Adrenal Pathology -Mesa Flashcards
What is the rate limiting step in the production of any steroid?
cholesterol–> pregnenolone
under the influence of ACTH
Why are functional malignancies rarely seen?
neoplastic processes tend to not have mature cells
won’t produce all of the hormones because they don’t have all of the necessary parts
What are the 2 most common causes of primary hyperaldosteronism?
cortical hyperplasia (60%) and cortical adenoma (Conn’s Syndrome -35%)
If a pt has too much androgen from the adrenal cortex is it more likely to be an adenoma or a carcinoma?
carcinoma*
this is different than most other functional neoplasms (normally adenomas)
What is the most common cause of congenital adrenal hyperplasia?
genetic defect resulting in 21-hydroxylase deficiency
can’t produce MC or GC so end up with a ton of testosterone (shunted)
What are the 3 types of adrenal insufficiency and what are the likely causes?
Primary acute (adrenal crisis from rapid withdrawal of GC, adrenal hemorrhage)
Primary Chronic (autoimmune–> Addison’s disease)
Secondary (decrease in ACTH–> pituitary or hypothalamus problem)
What are Crooke hyaline changes? What disease will they be seen in?
basophilic cytoplasm of ACTH producing cells (pituitary) changes from granular to homogenous due to the accumulation of intermediate filaments
seen in Cushings
What adrenal changes will be seen in Cushings?
atrophy of the fasciculata and reticular is but not glomerulosa
What is salt wasting syndrome? What genetic deficiency can this be seen with?
part of CAH (congenital adrenal hyperplasia) where there is hypotension shortly after birth from low aldosterone production–> low Na+ retention and increase serum K+
can cause cardiovascular collapse and death
can be seen with 21-hydroxylase deficiency (block the production of aldosterone and cortisol)
What is adrenogenital syndrome? How is it diagnosed?
adrenal secretes exces androgens causing changes toward adult masculinity in children or female adults
elevated DHEA
What is seen in 21-hydroxylase deficiency?Non-classic 21-hydroxylase deficiency?
21-hydroxylase:
block in aldosterone and cortisol, leading to elevated pregnanetriol, and elevated ACTH. Sx include virilizing syndrome, cortisol deficiency and variable salt wasting
Non-classic:
mild cortisol deficiency, excess adrenal androgens and no salt wasting
What would the cells in Waterhouse-Friderichsen Syndrome look like?
hemorrhage, necrosis, fibrin deposition, neutrophilic infiltration of medulla and cortex (zona glomerulosa may be spared)
due to the hemorrhagic necrosis of adrenal glands (often N. meningitides)
What mutation is associated with Type I polyglandular autoimmune syndrome? what are some characteristics of this?
21q22.3
autosomal recessive (type II is autosomal dominant)
Will aldosterone be affected in secondary renal insufficiency? Why?
no because it is under the control of renin and not ACTH
What are the proposed criteria for adrenal corticoadenoma malignancy in children?
>400 g >10.5 cm extension into soft tissue/vena cava -capsular or vascular invasion -tumor necrosis ->15 mitotic figures -atypical mitotic figures
