Professional Documents
Culture Documents
Absite CH 21 Adrenal PDF
Absite CH 21 Adrenal PDF
aorta
renal artery
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. 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:
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)
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)
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
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)