased 17-OH progesterone, leads to increased production of
testosterone
o 4- is salt wasting (decreased sodium, increased potassium) and causes
hypotension
o 5- Tx: cortisol, genitoplasty

o 2) 11-Hydroxylase deficiency
o 1- precocious puberty in males, virilization in females
o 2- increased 11-deoxycortisone
o 3- is salt saving (deoxycortisone acts as a mineralocorticoid) and causes
hypertension
o 4- Tx: cortisol, genitoplasty

o 3) 17-hydroxylase deficiency
o 1- ambiguous genitalia at birth
o 2- salt saving
14. Symptoms of Hyperaldosteronism (Conn's syndrome):
o 1) HTN secondary to sodium retention without edema
o 2) hypokalemia
o 3) also have weakness, polydipsia, polyuria
15. Primary hyperaldosteronism
o 1)low renin
 o 2) adenoma (80-90%)--> #1 cause of primary hyperaldosteronism
o 3) hyperplasia (10-20%)
o 4) ovarian tumors (rare)
o 5) cancer (rare)
16. Secondary hyperaldosteronism
o 1) high renin
o 2) more common than primary disease
o 3) Caused by:
o 1- CHF
o 2- renal artery stenosis
o 3- liver failure
o 4- pregnancy
o 5- diuretics
o 6- Bartter's syndrome (renin-secreting tumor)
17. Diagnosis for primary hyperaldosteronism
o 1) urine aldosterone after salt load test (will stay high)
o 2) decreased serum K, increased urine K, increased serum Na, metabolic
alkalosis
o 3) plasma renin activity will be low
o 4) Aldosterone: renin ratio >20
18. Localizing studies for hyperaldosteronism
o 1) MRI
o 2) NP-59 scintigraphy (shows hyperfunctioning adrenal tissue;
differentiates adenoma from hyperplasia; 90% accurate)
o 3) adrenal venous sampling if others nondiagnostic
19. Treatment for Hyperaldosteronsism
o 1) adenoma- resection has good results with adrenalectomy
o 2) hyperplasia seldom cured (increased morbidity with bilateral
resection)
o 1- try medical therapy first with hyperplasia using: spironolactone,
calcium channel blockers, and potassium
o 2- if bilateral resection is performed (usually done for refractory
hypokalemia) patient will need fludrocortisone postoperatively
20. Hypocortisolism
o 1) adrenal insufficiency, addison's disease
o 2) #1 cause - withdrawal of exogenous steroids
o 3) #1 primary disease- autoimmune disease
o 4) Also caused by:
o 1- pituitary disease
o 2- infection
o 3- adrenal hemorrhage
 o 4- adrenal metastasis
o 5- surgical resection or injury
o 5) get decreased cortisol and aldosterone
o 6) Dx: decreased serum Na, increased serum K, ACTH stimulation test
21. Acute adrenal insufficiency:
o 1) hypotension
o 2) fever
o 3) lethargy
o 4) abdominal pain
o 5) decreased glucose
o 6) decline in mental status
o 7) nausea/vomiting
o 8) increased K
22. Treatment of hypocortisolism:
o 1) dexamethasone
o 2) fluids
o 3) ACTH stimulation test (measure cortisol level after test)
23. What is the most common cause of hypercortisolism (cushings syndrome)

most commonly iatrogenic

24. Diagnosis of cushing's syndrome

o 1st- 24 hr urine cortisol (most sensitive test)
o 2nd- low-dose overnight dexamethasone suppression test; look to see
whether it suppresses cortisol production(measure in urine). If cortisol is
low, the diagnosis is Cushing's disease (the pituitary adenoma was
suppressed). If cortisol remains high, go to 3rd.
o 3rd- measure serum ACTH. If high, have either ectopic ACTH or
pituitary tumor that was not suppressed with lose-dose overnight
dexamethasone suppression test, go to 4th. If low ACTH, patient has
cortisol-secreting tumor (ie. adrenal tumor or adrenal hyperplasia).
o 4th- if serum ACTH high--> high-dose overnight dexamethasone
suppression test (positive=pituitary origin, negative=ectopic origin of
ACTH). In 20% you still cannot tell; to to 5th
o 5th- CRH test--> pituitary adenomas will increase ACTH; ectopic
producers will have no change in ACTH

o - MRI useful
o - NP-59 scintography localizes adrenal tumors and can help differentiate
them from hyperplasia
25. Pituitary adenoma (cushing's disease)
o 1) #1 noniatrogenic cause of cushing's syndrome--> 70-80% of cases
o 2) cortisol should be suppressed with either low-or high-dose
dexamethasone suppression test
o 3) mostly microadenomas
o 4) need petrosal sampling to figure out which side; MRI can help also
o 5) vertical incisions to find adenoma
o 6) Tx: most tumors removed with transsphenoidal approach;
unresectable or residual tumors treated with XRT
26. Ectopic ACTH
o 1) #2 noniatrogenic cause of cushing's syndrome
o 2) most commonly from small cell lung cancer
o 3) cortisol is not suppressed with either low or high-dose dexamethasone
suppression test
o 4) Chest and abdominal CT can help localize
o 5) Tx: resection of primary if possible; medical suppression or bilateral
adrenalectomy for inoperable lesions
27. Adrenal adenoma
o 1) #3 noniatrogenic cause of cushing's syndrome
o 2) decreased ACTH, unregulated steroid production; does not suppress
o 3) Tx: adrenalectomy
28. Adrenocortical carcinoma
o 1) rare cause of cushing's syndrome
o 2) Tx: radical adrenalectomy; debulking can help patients and prolong
survival
29. Adrenal hyperplasia (macro or micro)

Tx: bilateral adrenalectomy

30. Medical therapy for ectopic ACTH production or adrenocortical cancer with
residual or metastatic disease after resection:
o 1) ketoconazole and metyrapone- inhibit steroid formation
o 2) aminoglutethimide- inhibit cholesterol conversion
o 3) Op-DDD (mitotane)- adrenal-lytic, used for metastatic disease
31. Bilateral adrenalectomy
o 1) may be needed in patients with ectopic ACTH from tumor that is
unresectable or from pituitary adenoma that cannot be found.
o 2) need to remember to give steroids postoperatively
32. Adrenocortical carcinoma
o 1) bimodal distribution (before age 5 and in the 5th decade)
o 2) more common in females
o 3) 50% are functioning tumors
 o 1- cortisol
o 2- aldosterone
o 3- sex steroids
o 4) children display virilization 90% of the time (precocious puberty in
boys, viriluzation in females)
o 5) feminization in men; masculinization in women can occur
o 6) Symptoms: abdominal pain, weight loss, weakness
o 7) 80% have advanced disease at time of diagnosis
o 8) Tx:
o 1- radical adrenalectomy
o 2- mitotane for residual or recurrent disease
o 9) 20% 5 year survival rate
33. Adrenal medulla:
o 1) from ectoderm neural crest cells
o 2) catecholamine production:
o tyrosine--> dopa-->dopamine--> norepinephrine--> epinephrine
o 3) Tyrosine hydroxylase- rate-limiting step (tyrosine to dopa)
o 4) PNMT- enzyme that converts norepinephrine to epinephrine (requires
methylation)
o 1- enzyme is found only in the adrenal medulla (exclusive producers of
epinephrine)
o 5) Only adrenal pheochromocytomas will produce epinephrine
34. MAO (monoamine oxidase):
o 1) converts norepinephrine to normetanephrine
o 2) epinephrine to metanephrine
o 3) VMA produced from these
35. Extra-adrenal rest of neural crest tissue can exist:
o 1) usually in the retroperitoneum
o 2) most notably in the organ of Zuckerkandl
36. Pheochromocytoma
o 1) chromaffin cell
o 2) rare, usually slow growing, arise from sympathetic ganglia or ectopic
neural crest cells
o 3) 10% rule:
o 1- malignant
o 2- bilateral
o 3- in children
o 4- familial
o 5- extra-adrenal
37. What can pheochromocytomas be associated with:
o 1) MEN IIa
 o 2) MEN IIb
o 3) von Recklinghausen's disease
o 4) tuberous sclerosis
o 5) sturge weber disease
38. Is there a predominant side in pheochromocytosis?

right-sided predominance

39. Whats more likely malignant, extra adrenal or adrenal pheochromocytomas

extra-adrenal tumors more likely malignant

40. Symptoms of pheochromoctytoma:

o 1) HTN (frequently episodic)
o 2) headache
o 3) diaphoresis
o 4) palpitations
41. Diagnosis of pheochromocytoma:
o 1) urine metanephrines and VMA- breakdown products of epi and
norepinephrine
o 2) VMA most sensitive
o 3) MIGB scan (norepinephrine analogue)- can help identify location if
having trouble finding tumor
o 4) clonidine suppression test- tumor does not respond, keeps
catecholamines high
o 5) no venography- can cause hypertensive crisis
o 6) CT/MRI can help localize tumors
o 7) not able to localize--> still proceed with laparotomy
42. Preoperatively (pheochromocytoma)
o 1) volume replacement
o 2) alpha-blocker first (phenoxybenzamine--> avoids hypertensive crisis)
o 3) then beta-blocker if patient has tachycardia/arrhythmias
o 4) need to be careful with beta-blocker and give after alpha-blocker-->
can precipitate hypertensive crisis (unopposed alpha stimulation) and
heart failure in patients with cardiomyopathy
43. Treatment of pheochromocytomas:
o 1) check for other tumors at resection
o 2) ligate adrenal veins first to avoid spilling catecholamines during
tumor manipulation
o 3) debulking helps symptoms in patients with unresectable disease
o 4) metyrosine- inhibits tyrosine hydroxylase causing decreased
synthesis of catecholamines
 o 5) should have the following ready during the time of surgery:
o 1- nipride
o 2- neo-synephrine
o 3- anti-arrhythmic agents (e.g. amiodarone)
44. Postop conditions after pheochromocytoma surgery:
o 1) persistent hypertension
o 2) hypotension
o 3) hypoglycemia
o 4) bronchospasm
o 5) arrhythmias
o 6) intracerebral hemorrhage
o 7) CHF
o 8) MI
45. Other sites of pheochromocytomas:
o 1) vertebral bodies
o 2) opposite adrenal gland
o 3) bladder
o 4) aortic bifurcation
46. Where is the most common site of extramedullary tissue?

organ of zuckerkandle (inferior aorta near bifurcation)

47. Falsely elevated VMA

o 1) coffee + tea
o 2) fruits
o 3) vanilla
o 4) iodine contrast
o 5) labetalol
o 6) alpha + beta blockers
48. Extramedullary tissue

responsible for medullary Ca of the thyroid and extra-adrenal

pheochromocytoma

49. Ganglioneuroma
o 1) rare
o 2) benign
o 3) asymptomatic tumor of neural crest origin in the adrenal medulla or
sympathetic chain
50. Indications for Adrenalectomy
o Unilateral Adrenalectomy
o 1) aldosteronoma
 o 2) cortisol-secreting adenoma (cushing's syndrome or subclinical
cushing)
o 3) unilateral pheochromocytoma (sporadic or familial)
o 4) virilizing or feminizing tumors
o 5) non functioning unilateral tumor
o 1- size: >4-5cm
o 2- imaging features atypical for adenoma, myelolipoma, or cyst
o 3- adrenocortical carcinomas
o 4- solitary unilateral adrenal metastasis

o Bilateral Adrenalectomy
o 1) bilateral pheochromocytomas
o 2) cushing's syndrome from
o 1- bilateral nodular adrenal hyperplasia
o 2- ectopic ACTH-producing tumor unresponsive to primary therapy
o 3) cushing's disease (pituitary tumor) unsuccessfully treated by
surgery/radiation
51. Diagnosis and preoperative preparation of common adrenal tumors (pg 133)