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
2
Q
Desired approach to pituitary tumors
A
Transsphenoidal resection
3
Q
Characterized by episodic attack of rectal temperatures less than 30 degrees Centigrade, sweating, vasodilation, vomiting bradycardia
A
Periodic hypothermia syndrome
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
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
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
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
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
9
Q
Most common pituitary hormone hypersecretion hormone
A
Hyperprolactinemia
10
Q
Important physiologic causes of hyperprolactinemia
A
Pregnancy and lactation
11
Q
Can Increase PRL secretion
A
Nipple stimulation
Sexual orgasm
12
Q
Hallmarks of hyperprolacrinemia in women
A
Amenorrhea
Galactorrhea
Infertility
13
Q
Hallmarks of hyperprolacrinemia in males
A
Diminished libido
Infertility
Visual loss
14
Q
Effective for most cases of hyperprolactinemia
A
Dopamine agonist
15
Q
True or false. Dopamine agonist may worsen underlying psychiatric condition
A
True
16
Q
Main stay therapy for prolactinomas
A
Oral dopamine agonist (bromocriptine and cabergoline)
17
Q
Ergoline derivative; long acting dopamine agonist with high affinity to D2 receptor affinity
A
Cabergoline
18
Q
Ergot akaloid dopamine receptor agonist that suppresses prolactin secretion
A
Bromocriptine
19
Q
Most common cuss of GHRH mediated acromegaly
A
Chest or abdominal carcinoid tumor
20
Q
Most significant clinical impact of GH excess
A
Cardiovascular system
21
Q
Initial treatment for acromegaly? Adjuvant?
A
Initial: Surgical resection
Adjuvant: somatostatin analogue
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
23
Q
True or false. Irradiation is effective in normalizing IGF-1 levels
A
False. Irradiation is ineffective
24
Q
Preferred medical treatment for acromegaly
A
Somatostatin analogues: octreotide and lanreotide
25
Eight-amino-acid synthetic somatostatin analogues that as 40x potency than native somatostatin. What is its half life
Octreotide. Half life: 2 hours
26
Autogek slow release depot preparation of cyclic somastostatin octapeptide analogue that suppresses GH and IGF-1 hypersecretion
Lanreotide
27
Side effect of octreotide
Postprandial gallbladder hypomotility and delays gallbladder emptying
28
Antagonizes endogenius GH action by blocking peripheral GH binding to its receptor
Pegvisomant
29
Most common cause of cushingoid features
Iatrogenic hypercortisolism
30
Precise and cost effective screening test for Cushings syndrome
24 hour urine free cortisol
31
How to differentiate ACTH secreting pituitary tumor be ectopic ACTH secretion
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
32
Somatostatin analogue with high affinity for SST5 than SST2 recpetos and approved for treating patients wiht ACTH secreting pituitary tumor
Pasireotide
33
Imidazole derivative antimycotic agent inhibits several P450 enzymes and effectivelu lowers cortisol in most patient with Cushing syndrome?
Ketoconazole
34
Glucocorticoid receptor antagonist blocks peripheral cortisol action and approve for treatment of hyperglycemia in Cushing syndrome
Mifepristone
35
Inhibits 11 beta hydroxylase activity and normalize plasma cortisol
Metyrapone
36
Suppresses cortisol hypersecretion by inhibiting 11 beta hydroxylase and cholesterol side chain cleavage enzymes by destroying adrenocortical cells
Mitotane
37
Disorder characterized by rapid pituitary tumor enlargement and increased pigmentation secondary to high ACTH levels
Nelson's syndrome
38
Characteristic that suggest TSH secreting tumor
Presence of pituitary mass and elevated B subunit levels
39
47/Female suffered Sheehan syndrome. What is the expected laboratory finding?
low ACTH, low TSH, low FT4, low GH, Low FSH, Low LH
40
refers to intrapituitary hemorrhagic vascular vent that occurs in postpartum women; pituitary apoplexy
Sheehan's syndrome
41
what causes sheehan syndrome
due to hyperplastic enlargement of pituitary during pregnancy which increases risk for hemorrhage and infarction
42
present as the least resistance to soft tissue expansion
dorsal sellar diaphragm
43
common features of small intrasellar tumors
headache
44
Features of sellar mass lesion. Identify impacted structure if patient present with hypogonadism, hypothyroidism, growth failure and adult hyposomatoropism, hypoadrenalism
pituitary
45
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
Optic chiasm
46
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
hypothalamus
47
Features of sellar mass lesion. Identify impacted structure if patient present with opthalmoplegia with or without ptosis or diplopa, facial numbness
cavernous sinus
48
Features of sellar mass lesion. Identify impacted structure if patient present with personality disorder, anosmia
frontal lobe
49
Features of sellar mass lesion. Identify impacted structure if patient present with headache, hydrocephalus, pychosis, dementia, laughing seizures
brain
50
pituitary gland height in adults
8 mm
51
when is the pituitary gland biggest
during pregnancy and puberty and may reach 10-12 mm
52
presenting clinical features of functional pituitary adenomas
acromegaly, prolactinomas, Cushing syndrome
53
Initial hormonal evaluation if a pituitary adenoma is suspected based on MRI
5 tests. 1 basal prolactin, 2 insulin like growth factor, 3. 24 hr urinary free cortisol, 4. FSH, LH, 5. thyroid function tests
54
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
periodic hypothermia syndrome
55
damage to this area is associated with hyperphagia and obesity
damage to ventromedial hypothalamic nuclei
56
damage to this area is associated with polydipsia and hypodipsia
damage to central osmoreceptors located in preoptic nuclei
57
refers to benign suprasellar cystic masses that present with headache, visual field defects, variable degrees of hypopituitarism; are derived from Rathke's pouch
??
58
presents with bony clival erosion, local invasiveness and calcification.
Sella chordomas
59
True or false. Meningioma arising from sellar region may be difficult to distinguish from non functioning pituitary adenomas
True.
60
Presents with diabetes insipidus, exopthalmus, punched out lytic bone lesions
Hand schuller Christian disease
61
occurrence of pituitary metastases
3.00%
62
major cause of pituitary metastases
breast cancer
63
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
Pallister Hall syndrome
64
what causes pallistera hall syndrome
mutations in the carboxyl terminus of the GLI13 gene
65
most common cause of pituitary hormone hypersection and hyposecretion in adults
pituitary adenoma
66
Classification of pituitary adenoma. Clinical syndrome: hypogonadism, galactorrhea
lactorope PRL
67
Classification of pituitary adenoma. Clinical syndrome: silent or hypogonadism
Gonadotrope FSH LH
68
Classification of pituitary adenoma. Clinical syndrome: acromegaly/gigantism
Somatotrope GH
69
Classification of pituitary adenoma. Clinical syndrome: Cushing or silent
Corticotrope ACTH
70
Classification of pituitary adenoma. Clinical syndrome: thyrotoxicosis
Thyrotrope TSH
71
Characterized by spotty skin pigmentation, myxomas, endocrine tumors, testicular, adrenal and pituitary adenomas with mutations in the PRKARIA
Carney complex
72
Characterized bypolyostotic fibrous dysplasia, pigmented skin patches, endocrine disorders caused by inactivation of Gsa
McCune Albright syndrome
73
most common pituiary hormone hypersectretion in both men and women
hyperprolactinemia
74
important physiologic causes of hyperprolactinemia
pregnancy and lactation
75
True or false. Antipsychotics and antidepressants are common cause of hyperprolactinemia
True.
76
hallmarks of prolactinemia in women
amenorrhea, galactorrhea and infertility
77
usual presenting symptom of hyperprolactinemia in men
diminished libido, infertility and visual loss
78
what is galactorrhea
discharge of milk containing fluid the breast from longer than 6 months after childbirth or discontinuation of breast feeding
79
Normal PRL level
less than 20 ug/L
80
drug class effect for most cases of hyerprolactinemia
dopamine agonist
81
diameter of microadenoma
less than 1 cm in diameter
82
diameter of macroadenoma
more than 1 cm in diameter
83
True or false. Tumor size correlates directly with PRL concentrations
True.
84
True or false. Microadenomas rarely progress to become macroadenomas
True.
85
mainstay treatment of prolactinemias
dopamine agonist bromocriptine and carbergoline
86
what is mechanism of action of dopamine agonist
suppress PRL secretion and synthesis as well as lactotrope cell proliferation
87
when can dopamine agonist be discontinued
After 2 yrs patient achieved normoprolactinemia
88
True or false. About 20% of men are resistant to dopamine agonist treatment
True.
89
ergoline derivattive and a long acting dopamine agonist
cabergoline
90
what is the effectiveness of cabergoline
suppresses PRL for 14 days after a single oral dose
91
what is the dose of cabergoline
Cabergoline 0.5-1.0 mg twice weekly
92
ergot alkaloid that is short acting dopamine receptor agonist that suppresses PRL
bromocriptine mesylate
93
when is bromocriptine preferred
when pregnancy is desired
94
dose and titration of bromocriptine
Bromocriptin 0.625 mg -1.25 mg at bedtime with snack; most are controlled with a dose of 2.5 mg TID
95
side effects of dopamine agonist
constipation, nasal stuffiness, dry mouth, nightmares, insomnia and vertigo
96
Symptomatic prolactinoma. Macroadenoma. What to do next?
test visual fields, test pituitary function, titrate dopamine agonist. Repeat MRI after 4 months
97
Symptomatic prolactinoma. Macroadenoma. No tumor shrinkage on repeat MRI at 4 months
Consider sugery
98
Symptomatic prolactinoma. Macroadenoma. Drug intoleranace.
Change dopamin agoniost. Repat MRI after 4 months. Get serum PRL
99
Symptomatic prolactinoma. Macroadenoma. With tumor shrinkage on repeat MRI at 4 months
Monitor PRL and repeat MRI annually
100
Symptomatic prolactinoma. Microadenoma. What to do next?
Titrate dopamine agonist. Get serum PRL
101
Symptomatic prolactinoma. Microadenoma.PRL less than 20
Maintenance Rx
102
Symptomatic prolactinoma. Microadenoma.PRL 20-50
Reassess diagnosis and increase dose
103
Symptomatic prolactinoma. Microadenoma.PRL more than 50
Consider surgery
104
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
True.
105
what is the most common cause of GHRH mediate acromegaly
chest or abdominal carcinoid tumor
106
True or false. Protean manifestation of GH and IGF-I are indolent are not clinically diagnosed for 10 yr or more
True.
107
most significant impact of GH excess occurs in what body system
cardiovascular
108
Malignancy at increased risk with acromegaly
colonic polyps and mortality from colonic malignancy
109
Initial treatment for acromeglay? Adjuvant
surgical resection is the initial treatment; adjuvant somastotatin analogues
110
where does somastostatin analogues exert their action
SSTR2 and SSTR5 receptors
111
synthetic somastostatin analog that is relatively resistance to plasma degradation
ocretotide
112
what is the half life and potency of ocretotide compared to native somastostatin
2 hr half life. 40X potency
113
given to those resistant to ocreotide with preferential SST5 binding
pasireotide
114
side effects of ocreotide
supresses postprandial gallbladder contractility and delay gallbladder emptying
115
somastostatin analog with higher prevalence of glucose intolerance or new onset diabetes mellitus
pasireotide
116
treatment of acromegaly
somastostatin analogs, GH receptor antagonist, dopamin agonist, radiation
117
antagonized endogenous GH action by blocking peripheral GH binding to its receptor
Pegvisomant
118
True or false. Combined treatment with ocreotide and cabergoline may induce additive biochemical control compared to either drug alone
True.
119
side effect of pegvisomant
reversible liver enzyme elevation
120
most common cause of cushingoid features
iatrogenic hypercortisolism
121
how is Cushing disease diagnosed
laboratory documentation of endogenous hypercortisolism by measurement of 24 hr urine free cortisol
122
precise and cost effective screening test for Cushings disease
24h urine free cortisol
123
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
True.
124
Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Etiology
ACTH secreting: pituitary corticotrope Ectpic ACTH: bronchial, abdominal carcinoid, small cell lung cancer, thymoma
125
Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Sex
ACTH secreting: F > M Ectpic ACTH: M > F
126
Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Clinical features
ACTH secreting:Slow onset Ectpic ACTH: rapid onset, pigmentation, severe myopathy
127
Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Serum potassium less than 3.3 ug/L
ACTH secreting: less than 10% Ectpic ACTH: 75%
128
Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Low dose overnight dexamethasone suppression
ACTH secreting: more than5 ug/dl Ectpic ACTH: more than 5 ug/dl
129
Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. High dose overnight dexamethasone suppression
ACTH secreting: less than5 ug/dl Ectpic ACTH: more than 5 ug/dl
130
Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Inferior petrossal sinus sampling IPSS. Basal peripheral
ACTH secreting: more than 2 Ectpic ACTH: less than 2
131
Difference. ACTH secreting pituitary tumor vs ectopic ACTH sectretion. Inferior petrossal sinus sampling IPSS.CRH induced
ACTH secreting: more than 3 Ectpic ACTH: less than 3
132
Contraindications to inferior petrosal venous sampling
hypertension, patients with known cerebrovascular disease or in the presence of well visualized pituitary adenoma on MRI
133
treatment of choice for Cushing disease
selective transsphenoidal resection
134
remission rate for selective transsphenoidal resection.
80% for microadenoma and less than 50% for macroadenomas
135
risk associated with adrenalectomy
Nelson syndrome
136
If after transsphenoidal surgical resection there is biochemical cure, what to do next?
Serial biochemical and MRI evaluation
137
if after transsphenoidal surgical resection there is persistent hypercortisolism, what to do next?
Pasireotide, gluocorticoid receptor antagonist, steroidogenic inhibitors, pituitary irradiation, adrenalectomy
138
postoperatively how long may period of symptomatic ACTH deficiency last
up to 12 months
139
imidazole derivative antimycotic agents that inhibits several P450 enzymes and effectively lowers cortisol in most patients with Cushing disease
ketoconazole
140
what is the dose of ketoconazole in Cushing disease
600-1200 mg per day
141
glucocorticoid receptor antagonist that blocks peripheral cortisol action and approved to treat hyperglycemia in Cushings disease
Mifepristone 300-1200 mg per day
142
inhibits 11beta hydroxylase activity and normalized plasma cortisol in 75% of patients
metyrapone
143
Inhibits 11beta hydroxylase activity nad cholesterol side chain cleavage enzy,es and destroys adrenocortical cells
mitotane
144
other glucocorticod receptor antagonist
Aminoglutethimide 250 mg TID, trilostane 200-1000 mg/d, cyproheptadine 24 mg/d, and IV etomidate 0.3 mg/k/h
145
disorder characterized by rapid pituitary tumor enlargement and increased pigmentation secondary to high ACTH levels
Nelson syndrome
146
maybe indicated to prevent Nelsons syndrome after adrenalectomy
prophylactic radiation therapy
147
origin of most clinically nonfunctioning adenoma
gonadotrope
148
for macroadenoma who underwent surgery, what is the monitoring
About 6 months postoperatively MRI scan should performed yearly to detect tumor regrowth
149
After 5-6 years how many percent of nonfunctioning tumors recur
15.00%
150
True or false. Presence of a pituitary mass and elevated beta subunit levels are suggestive of TSH secreting tumor
True.
151
Workup for incidentally discovered sellar mass. To exclude acromegaly and GH deficiency
Age and sex matched IGF-1
152
Workup for incidentally discovered sellar mass. To exclude Cushing disease and secondary adrenal insufficiency
24h urine free cortisol or overnight dexamethasone test, ACTH
153
Workup for incidentally discovered sellar mass. To exclude prolactinoma
basal serum prolactin
154
basal serum prolactin
FT4 and TSH
155
Workup for incidentally discovered sellar mass. To exclude hypogonadism from mass compression
alpha subunit FSH, LSH
