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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
Q

Gene involved in Carnery complex?

A

protein kinase A, regulatory subunit 1 alpha (PRKAR1A)

26
Q

Gene involved in familial isolated pituitary adenoma syndrome?

A

Aryl hydrocarbon receptor interacting protein (AIP)

27
Q

Classic behavior of non-encapsulated pituitary adenomas

A

AGGRESSIVE; more likely an MACROadenoma; history = uniform monomorphic, polygonal cells in sheets or cords

28
Q

Female presentation of female with a prolactinemia

A

amenorrhea, galactorrhea, diminished libido, infertility and mass effect

29
Q

male presentation of male with a prolactinemia

A

decreased libido, decreased sperm count, mass effect

30
Q

major characteristics of lactotroph adenomas (Prolactinoma)

A

mostly composed of chromophobe cells (eosinophilic cells are rare); undergo dystrophic calcification; isolated psammoma bodies “pituitary stone”

31
Q

“pituitary stone” on histology

A

major characteristics of lactotroph adenomas (Prolactinoma); isolated psammoma bodies

32
Q

Treatment for a prolactinoma

A

Dopamine agonist Bromocriptine

33
Q

Somatotroph adenoma in kids

A

Gigantism; high GH and IGF-1

34
Q

Somatotroph adenoma in adults

A

Acromegaly; high GH and IGF-1

35
Q

Diagnosis and treatment of a somatotrophic adenoma?

A

failure of suppression of GH production with oral glucose tolerance test; tx w/ surgical removal or somatostatin analogues and GH receptor antagonist

36
Q

Histology of a corticotroph microadenoma

A

ACTH to Cushing syndrome; basophilic with PAS + staining; TPIT +

variant called Crooke cell adenoma = ring-like deposition of cytokeratin; aggressive

37
Q

Crooke cell adenoma

A

variant of an corticotrophin adenoma (Cushing syndrome); characterized by ring-like deposition of cytokeratin; aggressive

38
Q

Nelson syndrome

A

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
Q

Pituitary carcinoma

A

craniospinal or systemic Metz; most are function = produces mostly PRL and ACTH

40
Q

Pituitary blastoma

A

under 2 yr old pts; DICER1 mut carriers; “blastema-like” cells = small round blue cells and rosettes; common pathology is Cushing disease

41
Q

Two main causes of hypopituitarism?

A
  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
Q

Rathke cleft cyst

A

lined by ciliated cuboidal cells; comprised of goblet and anterior pituitary cells

43
Q

Empty sella syndrome

A

anything that destroys parts or all of the pituitary gland; primary = defect in diaphragm sella; secondary = mass/adenoma enlarges and compresses

44
Q

Most common pathology from a hypothalamic lesion

A

diabetes insipidus

45
Q

Most common pathology from an inflammatory disorder of the posterior pituitary

A

diabetes insipidus

46
Q

Central diabetes insipidus

A

def in ADH; polyuria - HYPERnatremic, increased serum sodium and osmolality; common mutations are AVD (AD) and AVPR2 (x-linked)

47
Q

Common mutations in diabetes insipidus?

A

AVD (AD) and AVPR2 (x-linked) - young boys

48
Q

Common carcinoma involved in SIADH

A

small cell carcinoma of the lungs

49
Q

SIADH

A

increased ADH; no diuresis (holding on to free water) HYPOnatremic; cerebral edema resulting in neurological dysfunction

50
Q

Craniopharyngioma

A

remnants of Rathke pouch; slow growing tumor; bimodal 5-15 yrs and 65 yrs +
headaches, visual disturbances, and growth retardation

51
Q

What are the two types of craniopharyngiomas?

A

KIDS - adamantinomatous; calcifications seen on radiographs “wet keratin” is a diagnostic feature; “machine oil” fluid; mut in CTNNB1

ADULTS - papillary; BRAF mut

52
Q

adamantinomatous

A

Craniopharyngioma found in kids; calcifications seen on radiographs “wet keratin” is a diagnostic feature; “machine oil” fluid; mut in CTNNB1

53
Q

Common mutation found in a adamantinomatous

A

mut in CTNNB1

54
Q

papillary craniopharyngiomas

A

Craniopharyngioma found in adults; BRAF mut

55
Q
A

FLAT hormones (basophilic)
PiG hormones (eosinophilic)
PRL can be chromophobic

56
Q
A

bitemporal hemianopsia; loss of peripheral vision; seen in compression of the optic chiasm by a pituitary mass

57
Q
A

evolution of pituitary apoplexy; preexisting adenoma; hemorrhage into adenoma w/ mass effect; neurosurgical emergency

58
Q
A

pituitary adenoma; soft and well-circumscribed; if not encapsulated then an aggressive adenoma - more likely to invade

59
Q
A

pituitary stone; extensive calcification seen in prolactinomas

60
Q
A
61
Q
A
62
Q
A

ENRP Exam II (10 decks)

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