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Endocrine Fellowship Board Review > Neuroendocrine tumors > Flashcards

Neuroendocrine tumors Flashcards

1
Q

most common location of carcinoid tumors

A

GI tract 55%

Lungs 35%

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2
Q

symptoms of carcinoid syndrome

A 
flushing
diarrhea
hypotension
pruritis
telangiectasias on the face (prolonged vasodilitation)
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3
Q

difference in pulmonary vs gastric NET flushing

A

gastric - short, patchy, intensely pruritic

pulmonary - hours - days, more severe

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4
Q

biochemical mediators of carcinoid syndrome

A 
serotonin (diarrhea)
histamine (flushing, wheezing)
kallikrein
bradykinin
tachykinin
prostaglandin
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5
Q

carcinoid syndrome does NOT usually occur with midgut NETs except in which case

A

extensive liver mets

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6
Q

what vitamin deficiency is associated with carcinoid syndrome

A

pellagra (vit B3 def) - large amounts of tryptophan are diverted from niacin synthesis to produce serotonin

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7
Q

carcinoid tumors can also cause which endocrine syndromes

A

cushings (ACTH or CRH producing)

acromegaly (GHRH)

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8
Q

5-HIAA is a metabolite of what

A

serotonin

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9
Q

metabolism of tryptohpan

A

tryptophan - serotonin - 5-HIAA

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10
Q

foods/meds that can cause false + 5-HIAA levels

A

tryptophan, serotonin

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11
Q

diagnosis of carcinoid syndrome

A

elevated 5-HIAA in urine

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12
Q

which test is NOT recommended in diagnosis of carcinoid syndrome

A

chromogranin A (nonspecific)

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13
Q

which carcinoids can cause carcinoid syndrome but have normal 5-HIAA levels

A

foregut carcinoids (lack enzyme to convert tryptophan to serotonin

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14
Q

best imaging to localize NETs

A

CT
MRI (better to eval for hepatic mets)
Octreoscan
DOTOATATE

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15
Q

what % of carcinoid tumors are metastatic at the time of diagnosis

A

90%

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16
Q

treatment of carcinoid syndrome

A 
somatostatin analogues
telotristat (inhibits rate limiting step in serotonin synthesis)
chemo (respond poorly)
radioreceptor therapy
hepatic resection/embolization
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17
Q

symptoms of carcinoid crisis

A

flushing, hypotension, wheezing/bronchospasm

18
Q

pretreatment of pts with carcinoid syndrome prior to going to the OR

A

octerotide 300-500mcg subQ 30-60 min prior to procedure

19
Q

which patients should received prophylaxis to prevent carcinoid crisis

A

symptomatic carcinoid syndrome

metastatic carcinoid - measure 5-HIAA levels and treat if elevated

20
Q

treatment of carcinoid crisis

A 
iv octreotide
glucocorticoids
methotrimeprazine
methoxamine
phentolamine
ondansetron
glucagon
NO ADRENERGIC OR SYMPATHOMIMETIC AGENTS -- can make the condition worse
21
Q

% of PNETs that are nonfunctional

A

50-70%

22
Q

what % of insulinomas are benign?

A

80-90%

23
Q

what % of PNETs (besides insulinoma) are malignant?

A

50-80%

24
Q

what % of MEN1 pts have PNETs?

A

80-100%

25
Q

clinical manifestations of gastrinomas

A

severe peptic ulcer disease
secretory diarrhea
also known as Zollinger-Ellison syndrome

26
Q

typical locations of gastrinomas

A

70% duodenum

25% pancreatic islets

27
Q

diagnosis of gastrinoma

A

serum gastrin level > 1000
gastric pH < 4.0
secretin stimulation test with gastrin rise > 200 pg/mL
off PPI for 1 week

28
Q

functional test for diagnosis of gastrinoma

A

secretin stim test

gastrin rise of > 200 pg/mL 15 minutes after IV secretin administration

29
Q

imaging modalities for gastrinoma localization

A 
CT/MRI
EUS
DOTATATE 
Octreoscan
transportal venous sampling
selective arterial secretin stimulation
30
Q

treatment of gastrinomas (benign)

A

surgery
high dose ppi
somatostatin analogues (octreotide, lanreotide)
H2 blockers
refractory - vagotomy or subtotal gastrectomy

31
Q

treatment of malignant gastrinomas

A

surgery

liver mets - surgery, embolization, liver xplant, radioreceptor therapy, chemo

32
Q

presentation of glucagonomas

A 
DM
wt loss
anemia
necrolytic migratory erythema
VTE
33
Q

diagnosis of glucagonoma

A

glucagon levels > 500 pg/mL (but not always this high)

34
Q

localization of glucagonomas

A 
same as gastrinomas:
CT/MRI
EUS
DOTATATE 
Octreoscan
transportal venous sampling
selective arterial secretin stimulation
35
Q

treatment of glucagonomas

A 
surgery for localized disease
somatostatin analogues
liver-directed therapies
chemo
radioreceptor therapy
tx overall similar to gastrinomas**
36
Q

tx for skin rash associated with glucagonomas

A

zinc, amino acid infusions

37
Q

characteristics of somatostatinomas

A 
DM
weight loss
steatorrhea
hypochloridia
cholelithiasis
38
Q

diagnosis of somatostatinoma

A

elevated serum somatostatin level

39
Q

treatment of somatostatinomas

A

surgery

40
Q

characteristics of VIPomas

A

watery diarrhea
kypokalemia
achlorhydria

41
Q

diagnosis of VIPoma

A

elevated serum VIP level

42
Q

treatment of VIPoma

A

surgery

Endocrine Fellowship Board Review (11 decks)

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