Pituitary Path Flashcards
Three manifestations of pituitary disease
Hyperpituitarism
Hypopituitarism
Local mass effect
Describe the local mass effect of the pituitary
Increased ICP, bitermoral hemoanopsia, pituitary apoplexy
What it this?
Acute hemorrhage into adenoma, leading to rapid enlargement of lesion. Neurosurgical emergency.
Pituitary apoplexy
Three causes of hyperpitutarism
Pituitary carcinoma
Hypothalamic disorders
PITUITARY ADENOMA
Name the Adenoma subtype: galactorrhea and amenorrhea (F); sexual dysfunction and infertility.
Lactotroph adenoma, silent lactotroph adenoma
Name the Adenoma subtype: giantism (chidlren) , acromegaly (Adults)
Densely granulated somatotroph adenoma, sparsely granulated somatotroph adenoma, silent somatotroph adenoma
Name the Adenoma subtype: combined features of GH and prolactin excess
Mammosomatotroph adenoma
Name the Adenoma subtype: Cushing Syndrome and Nelson Syndrome
Corticotroph Adenoma
Name the Adenoma subtype: Hyperthyroidism
Thyrotroph adenoma, silent thyrotroph adenoma
Name the Adenoma subtype: hypogonadism, mass effects, hypopotutarism
Gonadotroph adenomas, silent gonadotroph adenoma (“Null cell”, oncocytic adenomas)
Pituitary tumor most commonly associated with GNAS GoF mutation
GH adenoma (40% of somatotroph adenomas)
Pituitary tumor most commonly associated with PRKAR1A GoF mutation
GH and PRL adenomas
*Sporadic
Pituitary tumor most commonly associated with Cyclin D1 GoF mutation
Aggressive adenomas
Pituitary tumor most commonly associated with HRAS GoF mutation
Pituitary carcinomas
Pituitary tumor most commonly associated with MEN1 LoF mutation
GH, PRL, ACTH adenomas
*Sporadic
Pituitary tumor most commonly associated with CDKN1B LoF mutation
ACTH adenomas
*Sporadic
Pituitary tumor most commonly associated with AIP LoF mutation
GH adenomas (esp in people less than 35 years old)
*Sporadic
Pituitary tumor most commonly associated with RB LoF mutation
Aggressive adenomas
Acute hemorrhage into an adenoma associated with clinical evidence of rapid enlargement of the lesion. A neurosurgical emergency.
Pituitary Apoplexy (gonadotroph adenoma, hypopituitarism,
Transcription factor expressed on stem cells that differentiate into ant-pit hormones: somatotrophs, mammosomatotrophs, and lactotrophs
PIT-1
Transcription factors (two) expressed on stem cells that differentiate into gonadotrophs
SF-1 and GATA-2
Cellular _____ and absence of significant _____ _____ distinguishes pituitary adenomas from nonneoplastic anterior pituitary parenchyma?
Monomorphism
Reticulin netowks
What genetic characteristic of Atypical Adenomas result in higher propensity for aggressive behavior like invasion and recurrence?
TP53 mutatin
Chromophobic cells with _____ of PIT-1 TF are known as ______ granulated lactrotroph adenomas
juxtanuclear localization
Sparsely granulated lactotroph adenoma
Acidophilic cells with _____ PIT-1 expression are known as ____ granulated lactotroph adenomas.
Diffuse cytoplasmic
Densely granulated lactotroph adenoma
Ranging from isolated psammoma bodies to extensive calcification of virtually the entire lactotroph adenoma tumor mass (“pituitary stone”) – due to what?
Propensity of Lactotroph adenomas to undergo dystrophic calcification
Major clinical manifestation of Lactotroph adenomas
1/4 of all amenorrhea is caused by this
Age/sex of people who present with lactotroph adenoma
Female, 20-40 years old
GF associated with Somatotroph adenoma
IGF-1. Because perisitently elevated levels of GH stimulate hepatic secretion fo this.
Hyperostosis - define
Increase in bone density in spine and hips, seen in acromegaly.
Prognathism - define
Enlargement of jaw and resultant protrusion, as well as broadening of lower face, seen in acromegaly.
Six associations with GH excess (other than gigantism and acromegaly).
- Gonadal dysfunction
- Muscle weakness
- HTN
- Arthritis
- CHF
- Increased GI cancer risk
seven major causes of hyperPRL-emia other than lactotroph adenomas.
- Pregnancy
- Nipple stimulation
- Stress response
- Lactotroph hyperplasia
- Suprasellar compartment mass
- Renal failure HypOthyroidism
What type of cells present when somatotrophs adenomas and corticotroph adenoomas when densely granulated?
basophilic, monomorphic
Both dense and sparsely granulated stain positively for what?
PAS
Syndrome when a large destructive pituitary adenoma develops after surgical removal of adrenal glands for tx of Cushing Syndrome. Results in loss of inhibitory effect of adrenal CS on preexisting corticotroph adenoma. Results in what?
Nelson Syndrome
Results in mass effects of pituitary tumor and hyperpigmentation (ACTH stimulatory effect on melanocytes).
Does hypercotisolism develop in Nelson Sydnrome?
No. Hypercortisolism does not develop.
Adenoma that produces FSH and LH. age/sex
gonadotroph, middle aged M/F.
Middle age, Presents with vision loss, dipoplia, and possibly pituitary apoplexy
Gonadotroph adenoma
TSH secreting adenoma
Thyrotroph adenoma
Rare, but requires craniospinal or systemic metastases for diagnosis.
Most functional, with PRL and ACTH most common secreted hormones.
Pituitary carcnoma
Postpartum necrosis of anterior pituitary, most commonly form of ____.
sheehan syndrome - ischemic necrosis of pituitary, resulting in hypopituitarism
Physiological pit expansion during pregnancy = relative hypoxia + any obstetric hemorrhage/shock = infarction of anterior lobe. What is this?
sheehan syndrome - ischemic necrosis of pituitary, resulting in hypopituitarism
An obese woman (with hx of multiple pregnancies) presents with visual field defects, hyperPRL. When arachnoid mater and CSF herniates into sella, expanding and sella and compressive pituitary. What is this?
Primary Empty Sella
Tumors that met from what two locations can result in clinical hypopituitarism?
Breast and lung
Primary empty sella can result in clinical manifestations involving what hormones?
GH, LH/FSH, TSH, ACTH, PRL, Melanocyte Stimulating Hormone
Bimodal age - 5-15, then 65+
Presentation:
Adults – HA, visual
Children – growth retardation
Hypothalamic Supracellar Tumors
Morphologic defferences btw adamantinomatous cranipharyngioma and papillary pharyngeoma
Papillary - lack keratin, calcification, cysts
