Pathology of the Pituitary and Adrenal Glands Flashcards by Joanne McEwan

what is the anterior pituitary also known as and where does it come from?

adenohypophysis

derived from Rathkes pouch

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what type of hormones does the anterior pituitary secrete>

trophic = TSH, ACTH, FSH, LH
non-trophic = GH and prolactin

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what is the posterior pituitary also known as and where does it come from?

neurohypophysis

extension of neural tissue consists of modified glial cells and axonal processes

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what cells are present in the anterior pituitary?

islands, cords of cells
acidophils
- somatotrophs (GH)
- mammotrophs (prolactin)
basophils
- corticotrophs (ACTH)
- thyrotrophs (TSH)
- gonadotrophs (FSH/LH)
chromophobes

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what cells are present in the posterior pituitary?

non-myelinated axons of neurosecretory neurons

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what anterior pituitary pathology can cause hyperfunction?

adenoma

carcinoma

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what anterior pituitary pathology can cause hypofunction?

surgery/radiation
sudden haemorrhage into gland
ischaemic necrosis (sheehan syndrome)
tumours extending into sella
inflammatory conditions (sarcoidosis)

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what posterior pituitary pathology can cause decreased ADH secretion?

diabetes insipidus

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what posterior pituitary pathology can cause increased ADH secretion?

SIADH

ectopic secretion of ADH by tumours
primary disorder in pituitary

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what is a pituitary adenoma and what causes it?

tumour derived from cells of anterior pituitary

can be sporadic or associated with MEN1 gene (wermer syndrome)

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what are the possible complications of a large adenoma?

visual field defects
atrophy of surrounding normal tissue
infarction leading to panhypopituitarism

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classification of pituitary adenoma?

by cell type/hormone produced
prolactin
ACTH
FSH/LH
GH

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name 3 functional pituitary adenomas

prolactinoma (most common)
growth hormone secreting (2nd most common)
ACTH secreting (cushings)

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possible features of functional pituitary adenomas?

prolactinoma = infertility, amenorrhoea, lack of libido
GH secreting = bone, cartilage and connective tissue growth - gigantism/acromegaly
ACTH secreting = cushings

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what usually causes pituitary hypofunction?

panhypopituitarism
causes
- granulomatous inflammation - sarcoidosis
- infarction (shehans)
- primary or metastatic tumours

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what is craniopharyngioma?

tumour derived from remnants of rathkes pouch

slow growing, often cystic, can calcify, most are suprasellar

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how do craniopharyngiomas present?

usually 5-15 yrs old or 60s-70s
headaches and visual disturbances
children may have growth retardation
excellent prognosis

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name 2 syndromes of the posterior pituitary?

diabetes insipidus
SIADH secretion
- paraneoplastic syndrome of ectopic ADH production

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what are the 2 types of diabetes insipidus?

central
- ADH deficiency
- trauma, tumours and inflammatory disorders of hypothalamus/pituitary
nephrogenic
- renal resistance to ADH effects

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what are the 2 compartments of the adrenal gland?

outer cortex
central medulla
(capsule surrounds gland)

what other diseases may manifest as adrenal pathology?

pituitary disease (ACTH from functional adenoma)
Shock/DIC
any condition which destroys adrenal tissue

3 causes of adrenal hyperfunction?

hyperplasia
adenoma
carcinoma

2 types of adrenal hypofunction and what causes them?

acute
- waterhouse-friderichsen
chronic
- addisons disease

what causes congenital adrenocortical hyperplasia and what happens as a result?

group of autosomal recessive disorders causing deficiency/lack of enzyme required for steroid synthesis
altered synthesis leads to increased androgen production
reduced cortisol stimulates ACTH release and cortical hyperplasia
- can cause masculinisation or precocious puberty

what causes acquired adrenocortical hyperplasia and what happens?

endogenous ACTH production - pituitary adenoma - ectopic ACTH (paraneoplastic syndrome from small cell lung cancer) causes bilateral enlargement of adrenal glands - can be diffuse (ACTH dependant) or nodular (ACTH independent)

what is adrenogenital hyperplasia?

congenital adrenal hyperplasia

how does an adrenocortical tumour usually present?

``` mainly adults more males incidental finding (imaging etc) hormonal affects mass lesion carcinomas with necrosis can cause fever ```

describe adrenocortical adenoma?

``` well circumscribed, encapsulated lesion 2-3 cm yellow/yellow brown cut surface (lipid) cells resembling adrenocortical cells can be functional but usually not small nuclei, rare mitosis ```

describe an adrenocortical carcinoma

more likely to be functional, virilising tumours usually malignant can resemble adenoma spread - local invasion - metastases (usually vascular - liver etc) - peritoneum and pleura - regional lymph nodes

what features can help distinguish adrenocortical carcinoma from adenoma?

``` large size (often >20cm) haemorrhage and necrosis frequent/atypical mitosis lack of clear cells (still cytoplasm etc within the cells) capsular or vascular invasion ```

what are the two types of adrenocortical hypofunction?

primary (acute/chronic) | secondary

what causes secondary adrenocortical hypofunction?

failure to stimulate adrenal cortex (e.g hypopituitarism) | suppression of adrenal cortex (steroid therapy)

what causes acute primary adrenocortical insufficiency?

rapid withdrawal of steroid treatment massive adrenal haemorrhage crisis in patients with chronic adrenocortical insfficiency

what causes chronic adrenocortical insufficiency?

``` addisons disease autoimmune adrenalitis infections metastatic malignancy unusual causes (amyloid, sarcoidosis, haemochromatosis) ```

when do symptoms occur in chronic adrenocortical insufficiency?

once 90% of gland is destroyed

what are the symptoms of addisons disease?

insidious onset weakness, fatigue, anorexia, nausea, vomiting, weight loss, diarrhoea pigmentation (raised POMC)

what are the biological features of addisons disease?

decreased mineralocorticoids - K+ retention, Na+ loss - volume depletion and hypotension hypoglycaemia

what can cause crisis in addisons disease?

``` stress - infection, trauma, surgery features - vomiting - abdo pain - hypotension - shock - death ```

describe the anatomical features of the adrenal medulla?

innervated by pre-synaptic fibres from sympathetic NS | neuroendocrine (chromaffin) cells secrete catecholamines

what tumours can occur in the adrenal medulla?

phaeochromocytoma | neuroblastoma

what is a neuroblastoma?

composed of primitive appearing cells but can show maturation and differentiation towards ganglion cells 40% in adrenal medulla, rest usually in sympathetic chain diagnosed <18 months

what is a phaeochromocytoma?

neoplasm derived from chromaffin cells of the adrenal medulla secrete catecholamines rare cause of secondary hypertension

what are the features of phaemochromocytoma?

paroxysmal episodes of hypertension - associated with stress, exercise, posture etc complications - cardiac failure, infarction, arrhythmias - CVA

how is phaemochromocytoma diagnosed?

detection of urinary excretion of catecholamines and metabolites

"10% tumour"?

``` phaeochromocytoma 10% extra-adrenal 10% bilateral 10% malignant 10% not associated with hypertension 25% are familial ```

what does a phaeochromocytoma look like?

range from small to large (1g - 2Kg) may see adrenal remnants on surface yellow, red/brown to haemorrhagic and necrotic K2Cr2O7 will turn tumour dark brown due to oxidation of catecholamines in tumour cells

benign vs malignant phaeochromocytoma?

malignant - tend to be large and necrotic - metastases = only definite evidence of malignancy

where is a phaeochromocytoma likely to metastasise?

skeletal regional lymph nodes liver long

phaeochromocytoma is a feature of what other neoplastic disease?

multiple endocrine neoplasia - MEN2A (sipple syndrome) - MEN2B