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M&M Test 3 > B6-059 MEN > Flashcards

B6-059 MEN Flashcards

1
Q

low TSH indicates

A

hyperthyroidism

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2
Q

high TSH indicates

A

hypothyroidism

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3
Q

initial imaging modality for suspected thyroid mass

A

ultrasound

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4
Q

the more cyst content in a thyroid nodule, the […] suspicion of malignancy

A

higher

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5
Q

medullary thyroid carcinoma is associated with MEN[…]

A

MEN2

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6
Q

inheritance pattern of MEN2 mutation

A

autosomal dominant

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7
Q

3 diseases commonly associated with MEN2A

A

medullary thyroid carcinoma (100%)
pheochromocytoma (50%)
primary hyperparathyroidism (20%)

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8
Q

RET proto-oncogene mutation is associated with

A

MEN2A

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9
Q

2 important tumor markers for medullary carcinoma

A

calcitonin
CEA measurement

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10
Q

every MTC patient who is RET positive needs to be evaluated and screened for [2]

A

pheochromocytoma
primary hyperparathyroidism

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11
Q

if an MTC patient is RET negative, but has high calcitonin (>500), what is the next best step?

A

imaging studies to evaluate for metastasis

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12
Q

if an MTC patient is RET negative, and has low calcitonin (<500), what is the next best step?

A

thyroidectomy with or w/o cervical LAD

consider external beam radiation for extensive disease

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13
Q

which is more common, MEN2A or MEN2B?

A

MEN2A

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14
Q

conditions associated with MEN2B [4]

A

MTC (100%)
pheochromocytoma (50%)
marfanoid habitus
mucosal and digestive neurofibromas

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15
Q

MEN2[…] is generally more aggressive with a younger age of onset

A

MEN2B

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16
Q

is MEN2B associated with primary hyperparathyroidism?

A

no

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17
Q

marfanoid appearance
mucosal neuroma

A

MEN2B

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18
Q

all new sporadic MTC cases should be screened for […] mutation

A

RET

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19
Q

when is thyroidectomy recommended for patients with a RET mutation in codon 918?

A

within the first year of life

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20
Q

why is genetic testing for the RET mutation so important?

A

determines when prophylactic thyroidectomy and routine screenings for PHEO and hyperparathyroidism should be done

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21
Q

how do you screen for pheochromocytoma? [3]

A

-plasma fractionated metanephrines 24 -hour urinary fractionated catecholamines and metanephrines
-abdominal CT

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22
Q

rule of 10s for pheochromocytomas

A

-bilateral adrenal glands
-extra adrenal
-above diaphragm
-familial (now 40%)
-malignant

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23
Q

pheochromocytomas triad

A

headaches
sweating
palpitations

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24
Q

half of affected pheochromocytoma patients have sustained

A

hypertension

**most of remainder have paroxysmal spells

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25
Q

diagnostic criteria for pheochromocytoma [3]

A

-elevated plasma fractionated metanephrine
-elevated urinary fractionated catecholamine, metanephrine, or VMA
-presence of an adrenal mass on CT or T2 weighted MRI

26
Q

pheochromocytoma requires preoperative treatment with

A

alpha blockers followed by beta blockers

**to avoid unopposed alpha adrenergic receptor stimulation, avoid hypertensive crisis

27
Q

syndromes associated with pheochromocytoma [5]

A

von Hippel Lindau syndrome
NF 1
MEN2
familial paragangliomas (SDH mutation)
TMEM 127 mutation

28
Q

regulate serum calcium in the body [2]

A

vitamin D
parathyroid

**makes them good labs for suspected hyperparathyroidism

29
Q

high calcium and high PTH
is diagnostic for?

A

primary hyperparathyroidism

30
Q

most effective treatment for hyperparathyroidism?

A

parathyroidectomy

31
Q

when serum albumin is abnormal, you need to

A

correct calcium

**for “missing” calcium that is normally bound to albumin

32
Q

equation to correct Calcium

A

ca+0.8 x (4-albumin)

33
Q

if calcium is low, PTH will do what to correct it? [3 mechanisms]

A
  1. increase bone reabsorption
  2. increase calcium reabsorption and decreased phosphorus reabsorption in kidney, activate vitamin D
  3. increase calcium absorption in intestine with the activated 125 vitamin D
34
Q

when does primary hyperparathyroidism need to be treated surgically? [4]

A
  1. if serum calcium is above upper limit of normal
  2. evidence of osteoporosis
  3. evidence of kidney dysfunction (including stones)
  4. age <50
35
Q

MEN1 related PHPT or sporadic PHPT:
onset 20-25

A

MEN1

36
Q

MEN1 related PHPT or sporadic PHPT:
M:F 1:1

A

MEN1

37
Q

MEN1 related PHPT or sporadic PHPT:
multiglandular

A

MEN1

38
Q

MEN1 related PHPT or sporadic PHPT:
high recurrence rate following surgery

A

MEN1

39
Q

MEN1 related PHPT or sporadic PHPT:
50-70 y

A

sporadic

40
Q

MEN1 related PHPT or sporadic PHPT:
M:F 1:3

A

sporadic

41
Q

inheritance pattern of MEN1 mutation

A

autosomal dominant

42
Q

[…] mutation is related to primary hyperthyroidism occurring at unusually young age

A

MEN1 gene

43
Q

MEN1 mutation is associated with cancer in what organs?

A

3 Ps
pituitary
pancreas
parathyroid

44
Q

tumor suppressor gene that encodes menin

A

MEN1

45
Q

diagnostic criteria for MEN1 [3]

A

2 or more MEN1 tumors OR
familial MEN1 tumors in first degree relative OR
MEN1 mutation positive

46
Q

[MEN1 or MEN2]
excellent phenotype-genotype correlation

A

MEN2

47
Q

because MEN[..] is not well-differentiated, it has a higher mortality

A

MEN1

48
Q

leading cause of death in MEN1 patient

A

untreated tumors

screening/surveillance important

49
Q

which MEN disorder is recurrent primary hyperparathyroidism at an early age and multiple family members with NETs more associated with?

A

MEN1

50
Q

Zollinger-Ellison
insulinomas
VIPomas
glucagonomas

are associated with

A

MEN1

**fall under pancreatic endocrine tumors

51
Q

3 Ps of MEN1

A

pituitary tumors
pancreatic endocrine
parathyroid adenomas

52
Q

associated with MEN2B [4]

A

medullary thyroid carcinoma
pheochromocytoma
mucosal neuromas
marfanoid habitus

53
Q

associated with MEN2A [3]

A

parathyroid hyperplasia
medullary thyroid carcinoma
pheochromocytoma

54
Q

most appropriate next test in patient with hypercalcemia

A

PTH

55
Q

urine 24-free cortisol and dexamethasone suppression test for

A

cushings

56
Q

plasma fractionated metanephrines test for

A

pheochromocytoma

57
Q

should be obtained on all patients who present with medullary thyroid carcinoma or MEN2 related diseases

A

RET proto-oncogenes

58
Q

serum CEA and calcitonin are used to screen for

A

medullary thyroid cancer

59
Q

serum calcium and PTH are used to screen for

A

primary hyperparathyroidism

60
Q

calcitonin over 500 indicates

A

metastasis of medullary thyroid carcinoma

61
Q

primary hyperparathyroidism is associated with

A

MEN1 and MEN2A

NOT MEN2B

62
Q
A

M&M Test 3 (16 decks)

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