Pathology of the Adrenal II Flashcards
adrenal crisis
primary acute adrenocortical insufficiency
addison disease
primary chronic adrenocortical insufficiency
secondary adrenocortical insufficiency
low pituitary function
rapid withdrawal of steroids
primary acute adrenocortical insufficiency can occur
massive adrenal hemorrhage
damages the adrenal cortex causing primary acute adrenocortical insufficiency
newborns - following prolonged and difficult delivery with considerable trauma and hypoxia
patients on anticoagulant therapy
primary acute adrenocortical insufficiency
waterhouse friederichsen syndrome
complication of disseminated bacterial infection
leads to primary acute adrenocortical insufficiency
massive bilateral adrenal hemorrhage
infection, hypotension, widespread purpura
disseminated intravascular coagulation
can lead to waterhouse friederichsen syndrome
common infection with waterhouse friedrichsen
neisseria meningitidis
psendomonas
pneumococci
h. flu
staphylococci
addison disease
primary chronic adrenocortical insufficiency
autoimmune adrenalitis
autoimmune polyendocrine syndrome
type 1 and type 2
main cuases of autoimmune adrenalitis
infection with addison
tuberculosis and fungi
primary chronic adrenocortical insufficiency
metastatic neoplasms
may involve adrenals
-lead to addison disease
majority lung and breast**
tuberculous and fungal disease of adrenal
granolomatous inflammation
priamry autoimmune adrenalitis
irregularly shrunken glands
difficult to identify in suprarenal adipose tissue
metastatic carcinoma to adrenal
enlarged and obscured architecture
progressive weakness and fatigability
initial manifestations of addisons
doesn’t come to attention until glucocorticoids and mineralocorticoids are significantly decreased
anorexia, nausea, vomiting, weight loss, diarrhea
seen in addisons
hyperpigmentation
seen in addisons
-sun exposed areas and pressure points
cause of hyperpigmentation in addisons
elevated POMC from anterior pituitary
is precursor of both ACTh and MSH
hyperkalemia, hpyonatremia, volume depletion, hypotension
seen in addisons
potassium retention and sodium loss with decreased mineralocorticoids
waterhouse friedrichsen
acute adrenocortical insufficiency
addison
chronic adrenocortical insufficiency
APS1 mutation
AIRE
autoimmune attack against multiple endocrine organs and auto Abs against IL-17
secondary adrenocortical insufficiency
hypothalamus and pituitary dysfunction
reduced ACTH
NO hyperpigmentation**
-no elevation of MSH
no hyponatremia or hyperkalemia
in secondary adrenocortical insufficiency
-because near-normal aldosterone synthesis
just no cortisol and androgen output
secondary hypoadrenalism morph
decreased in size and still yellow color
adrenocortical neoplasms
adults - adenomas and carcinomas equally
children - more carcinomas
TP53 mutation
li fraumeni syndrome
familial cancer syndromes with adrenocortical carcinomas
epigenetic imprinting disorder
beckwith wiedemann syndrome
familial cancer syndromes with adrenocortical carcinomas
virilizing neoplasm of adrenal
carcinoma
hyperaldosteronism and cushing causing adrenal neoplasm
functional adenoma
bilateral adrenal enlargement
mets until proven otherwise
functionality of adrenocortical neoplasms
determined by clinical evaluation and measure of hormones and hormone metabolites
majority of adrenocortical adenomas
incidentalomas
adrenocortical carcinoma
associated with virilism
large and invasive lesions
lung cancer
mets to adrenals
metastasis to adrenal
more common than primary adrenocortical carcinoma
53yo M, feels bad, weight loss, smoker, bronchogenic lung cancer
b/l mets to adrenals
adrenal incidentaloma
incidental finding in asymptomatic patient
majority - of no clinical importance - nonsecreting cortical adenomas
needle biopsy of adrenal
NO - lots of blood supply
chromaffin cells
in adrenal medulla
adrenal medulla products
catecholamines - NE and E
paraganglion system
neuroendocrie cells dispersed in extra-adrenal system of clusters and nodules
bronchiomeric
intravagal
aorticosympathetic
pheochromocytoma
neoplasm of chromaffin cells
adrenal medulla noeplasm
neuroblastoma
neuronal neoplasm - in children
53yo intermittent HA, sweating, tremors, HTN, metanephrines on urine
pheochromocytoma
surgically correctable HTN
pheochromcytoma
also - renal artery stenosis
rule of 10s**
for pheochromocytoma
10% extra-adrenal - paraganglionomas
10% bilateral
10% biologically malignant
10% not associated with HTN
HTN with pheochromocytoma
2/3 have paroxysmal episodes
mutations for pheochromocytoma
RET NF1 VHL SDHD SDHC SCHB
adrenal colors**
yellow - cortex
red - medulla
zellballen
in pheochromocytoma
paroxysmal HTN in pheochromocytoma
precipitated by stress, exercise, postural changes, palpation in region of tumor
catecholamine cardiomyopathy
cardiac complications with pheochromocytoma
myocardial instability and ventricular arrhythmias
diagnosis of pheochromcytoma
increased urinary catecholamines, VMA, and metanephrines**
tx of pheochromocytoma
benign tumors - surgical excision
adrenergic block agents to prevent HTN crisis