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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
diagnostic criteria for pheochromocytoma [3]
-elevated plasma fractionated metanephrine -elevated urinary fractionated catecholamine, metanephrine, or VMA -presence of an adrenal mass on CT or T2 weighted MRI
26
pheochromocytoma **requires** preoperative treatment with
alpha blockers followed by beta blockers **to avoid unopposed alpha adrenergic receptor stimulation, avoid hypertensive crisis
27
syndromes associated with pheochromocytoma [5]
von Hippel Lindau syndrome NF 1 MEN2 familial paragangliomas (SDH mutation) TMEM 127 mutation
28
regulate serum calcium in the body [2]
vitamin D parathyroid **makes them good labs for suspected hyperparathyroidism
29
high calcium and high PTH is diagnostic for?
primary hyperparathyroidism
30
most effective treatment for hyperparathyroidism?
parathyroidectomy
31
when serum albumin is abnormal, you need to
correct calcium **for "missing" calcium that is normally bound to albumin
32
equation to correct Calcium
ca+0.8 x (4-albumin)
33
if calcium is low, PTH will do what to correct it? [3 mechanisms]
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
when does primary hyperparathyroidism need to be treated surgically? [4]
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
MEN1 related PHPT or sporadic PHPT: onset 20-25
MEN1
36
MEN1 related PHPT or sporadic PHPT: M:F 1:1
MEN1
37
MEN1 related PHPT or sporadic PHPT: multiglandular
MEN1
38
MEN1 related PHPT or sporadic PHPT: high recurrence rate following surgery
MEN1
39
MEN1 related PHPT or sporadic PHPT: 50-70 y
sporadic
40
MEN1 related PHPT or sporadic PHPT: M:F 1:3
sporadic
41
inheritance pattern of MEN1 mutation
autosomal dominant
42
[...] mutation is related to primary hyperthyroidism occurring at unusually young age
MEN1 gene
43
MEN1 mutation is associated with cancer in what organs?
3 Ps pituitary pancreas parathyroid
44
tumor suppressor gene that encodes menin
MEN1
45
diagnostic criteria for MEN1 [3]
2 or more MEN1 tumors OR familial MEN1 tumors in first degree relative OR MEN1 mutation positive
46
[MEN1 or MEN2] excellent phenotype-genotype correlation
MEN2
47
because MEN[..] is not well-differentiated, it has a higher mortality
MEN1
48
leading cause of death in MEN1 patient
untreated tumors **screening/surveillance important**
49
which MEN disorder is recurrent primary hyperparathyroidism at an early age and multiple family members with NETs more associated with?
MEN1
50
Zollinger-Ellison insulinomas VIPomas glucagonomas are associated with
MEN1 **fall under pancreatic endocrine tumors
51
3 Ps of MEN1
pituitary tumors pancreatic endocrine parathyroid adenomas
52
associated with MEN2B [4]
medullary thyroid carcinoma pheochromocytoma mucosal neuromas marfanoid habitus
53
associated with MEN2A [3]
parathyroid hyperplasia medullary thyroid carcinoma pheochromocytoma
54
most appropriate next test in patient with hypercalcemia
PTH
55
urine 24-free cortisol and dexamethasone suppression test for
cushings
56
plasma fractionated metanephrines test for
pheochromocytoma
57
should be obtained on all patients who present with medullary thyroid carcinoma or MEN2 related diseases
RET proto-oncogenes
58
serum CEA and calcitonin are used to screen for
medullary thyroid cancer
59
serum calcium and PTH are used to screen for
primary hyperparathyroidism
60
calcitonin over 500 indicates
metastasis of medullary thyroid carcinoma
61
primary hyperparathyroidism is associated with
MEN1 and MEN2A **NOT MEN2B**
62

M&M Test 3 (16 decks)

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