Adrenal Gland Pathology Flashcards
how much do adrenal glands weigh
4-5 grams each
where do the adrenal glands sit
superior and medial to upper pole of kidneys (embedded in perineal fat)
what are the adrenal glands composed of
outer cortex and inner medulla
what 3 zones are is the adrenal cortex split up into
zona glomerulosa, zona fasciculata and zona reticularis
what is produced and secreted in the zona glomerulosa
mineralocorticoids and aldosterone
what is produced and secreted in the zona fasciculata
glucocorticoids and cortisol
what is produced in the zona reticularis
sex steroids and glucocorticoids
where is the adrenal medulla
central core of adrenal (extension of nervous system)
what innervates the adrenal medulla
pre-synaptic fibres from sympathetic nervous system
what do neuroendocrine (chromaffin) cells secrete
catecholamines
what causes adrenocortical hyperfunction
hyperplasia, adenoma, carcinoma
what causes adrenocortical hypofunction
acute: Waterhouse-Friderichsen syndrome
chronic: Addison’s disease
what is the sequence of events leading to congenital adrenocortical hyperplasia
group of autosomal recessive disorders, deficiency/lack of enzyme required for steroid biosynthesis, altered biosynthesis leads to increased androgen production (masculinisation or precocious puberty), reduced cortisol stimulated ACTH release and cortical hyperplasia (10-15X normal. weight)
what can cause acquired adrenocortical hyperplasia
endogenous ACTH production (pituitary adenoma (cushings disease) or ectopic ACTH secretion (paraneoplastic syndrome from small cell lung cancer)), bilateral adrenal enlargement (up to 30g), diffuse or nodular (diffuse-ACTH driven and nodular-usually ACTH independent)
who gets adrenocortical tumours
usually adults
what syndrome is associated with younger patients with adrenocortical tumours
Li-Fraumeni syndrome (germline mutation of p53 gene)
one feature of this syndrome is an association with adrenocortical tumours
how does an adrenocorical tumour present
incidental, hormonal effects if functional, mass lesion (has to be quite big-can undergo central necrosis), carcinomas with necrosis can cause fever (fever with no known cause is. sign of malignancy)
are adrenocortical adenomas benign or malignant
benign
how are adrenocortical adenomas usually found
incidentally
what is characteristic of adrenocortical adenomas
well circumscribed, encapsulated lesions, well differentiated, small nuclei, rare, mitoses, yellow
how big are adrenocortical adenomas
2-3cm (won’t present as mass lesion)
what cells are adrenocortical adenomas composed of
cells resembling adrenocortical cells
are adrenocortical adenomas usually functional or non-functional
non-functional (cause no problems)
are adrenocortical carcinomas common or rare
rare
are adrenocortical tumours usually functional or non-functional
functional (virtualising tumours usually malignant)
are adrenocortical adenomas or carcinomas more likely to be function
adrenocortical carcinomas
what is associated with the spread of adrenocortical carcinomas
local invasions (retroperitoneum, kidney), metastasis (vascular: liver, lung and bone (usually first sign of metastatic disease)), peritoneum and pleura and regional lymph nodes (tends to be vascular over lymphatic though)
what is the 5 year survival rate and mortality of adrenocarcinomas
20-35% at 5 years, 50% dead in 2 years (could be due to arising in relatively silent site, by the time it is found not a lot that can be done)
what is the only criteria to distinguish between benign and malignant tumours
metastasis
what are some features to suggest carcinoma over adenoma
large size (>50g often >20cm in max dimension), haemorrhage and necrosis, frequent mitoses, atypical mitoses, lack of clear cells, capsular or. vascular invasion
what causes primary. hyperaldosteronism
Conn’s syndrome, usually 60% associated with diffuse or nodular hyperplasia of both adrenal glands, adenoma, rarely carcinoma, glucocorticoid remediable
what causes secondary hyperaldisteronism
increased renin, decreased renal perfusion, hypovolaemia, pregnancy
what causes hypercortisolism
cushings,
