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Endocrine > 373 Ant Pituitary > Flashcards

373 Ant Pituitary Flashcards

Chapter 373. Anterior Pituitary Tumor Syndromes

1
Q

Common features of small intrasellar tumors with no demonstrable suprasellar extension

A

Headaches

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2
Q

Desired approach to pituitary tumors

A

Transsphenoidal resection

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3
Q

Characterized by episodic attack of rectal temperatures less than 30 degrees Centigrade, sweating, vasodilation, vomiting bradycardia

A

Periodic hypothermia syndrome

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4
Q

Benign suprasellar cystic masses that present with headaches, visual field deficits, variable degrees of hypopituitarism; derived from Rathke’s pouch and arise near the pituitary stalk

A

Craniopharyngiomas

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5
Q

Hypothalamic hamatomas associated with craniofacial abnormalities; imperforate anus, cardiac, renal and lung disorders, pituitary failure; caused by mutation in carbxy terminus of GLI3 gene

A

Pallister- Hall syndrome

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6
Q

Autosomal dominant syndrome characterized by a genetic predisposition to Parathyroid, pancreatic islet, and pituitary adenoma caused by I activating mutations in MENIN found on chromosome 11q13

A

MEN 1

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7
Q

Characterized by spotty skin pigmentation, myxomas, endocrine tumors associated with mutation in R1 alpha subunits of protein kinase A (PRKAR1A)

A

Carneys syndrome

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8
Q

Consist of variety of endocrine disorders, polyostotic fibrous dysplasia, pigmented skin patches that results from constitutive cyclic AMP production causes by GTPase activity of Gsa

A

McCune- Albright syndrome

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9
Q

Most common pituitary hormone hypersecretion hormone

A

Hyperprolactinemia

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10
Q

Important physiologic causes of hyperprolactinemia

A

Pregnancy and lactation

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11
Q

Can Increase PRL secretion

A

Nipple stimulation

Sexual orgasm

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12
Q

Hallmarks of hyperprolacrinemia in women

A

Amenorrhea
Galactorrhea
Infertility

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13
Q

Hallmarks of hyperprolacrinemia in males

A

Diminished libido
Infertility
Visual loss

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14
Q

Effective for most cases of hyperprolactinemia

A

Dopamine agonist

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15
Q

True or false. Dopamine agonist may worsen underlying psychiatric condition

A

True

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16
Q

Main stay therapy for prolactinomas

A

Oral dopamine agonist (bromocriptine and cabergoline)

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17
Q

Ergoline derivative; long acting dopamine agonist with high affinity to D2 receptor affinity

A

Cabergoline

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18
Q

Ergot akaloid dopamine receptor agonist that suppresses prolactin secretion

A

Bromocriptine

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19
Q

Most common cuss of GHRH mediated acromegaly

A

Chest or abdominal carcinoid tumor

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20
Q

Most significant clinical impact of GH excess

A

Cardiovascular system

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21
Q

Initial treatment for acromegaly? Adjuvant?

A

Initial: Surgical resection
Adjuvant: somatostatin analogue

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22
Q

After surgery, when does GH levels return to normal? IGF-1?

A

GH: normalize in one hour

IGF-1: normalize in 3-5 days

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23
Q

True or false. Irradiation is effective in normalizing IGF-1 levels

A

False. Irradiation is ineffective

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24
Q

Preferred medical treatment for acromegaly

A

Somatostatin analogues: octreotide and lanreotide

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25
Q

Eight-amino-acid synthetic somatostatin analogues that as 40x potency than native somatostatin. What is its half life

A

Octreotide. Half life: 2 hours

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26
Q

Autogek slow release depot preparation of cyclic somastostatin octapeptide analogue that suppresses GH and IGF-1 hypersecretion

A

Lanreotide

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27
Q

Side effect of octreotide

A

Postprandial gallbladder hypomotility and delays gallbladder emptying

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28
Q

Antagonizes endogenius GH action by blocking peripheral GH binding to its receptor

A

Pegvisomant

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29
Q

Most common cause of cushingoid features

A

Iatrogenic hypercortisolism

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30
Q

Precise and cost effective screening test for Cushings syndrome

A

24 hour urine free cortisol

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31
Q

How to differentiate ACTH secreting pituitary tumor be ectopic ACTH secretion

A

Hypokalemia in Ectopic ACTH secretion
Dexamethasone 2 mg higb dose suppression test
-ectopic cortisol more than 5 ug/L
-ACTH secreting tumor cortisol less than 5 ug/L

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32
Q

Somatostatin analogue with high affinity for SST5 than SST2 recpetos and approved for treating patients wiht ACTH secreting pituitary tumor

A

Pasireotide

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33
Q

Imidazole derivative antimycotic agent inhibits several P450 enzymes and effectivelu lowers cortisol in most patient with Cushing syndrome?

A

Ketoconazole

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34
Q

Glucocorticoid receptor antagonist blocks peripheral cortisol action and approve for treatment of hyperglycemia in Cushing syndrome

A

Mifepristone

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35
Q

Inhibits 11 beta hydroxylase activity and normalize plasma cortisol

A

Metyrapone

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36
Q

Suppresses cortisol hypersecretion by inhibiting 11 beta hydroxylase and cholesterol side chain cleavage enzymes by destroying adrenocortical cells

A

Mitotane

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37
Q

Disorder characterized by rapid pituitary tumor enlargement and increased pigmentation secondary to high ACTH levels

A

Nelson’s syndrome

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38
Q

Characteristic that suggest TSH secreting tumor

A

Presence of pituitary mass and elevated B subunit levels

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39
Q

47/Female suffered Sheehan syndrome. What is the expected laboratory finding?

A

low ACTH, low TSH, low FT4, low GH, Low FSH, Low LH

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40
Q

refers to intrapituitary hemorrhagic vascular vent that occurs in postpartum women; pituitary apoplexy

A

Sheehan’s syndrome

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41
Q

what causes sheehan syndrome

A

due to hyperplastic enlargement of pituitary during pregnancy which increases risk for hemorrhage and infarction

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42
Q

present as the least resistance to soft tissue expansion

A

dorsal sellar diaphragm

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43
Q

common features of small intrasellar tumors

A

headache

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44
Q

Features of sellar mass lesion. Identify impacted structure if patient present with hypogonadism, hypothyroidism, growth failure and adult hyposomatoropism, hypoadrenalism

A

pituitary

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45
Q

Features of sellar mass lesion. Identify impacted structure if patient present with loss of red perception, bitemporal hemianopia, superior or bitemporal field defect, scotoma, blindness

A

Optic chiasm

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46
Q

Features of sellar mass lesion. Identify impacted structure if patient present with temperature dysregulation, appetite and thirst disorders, obesity, diabetes insipidus, behavioral dysfunction, autonomic dysfunction

A

hypothalamus

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47
Q

Features of sellar mass lesion. Identify impacted structure if patient present with opthalmoplegia with or without ptosis or diplopa, facial numbness

A

cavernous sinus

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48
Q

Features of sellar mass lesion. Identify impacted structure if patient present with personality disorder, anosmia

A

frontal lobe

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49
Q

Features of sellar mass lesion. Identify impacted structure if patient present with headache, hydrocephalus, pychosis, dementia, laughing seizures

A

brain

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50
Q

pituitary gland height in adults

A

8 mm

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51
Q

when is the pituitary gland biggest

A

during pregnancy and puberty and may reach 10-12 mm

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52
Q

presenting clinical features of functional pituitary adenomas

A

acromegaly, prolactinomas, Cushing syndrome

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53
Q

Initial hormonal evaluation if a pituitary adenoma is suspected based on MRI

A

5 tests. 1 basal prolactin, 2 insulin like growth factor, 3. 24 hr urinary free cortisol, 4. FSH, LH, 5. thyroid function tests

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54
Q

Characterized by episodic attacks of rectal temperatures less than 30 degrees Celsius or less than 86 degrees Fahrenheit, sweating, vasodilation, vomiting and bradycardia associated with hypothalamic lesion

A

periodic hypothermia syndrome

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55
Q

damage to this area is associated with hyperphagia and obesity

A

damage to ventromedial hypothalamic nuclei

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56
Q

damage to this area is associated with polydipsia and hypodipsia

A

damage to central osmoreceptors located in preoptic nuclei

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57
Q

refers to benign suprasellar cystic masses that present with headache, visual field defects, variable degrees of hypopituitarism; are derived from Rathke’s pouch

A

??

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58
Q

presents with bony clival erosion, local invasiveness and calcification.

A

Sella chordomas

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59
Q

True or false. Meningioma arising from sellar region may be difficult to distinguish from non functioning pituitary adenomas

A

True.

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60
Q

Presents with diabetes insipidus, exopthalmus, punched out lytic bone lesions

A

Hand schuller Christian disease

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61
Q

occurrence of pituitary metastases

A

3.00%

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62
Q

major cause of pituitary metastases

A

breast cancer

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63
Q

hypothalamic hamartomas that is associated with craniofacial abnormalities, imperforate anus, cardiac, renal and lung disorders, and pituitary failure cauised by mutations in the caboxy terminus of the GLI13 gene

A

Pallister Hall syndrome

64
Q

what causes pallistera hall syndrome

A

mutations in the carboxyl terminus of the GLI13 gene

65
Q

most common cause of pituitary hormone hypersection and hyposecretion in adults

A

pituitary adenoma

66
Q

Classification of pituitary adenoma. Clinical syndrome: hypogonadism, galactorrhea

A

lactorope PRL

67
Q

Classification of pituitary adenoma. Clinical syndrome: silent or hypogonadism

A

Gonadotrope FSH LH

68
Q

Classification of pituitary adenoma. Clinical syndrome: acromegaly/gigantism

A

Somatotrope GH

69
Q

Classification of pituitary adenoma. Clinical syndrome: Cushing or silent

A

Corticotrope ACTH

70
Q

Classification of pituitary adenoma. Clinical syndrome: thyrotoxicosis

A

Thyrotrope TSH

71
Q

Characterized by spotty skin pigmentation, myxomas, endocrine tumors, testicular, adrenal and pituitary adenomas with mutations in the PRKARIA

A

Carney complex

72
Q

Characterized bypolyostotic fibrous dysplasia, pigmented skin patches, endocrine disorders caused by inactivation of Gsa

A

McCune Albright syndrome

73
Q

most common pituiary hormone hypersectretion in both men and women

A

hyperprolactinemia

74
Q

important physiologic causes of hyperprolactinemia

A

pregnancy and lactation

75
Q

True or false. Antipsychotics and antidepressants are common cause of hyperprolactinemia

A

True.

76
Q

hallmarks of prolactinemia in women

A

amenorrhea, galactorrhea and infertility

77
Q

usual presenting symptom of hyperprolactinemia in men

A

diminished libido, infertility and visual loss

78
Q

what is galactorrhea

A

discharge of milk containing fluid the breast from longer than 6 months after childbirth or discontinuation of breast feeding

79
Q

Normal PRL level

A

less than 20 ug/L

80
Q

drug class effect for most cases of hyerprolactinemia

A

dopamine agonist

81
Q

diameter of microadenoma

A

less than 1 cm in diameter

82
Q

diameter of macroadenoma

A

more than 1 cm in diameter

83
Q

True or false. Tumor size correlates directly with PRL concentrations

A

True.

84
Q

True or false. Microadenomas rarely progress to become macroadenomas

A

True.

85
Q

mainstay treatment of prolactinemias

A

dopamine agonist bromocriptine and carbergoline

86
Q

what is mechanism of action of dopamine agonist

A

suppress PRL secretion and synthesis as well as lactotrope cell proliferation

87
Q

when can dopamine agonist be discontinued

A

After 2 yrs patient achieved normoprolactinemia

88
Q

True or false. About 20% of men are resistant to dopamine agonist treatment

A

True.

89
Q

ergoline derivattive and a long acting dopamine agonist

A

cabergoline

90
Q

what is the effectiveness of cabergoline

A

suppresses PRL for 14 days after a single oral dose

91
Q

what is the dose of cabergoline

A

Cabergoline 0.5-1.0 mg twice weekly

92
Q

ergot alkaloid that is short acting dopamine receptor agonist that suppresses PRL

A

bromocriptine mesylate

93
Q

when is bromocriptine preferred

A

when pregnancy is desired

94
Q

dose and titration of bromocriptine

A

Bromocriptin 0.625 mg -1.25 mg at bedtime with snack; most are controlled with a dose of 2.5 mg TID

95
Q

side effects of dopamine agonist

A

constipation, nasal stuffiness, dry mouth, nightmares, insomnia and vertigo

96
Q

Symptomatic prolactinoma. Macroadenoma. What to do next?

A

test visual fields, test pituitary function, titrate dopamine agonist. Repeat MRI after 4 months

97
Q

Symptomatic prolactinoma. Macroadenoma. No tumor shrinkage on repeat MRI at 4 months

A

Consider sugery

98
Q

Symptomatic prolactinoma. Macroadenoma. Drug intoleranace.

A

Change dopamin agoniost. Repat MRI after 4 months. Get serum PRL

99
Q

Symptomatic prolactinoma. Macroadenoma. With tumor shrinkage on repeat MRI at 4 months

A

Monitor PRL and repeat MRI annually

100
Q

Symptomatic prolactinoma. Microadenoma. What to do next?

A

Titrate dopamine agonist. Get serum PRL

101
Q

Symptomatic prolactinoma. Microadenoma.PRL less than 20

A

Maintenance Rx

102
Q

Symptomatic prolactinoma. Microadenoma.PRL 20-50

A

Reassess diagnosis and increase dose

103
Q

Symptomatic prolactinoma. Microadenoma.PRL more than 50

A

Consider surgery

104
Q

True or false. Patients with partially empty sellae may present with GH hypersecretion due to small GH secreting adenoma within the compressed rim of pituitary tissue

A

True.

105
Q

what is the most common cause of GHRH mediate acromegaly

A

chest or abdominal carcinoid tumor

106
Q

True or false. Protean manifestation of GH and IGF-I are indolent are not clinically diagnosed for 10 yr or more

A

True.

107
Q

most significant impact of GH excess occurs in what body system

A

cardiovascular

108
Q

Malignancy at increased risk with acromegaly

A

colonic polyps and mortality from colonic malignancy

109
Q

Initial treatment for acromeglay? Adjuvant

A

surgical resection is the initial treatment; adjuvant somastotatin analogues

110
Q

where does somastostatin analogues exert their action

A

SSTR2 and SSTR5 receptors

111
Q

synthetic somastostatin analog that is relatively resistance to plasma degradation

A

ocretotide

112
Q

what is the half life and potency of ocretotide compared to native somastostatin

A

2 hr half life. 40X potency

113
Q

given to those resistant to ocreotide with preferential SST5 binding

A

pasireotide

114
Q

side effects of ocreotide

A

supresses postprandial gallbladder contractility and delay gallbladder emptying

115
Q

somastostatin analog with higher prevalence of glucose intolerance or new onset diabetes mellitus

A

pasireotide

116
Q

treatment of acromegaly

A

somastostatin analogs, GH receptor antagonist, dopamin agonist, radiation

117
Q

antagonized endogenous GH action by blocking peripheral GH binding to its receptor

A

Pegvisomant

118
Q

True or false. Combined treatment with ocreotide and cabergoline may induce additive biochemical control compared to either drug alone

A

True.

119
Q

side effect of pegvisomant

A

reversible liver enzyme elevation

120
Q

most common cause of cushingoid features

A

iatrogenic hypercortisolism

121
Q

how is Cushing disease diagnosed

A

laboratory documentation of endogenous hypercortisolism by measurement of 24 hr urine free cortisol

122
Q

precise and cost effective screening test for Cushings disease

A

24h urine free cortisol

123
Q

True or false. Mean basal ACTH levers are about 8x higher in patients with ectopic ACTH secretion than in those with pituitary ACTH secreting adenomas

A

True.

124
Q

Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Etiology

A

ACTH secreting: pituitary corticotrope Ectpic ACTH: bronchial, abdominal carcinoid, small cell lung cancer, thymoma

125
Q

Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Sex

A

ACTH secreting: F > M Ectpic ACTH: M > F

126
Q

Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Clinical features

A

ACTH secreting:Slow onset Ectpic ACTH: rapid onset, pigmentation, severe myopathy

127
Q

Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Serum potassium less than 3.3 ug/L

A

ACTH secreting: less than 10% Ectpic ACTH: 75%

128
Q

Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Low dose overnight dexamethasone suppression

A

ACTH secreting: more than5 ug/dl Ectpic ACTH: more than 5 ug/dl

129
Q

Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. High dose overnight dexamethasone suppression

A

ACTH secreting: less than5 ug/dl Ectpic ACTH: more than 5 ug/dl

130
Q

Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Inferior petrossal sinus sampling IPSS. Basal peripheral

A

ACTH secreting: more than 2 Ectpic ACTH: less than 2

131
Q

Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Inferior petrossal sinus sampling IPSS.CRH induced

A

ACTH secreting: more than 3 Ectpic ACTH: less than 3

132
Q

Contraindications to inferior petrosal venous sampling

A

hypertension, patients with known cerebrovascular disease or in the presence of well visualized pituitary adenoma on MRI

133
Q

treatment of choice for Cushing disease

A

selective transsphenoidal resection

134
Q

remission rate for selective transsphenoidal resection.

A

80% for microadenoma and less than 50% for macroadenomas

135
Q

risk associated with adrenalectomy

A

Nelson syndrome

136
Q

If after transsphenoidal surgical resection there is biochemical cure, what to do next?

A

Serial biochemical and MRI evaluation

137
Q

if after transsphenoidal surgical resection there is persistent hypercortisolism, what to do next?

A

Pasireotide, gluocorticoid receptor antagonist, steroidogenic inhibitors, pituitary irradiation, adrenalectomy

138
Q

postoperatively how long may period of symptomatic ACTH deficiency last

A

up to 12 months

139
Q

imidazole derivative antimycotic agents that inhibits several P450 enzymes and effectively lowers cortisol in most patients with Cushing disease

A

ketoconazole

140
Q

what is the dose of ketoconazole in Cushing disease

A

600-1200 mg per day

141
Q

glucocorticoid receptor antagonist that blocks peripheral cortisol action and approved to treat hyperglycemia in Cushings disease

A

Mifepristone 300-1200 mg per day

142
Q

inhibits 11beta hydroxylase activity and normalized plasma cortisol in 75% of patients

A

metyrapone

143
Q

Inhibits 11beta hydroxylase activity nad cholesterol side chain cleavage enzy,es and destroys adrenocortical cells

A

mitotane

144
Q

other glucocorticod receptor antagonist

A

Aminoglutethimide 250 mg TID, trilostane 200-1000 mg/d, cyproheptadine 24 mg/d, and IV etomidate 0.3 mg/k/h

145
Q

disorder characterized by rapid pituitary tumor enlargement and increased pigmentation secondary to high ACTH levels

A

Nelson syndrome

146
Q

maybe indicated to prevent Nelsons syndrome after adrenalectomy

A

prophylactic radiation therapy

147
Q

origin of most clinically nonfunctioning adenoma

A

gonadotrope

148
Q

for macroadenoma who underwent surgery, what is the monitoring

A

About 6 months postoperatively MRI scan should performed yearly to detect tumor regrowth

149
Q

After 5-6 years how many percent of nonfunctioning tumors recur

A

15.00%

150
Q

True or false. Presence of a pituitary mass and elevated beta subunit levels are suggestive of TSH secreting tumor

A

True.

151
Q

Workup for incidentally discovered sellar mass. To exclude acromegaly and GH deficiency

A

Age and sex matched IGF-1

152
Q

Workup for incidentally discovered sellar mass. To exclude Cushing disease and secondary adrenal insufficiency

A

24h urine free cortisol or overnight dexamethasone test, ACTH

153
Q

Workup for incidentally discovered sellar mass. To exclude prolactinoma

A

basal serum prolactin

154
Q

basal serum prolactin

A

FT4 and TSH

155
Q

Workup for incidentally discovered sellar mass. To exclude hypogonadism from mass compression

A

alpha subunit FSH, LSH

Endocrine (15 decks)

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