Endo-pituitary pathology Flashcards
A lactotroph is more commonly ____ granulated vs ___ granulated on histology
MC sparsely granulated vs densely granulated on histology
Northern Ireland has an large number of ____ gene carriers and therefore ___ patients
AIP gene carriers causing acromegaly patients (via somatotroph)
“Irish Giants”
is size important in terms of a corticotroph
size can be misleading, a very small corticotrophin adenoma can be very hyper functional and present with all sx of Cushings syndrome
USP8 somatic mutation seen in corticotrophs result in an upregulation of _____
EGFR
sign and sx of apoplexy into pituitary
sudden onset of excruciating HA, diplopia due to pressure on oculomotor nerves, and hypopituitarism
(can be a result of mass effect)
most common cause of cushing syndrome (not disease)
iatrogenic (healthcare associated)
-due to long term glucocorticoid administration (steroids)
how to dx somatotrophs by pathology
- Diffuse growth (sheet like: common in all adenomas)
- confirmation made with GH immunohistochemistry (immunostain for somatotroph)
- can be sparsely or densely granulated
- can be mixed with PRL-Secreting cells (mammosomatotroph)
define central diabetes insipidus ? sx? labs? how to dx? tx?
- insufficient ADH secretion due to posterior pituitary or hypothalamus pathology (tumor, trauma, infection, inflammation)
- results in decreased reclaimed free water from the renal collecting system
- sx: polyuria, polydipsia, life threatening dehydration
- Labs: hypernatremia, high serum osmolality, low urine osm and specific gravity
- dx: water deprivation test fails to increase urine osm. or desmopression tx shows increased urine sodium/osm
tx: desmopressin
tx of somatotrophs
somatostatin analogs
GH receptor antagonists
surgery
**SOMATOSTAIN inhibits GH
tx for corticotroph
somatostatin analog, bromocriptine (dopamine agonist), or surgery
**corticotrophs can express dopamine and somatostatin receptors
define Nelson Syndrome
- enlargement of of pituitary tumor (corticotroph adenoma) after surgical removal of both hyperplastic adrenal glands (used to tx hypercortisol secretion/Cushing syndrome from high ACTH)
- main sx: hyperpigmentation (ACTH affect on melanocyte stimulation)
how do gonadotrophs usually present
usually non-functioning or “silent”/”null cell” adenoma
-usually present with mass effect due to minimal function
presentation of lactotroph adenoma in women vs men
WOMEN
- present earlier due to menstrual irregularities, galactorrhea, decreased libido, infertility, and possible mass effect if large
- *responsible for >20% of cases of amenorrhea
MEN
-more present later with mass effect symptoms. early sx are decreased libido and decrease sperm count
what somatic genetic mutation are linked to pituitary adenomas? what familial mutations?
somatic:
1. GNAS - somatotrophs
2. USP8- corticotrophs
familial:
1. AIP - somatotrophs
(AIP gene causes FIPA- Familial isolated pituitary adenomas; via PAP- pituitary adenoma predisposition)
size of microadenoma vs macroadenoma vs giant adenoma of the Pituitary gland, and what are the implications?
micro- <1cm
macro- 1-4cm
giant- >4cm
*size is VERY important to determine how one presents clinically and extent of disease (i.e with mass effect, etc)
identify the nomenclature of the pituitary gland (anterior, posterior lobe, etc)
anterior lobe = adenohypophysis (vascular connections to hypothalamus)
posterior lobe = neurohypophysis (neuronal connection to the hypothalamus)