Endocrine Flashcards
mc pituitary adenoma?
prolacitnoma.
`prolactin dx?
> 150 (symptomatic)
sx of prolactinoma?
galactorrhea, irregular menses, decreased libido, infertility
mgmt prolactinoma?
asx and <1 cm, follow with MRI.
if sx or if >1cm, bromocriptine or cabergoline»_space;> transsphenoid resection if failed mgmt
what is deadly about acromegaly?
cardiac sx (valve dysfunction, cardiomyopathy).
acromegaly GH dx?
elevated IGF-1 > 10 in 90%; MRI
mgmt acromegaly?
transsphenoid resection, XRT, bromocriptine, octreotide, pegvisomant (GH-R antagonist)
Sheehan’s syndrome?
postpartum lactation issues, amenorrhea, AI, hypothyroid
anterior pit ischemia after peripartum hemorrhage
mgmt sheehan’s?
steroids, hormone replacement
pituitary apoplexy
bleeding into pituitary tumor with gland destruction; acute bleeding sx
treat: steroids emergently; hormone replacement
craniopharyngioma origin?
rathke’s pouch; benign calcified cyst.
mgmt craniopharyngioma?
resection (endocrine abnormalities, bitemporal hemianopsia, HA, hydrocephalus, etc.)
complication of craniopharyngioma resection
DI
Nelson’s syndrome?
after b/l adrenalectomy; increased CRH (enlarged pituitary).
sx: amenorrhea, bitemporal hemianopia, hyperpigmentation (B-MSH byproduct of ACTH)
mgmt Nelson’s
steroids.
Waterhouse Friderichsen syndrome?
adrenal gland hemorrhage after meningococcal sepsis leading to AI.
arterial supply to adrenals?
superior: inferior phrenic
middle: aorta
inferior: renal.
venous drainage from adrenals?
left: left renal
right: cava.
innervation to the adrenals?
cortex: none
medulla: from sympathetic splanchnics.
lymphatic drainage from adrenals?
subdiaphragmatic and renal LNs.
rate of adrenal incidentaloma?
1-2% of CTs. 5% are mets.
when to check for functionalty of adrenal mass?
ASAP, BEFORE IMAGING.
concerning adrenal incidentaloma findings
> 4 cm
10 HU
internal calcs/hemorrhage
high vascularity
heterogeneous
interval tumor growth
functional studies for incidentaloma?
urine metanephrines/VMA/catecholamines
urinary hydroxycorticosteroids,
serum K, plasma renin/aldosterone
low dose dexamethasone suppression test
indication for adrenalectomy?
- 4+ cm or ENLARGING 0.5 cm/6 mos
- functional
- non-homogenous on CT scan >10 HU
how to follow incidentaloma < 4 cm and nonfunctional and homogenous (AKA not going to resect)?
CT scan q3mo x 1 year then yearly.
how to access (post or ant) adrenal for resection?
anterior approach. reflect right lobe of liver or reflect spleen & pancreas.
common mets to adrenal?
LUNG»_space;» breast, melaoma, renal. must biopsy before.
isolated mets to adrenal? chemo or surgery.
just resect.
salt sugar sex. GFR
glomerulosa: aldosterone
fasciculata: glucocorticoids
reticularis: androgens/estrogens
where do hormones originate from? base.
cholesterol > progesterone > hormones x 3.
common hydroxylases?
21 and 11 B-hydroxylase.
posterior pituitary gland secretes what 2 things?
ADH and oxytocin alone.
congenital adrenal hyperplasia? 21
21 deficiency: MC. hypotensive man. increased 17-hydrogyproge
early puberty in M, virilization in F. salt wasting (hypoTN).
treat: cortisol + genitoplasty.
congenital adrenal hyperplasia? 11
11 deficiency. hypertensive man. increased 11-deoxycortisone, later…
early puberty M, virilization in F. salt saving (causes HTN)
treat: cortisol, genitoplasty.
Conn syndrome sx?
hypertension and hypokalemia (weakness, polydipsia, polyuria)
primary Conn (dx: renin)
MC from ADENOMA 85%.»_space;> hyperplasia > ovarian tumor, ca
renin is LOW
secondary hyperaldosteronism/Conn?
more common than primary Conn’s
etiology: CHF, RAS, liver failure, diuretics, Bartter’s (renin-secreting tumor)
renin is HIGH
Bartter’s syndrome
renin secreting tumor
how to dx primary Conn? 2 modalities
- salt load suppression test (urine aldosterone stays HIGH)
- AND aldosterone:renin > 25 (renin low)
then.. localize.
imaging for adrenal tumor?
CT scan
consider: MRI, NP-59 scintigraphy, venous sampling
when to consider adrenal scintigraphy?
when cross sectional is nondiagnostic.
NP-59 scintigraphy can show what
adenoma from hyperplasia
how to optimize prior to adrenalectomy
BP and K control.
adenoma tx
adrenalectomy
hyperplasia tx MC: conn
medical therapy first (spironolactone, ACEi, CCBs nifedipine, potassium)
then bilateral resection for refractory hypokalemia
what supplement will be required after bilateral adrenal resection?
fludrocortisone.
adrenal insufficiency Addison’s disease = hypocortisolism
#1 cause?
withdrawal of exogenous steroids
global: TB
developed: autoimmune
sx adrenal CRISIS
all loss…. refractory hypotension, fever, lethargy, pain, n/v
sx addisons disease
weakness, nausea, anorexia, weight loss, hyperK
2 cause hypocortisolism
autoimmune disease
labs for hypocortisolism?
- decreased AM cortisol? then proceed.
- ACTH stimulation test - cosyntropin – should stay LOW
- get AM ACTH level: if high, primary adrenal insufficiency; if low/nl , secondary adrenal insufficiency
dx hypocortisolism?
cosyntropic stimulation test. give ACTH, cortisol will remain LOW
how to tx acute adrenal crisis/insufficiency?
dexamethasone, fluids, tx the hyperkalemia & hypoglycemia
how to tx chronic adrenal insufficiency?
steroids
hypercortisolism = Cushing’s SYNDROME; #1 cause
IATROGENIC.
dx hypercortisolism?
24 hour urine cortisol and ACTH
ACTH low then primary (adrenal) secreting lesion
ACTH high then secondary (pituitary or ectopic)
if ACTH and cortisol is high on 24 hour urine collection?
high dose dexamethasone suppression test
pituitary adenoma will have SUPPRESSED cortisol “Cushing’s disease”
ectopic source (small cell lung ca) will not suppress
Cushing’s *DISEASE
= PITUITARY ADNOMA.
#1 non-iatrogenic cause of Cushing’s syndrome 80%
1 cause of noniatrogenic Cushing’s syndrome
Cushing’s disease - pituitary adenoma
dx Cushing’s disease
brain MRI
treat Cushing’s disease
transsphenoidal resection
radiation if too large for resection or if postop high cortisol that responds to HD dexa… there’s still some tissue there
dx ectopic ACTH secretion (failed high dose suppression test)
CT CAP to localize primary tumor
treat ectopic
resect primary and medically suppress if inoperable
adrenal cause of hypercortisolism? low ACTH. and high cortisol.
adenoma > hyperplasia
treat adrenal hypercortisolism adenoma vs hyperplasia
adenoma: adrenalectomy
hyperplasia: metyrapone (block cortisol synthesis), aminoglutethimide (inhibit steroid production), bilateral adrenalectomy if all else fails.
when to consider bilateral adrenalectomy without adrenal lesion
when cannot find ectopic tumor source
when ectopic tumor source is unresectable (and it’s slow growing)
when pituitary adenoma cannot be found
what age gets adrenocortical carcinoma?
before 5 YO
after 50 YO
bimodal!
F or M get adrenocortical carcinoma?
F
are adrenocortical caricinomas functional?
50% are.
treat adrenocortical carcinoma?
radical (take kidney) adrenalectomy with debulking
+ mitotane/external beam RT (chemoXRT) if high risk local recurrence (positive margin, invasion, intraop spillage, Ki67 > 10%)
role of debulking in adrenocortical carcinoma?
helps with sx. and prolongs survival.
how to treat recurrent, residual, or metastatic disease after adrenocortical carcinoma?
mitotane (adrenalytic + XRT
5yr survival rate adrenocortical carcinoma?
20%.
postop adrenalectomy if negative margins?
CT CAP q 3 mos x 2 years
if R1 adrenalectomy,/invasiom/spillage/Ki67 >10%>
postop chemoradiation with mitotane
neural crest cell to adrenal medulla catecholamines?
tyrosine >tyrosine hydroxylase> dopa > dopamine > NE >PNMT> Epi
VMA vanillylmandelic acid
byproduct of epinephrine to metanpehrine and norepi to normetapnephrine via MAO.
pheo origin
slow growing from sympathetic ganglia or ectopic neural crest cells
10% rule of pheo
10% malignant, bilateral, kids, familial, extraadrenal
pheo laterality
R>L
dx pheo?
- plasma non specific
- confirm with urine metanephrines (24 hr) and VMA
- localize CT vs hyperintense T2 MRI vs mIBG
adjunct pheo diagnostics?
131Iodinine-MIBG (norepi analgoue) can ID location if CT/MRI cannot help
clonidine suppression will have no effect on the elevated hormones (used as adjunct if all levels are normal)
venography in pheo dx?
DO NOT DO. can cause hypertensive crisis.
preoperative preparation for pheo removal?
a-blocker first (phenoxybenzamine, prazosin) and volume replacement then B block if tachycrdic or arrhythmias ssen
mgmt of pheo?
adrenalectomy (ligate veins first to avoid spilling catecholamines)
debulking role in pheo?
helps with sx
metyrosine role in pheo?
can give preop or in unresectable.
inhibits tyrosine hydroxylase to decrease synthesis of catecholamines.
extraadrenal 10% mostly malignant; locations?
MC: aortic bifurcation = origin IMA = organ Zuckerkandl
other: vertebral bodies, bladder
extramedullary tissue: organ of Zuckerkandl
inferior aorta near bifurcation
falsely elevated VMA
coffee, tea, fruit, vanilla, iodine contrast, labetalol, a/B-blocker
pharyngeal arches that make up the thyroid?
1st and 2nd.
byproduct of T3 to T4 conversion?
cAMP
superior thyroid aa origin?
1st branch of ECA.
inferior thyroid aa origin?
off thyrocervical trunk; supplies both inferior and superior parathyroids.
Ima aa?
1%.
arises from innominate or aorta; traverses towards isthmus.
venous drainage from thyroid?
superior/middle thyroid: into IJ
inferior: into innominate.
innervation around thyroid?
external branch superior laryngeal nerve, recurrent laryngeal nerve.
external branch superior laryngeal nerve function?
motor to cricothyroid muscle
INJURY = loss of PROJECTION and voice FATIGUABILITY.
where dose superior laryngeal nerve run?
lateral to thyroid lobes. close to superior thyroid aa.
MC injured nerve in thyroidectomy?
superior laryngeal nerve.