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ENRP Exam II > Pituitary Pathology (Martin) > Flashcards

Pituitary Pathology (Martin) Flashcards

1
Q

What is the one exception to a neoplasia being the primary endocrine HYPERfunction?

A

Thyroid; all others most common form of primary endocrine hyper function is NEOPLASIA

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2
Q

Embryology of posterior pituitary

A

Neurohypophysis; pituicytes (modified glial cells); pars nervosa

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3
Q

Embryology of anterior pituitary

A

Adenohypophysis; oral cavity origin; pars distalis

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4
Q

Eosinophilic/acidophilic staining cells and the hormones they produce

A

PiG
Somatotrophs = GH
Mammosomatotrophs = GH + PRL
Lactotrophs = PRL

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5
Q

Somatotrophs produce which hormone(s)?

A

GH (eosinophilic/acidophilic)

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6
Q

Mammosomatotrophs produce which hormone(s)?

A

GH and PRL (eosinophilic/acidophilic)

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7
Q

Lactotrophs produce which hormone(s)?

A

PRL (eosinophilic/acidophilic)

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8
Q

What are the two inhibitory hormones produced by the hypothalamus?

A

Somatostatin and dopamine

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9
Q

PIT-1 cells

A

PiG
Somatotrophs = GH
Mammosomatotrophs = GH + PRL
Lactotrophs = PRL (also express ER alpha)

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10
Q

TPIT cells

A

Corticotrophs

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11
Q

SF-1 and GATA-2 expressing cells

A

Gonadotrophs

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12
Q

Basophilic staining cells and the hormones they produce?

A

FLAT
Corticoptrophs = ACTH, PMOC, MSH
Thyrotrophs = TSH
Gonadotrophs = FSH and LH

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13
Q

Transcription factor gonadotroph cells express?

A

SF-1 and GATA-2

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14
Q

Transcription factor corticotrophs express?

A

TPIT

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15
Q

Hyperpituitarism

A

excess secretion of hormones; hyperplasias, adenomas, and carcinomas

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16
Q

Hypopituitarism

A

deficiency of hormones; ischemic injury, surgery/radiation, mass effect

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17
Q

Mass effect

A

compression of optic chasm; bitemporal hemianopsia and increased intracranial pressure; can be due to pituitary apoplexy (acute hemorrhage into an adenoma)

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18
Q

Pituitary apoplexy

A

rapid enlargement of pituitary following acute hemorrhage into an adenoma; neurosurgical emergency; sudden death

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19
Q

What are the two most common hormones secreted by an anterior lobe adenoma?

A

GH and PRL

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20
Q

Null cell adenomas

A

nonfunctional adenoma; only present as mass effect

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21
Q

Transcription factor associated with Gigantism and Acromegaly?

A

PIT-1; GH = somatotrophs (PiG)

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22
Q

Transcription factor associated with Galactorrhea and amenorrhea in females and sexual dysfunction and infertility in males?

A

PIT-1; GH = somatotrophs and Lactotrophs = PRL (PiG)

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23
Q

Transcription factor associated with Cushing syndrome and Nelson syndrome?

A

TPIT; ACTH = corticotrophs (FLAT)

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24
Q

Activating mutation in corticotrophin adenomas?

A

Ubiquitin-specific protease 8 (USP8)

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25
Gene involved in Carnery complex?
protein kinase A, regulatory subunit 1 alpha (PRKAR1A)
26
Gene involved in familial isolated pituitary adenoma syndrome?
Aryl hydrocarbon receptor interacting protein (AIP)
27
Classic behavior of non-encapsulated pituitary adenomas
AGGRESSIVE; more likely an MACROadenoma; history = uniform monomorphic, polygonal cells in sheets or cords
28
Female presentation of female with a prolactinemia
amenorrhea, galactorrhea, diminished libido, infertility and mass effect
29
male presentation of male with a prolactinemia
decreased libido, decreased sperm count, mass effect
30
major characteristics of lactotroph adenomas (Prolactinoma)
mostly composed of chromophobe cells (eosinophilic cells are rare); undergo dystrophic calcification; isolated psammoma bodies "pituitary stone"
31
"pituitary stone" on histology
major characteristics of lactotroph adenomas (Prolactinoma); isolated psammoma bodies
32
Treatment for a prolactinoma
Dopamine agonist Bromocriptine
33
Somatotroph adenoma in kids
Gigantism; high GH and IGF-1
34
Somatotroph adenoma in adults
Acromegaly; high GH and IGF-1
35
Diagnosis and treatment of a somatotrophic adenoma?
failure of suppression of GH production with oral glucose tolerance test; tx w/ surgical removal or somatostatin analogues and GH receptor antagonist
36
Histology of a corticotroph microadenoma
ACTH to Cushing syndrome; basophilic with PAS + staining; TPIT + variant called Crooke cell adenoma = ring-like deposition of cytokeratin; aggressive
37
Crooke cell adenoma
variant of an corticotrophin adenoma (Cushing syndrome); characterized by ring-like deposition of cytokeratin; aggressive
38
Nelson syndrome
after surgical removal of adrenal glands; adrenals are absent so hypercortisolism does NOT develop; no negative feedback to pituitary (lack of adrenal hormones); pituitary adenoma develops; mass effect also increased release of ACTH - goes to POMC - to MSH - to melanocytes = hyperpigmentation
39
Pituitary carcinoma
craniospinal or systemic Metz; most are function = produces mostly PRL and ACTH
40
Pituitary blastoma
under 2 yr old pts; DICER1 mut carriers; "blastema-like" cells = small round blue cells and rosettes; common pathology is Cushing disease
41
Two main causes of hypopituitarism?
1. Pituitary apoplexy = sudden hemorrhage into adenoma; CVD collapse and sudden death 2. Sheehan syndrome = postpartum hemorrhage/necrosis (poor lactation and loss of pubic hair)
42
Rathke cleft cyst
lined by ciliated cuboidal cells; comprised of goblet and anterior pituitary cells
43
Empty sella syndrome
anything that destroys parts or all of the pituitary gland; primary = defect in diaphragm sella; secondary = mass/adenoma enlarges and compresses
44
Most common pathology from a hypothalamic lesion
diabetes insipidus
45
Most common pathology from an inflammatory disorder of the posterior pituitary
diabetes insipidus
46
Central diabetes insipidus
def in ADH; polyuria - HYPERnatremic, increased serum sodium and osmolality; common mutations are AVD (AD) and AVPR2 (x-linked)
47
Common mutations in diabetes insipidus?
AVD (AD) and AVPR2 (x-linked) - young boys
48
Common carcinoma involved in SIADH
small cell carcinoma of the lungs
49
SIADH
increased ADH; no diuresis (holding on to free water) HYPOnatremic; cerebral edema resulting in neurological dysfunction
50
Craniopharyngioma
remnants of Rathke pouch; slow growing tumor; bimodal 5-15 yrs and 65 yrs + headaches, visual disturbances, and growth retardation
51
What are the two types of craniopharyngiomas?
KIDS - adamantinomatous; calcifications seen on radiographs "wet keratin" is a diagnostic feature; "machine oil" fluid; mut in CTNNB1 ADULTS - papillary; BRAF mut
52
adamantinomatous
Craniopharyngioma found in kids; calcifications seen on radiographs "wet keratin" is a diagnostic feature; "machine oil" fluid; mut in CTNNB1
53
Common mutation found in a adamantinomatous
mut in CTNNB1
54
papillary craniopharyngiomas
Craniopharyngioma found in adults; BRAF mut
55
FLAT hormones (basophilic) PiG hormones (eosinophilic) PRL can be chromophobic
56
bitemporal hemianopsia; loss of peripheral vision; seen in compression of the optic chiasm by a pituitary mass
57
evolution of pituitary apoplexy; preexisting adenoma; hemorrhage into adenoma w/ mass effect; neurosurgical emergency
58
pituitary adenoma; soft and well-circumscribed; if not encapsulated then an aggressive adenoma - more likely to invade
59
pituitary stone; extensive calcification seen in prolactinomas
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ENRP Exam II (10 decks)

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