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ICC2 > GIST and endocrine tumors > Flashcards

GIST and endocrine tumors Flashcards

1
Q

what is the most common type of sarcoma ?

A

gastrointestinal stromal tumors

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2
Q

which cells are affected in GIST ?

A

interstitial cells of cajal

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3
Q

what is the most common site of GIST ?

A

stomach

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4
Q

what is the gene mutation involved in GIST ?

A

C-KIT which is the receptor for stem cell factor

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5
Q

where are cells of cajal located ?

A

muscularis propria

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6
Q

what is the most common presentation of GIST ?

A

bleeding or abdominal pain

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7
Q

how is a definitive diagnosis of GIST made ?

A

through histological examination and immunohistochemistry

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8
Q

what is the most specific marker ?

A

DOG1

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9
Q

what is the most useful marker in GIST?

A

c-KIT CDK117, which is used in targeted therapy

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10
Q

what is the treatment for GIST ?

A

surgical resection
gleevec - imatinib

imatinib - gleevec

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11
Q

what are the neuroendocrine tumors that cause chronic diarrhea ?

A 
gastrinoma 
insulinoma 
VIPoma 
Glucagonoma 
somatostatinoma 
carcinoid
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12
Q

what is the mediator for gastrinoma and the associated effect ?

A

gastrin associated with zollinger ellison syndrome

zollinger-ellison syndrome

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13
Q

what is the mediator for insulinoma and the associated effect ?

A

insulin associated with recurrent hypoglycemia

recurrent hypoglycaemia

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14
Q

what is the mediator for VIPoma and the associated effect ?

A

vasoactive intestinal peptide associated with vernerr morrison syndrome

Vernner-morrison syndrome

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15
Q

what is the mediator for glucagonoma and the associated effect ?

A

glucagon
Skin rashes ( migrating necrotizing erythema)
diabetes

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16
Q

what is the mediator for somatostatinoma and the associated effect ?

A

somatostatin
non-ketotic DM
steatorrhea
gall stones

17
Q

what is the mediator for carcinoid and the associated effect ?

A

serotonin, kinins , associated with flushing, wheezing, right sided cardiac valvular disease

flushing , wheezing and right sided valvular heart disease

18
Q

what is zollinger ellison syndrome ?

A

peptic ulcer disease due to overproduction of acid , ulcers refractory to treatment
ulcers with unexplained diarrhea

19
Q

how do we diagnose zollinger ellison syndrome ?

A

fasting hypergastrinemia , patient must be off PPis and H2 agonists

patient must be off h2 receptor blockers

20
Q

if the patient is suffering from pulmonary murmur and flushing what should we suspect ?

A

carcinoid

21
Q

if the urine 5HIAA is high what should we suspect ?

A

carcinoid

22
Q

what is whipples triad ?

A

symptoms of hypoglycaemia
low blood glucose measure
symptoms revolve when glucose is administered
which is suggestive of insulinoma

23
Q

what is the 4D syndrome associated with ?

A 
diabetes 
dermatitis 
DVT 
depression
are all associated with glucagonoma
24
Q

what is the prominent symptom inn VIPoma ?

A

severe diarrhea despite fasting

25
Q

what is the pentad of somatostatinoma ?

A 
DM 
cholethiasis 
weight loss 
stratorrhea and diarrhea 
hypochlorydia
26
Q

what are the features of MEN 1?

A

3ps
pancreatic tumors
pituitary adenoma
parathyroid hyperplasia

27
Q

what is verner morrison syndrome ?

A

watery diarrhea associated with hypokalemia and achlorhydria which is seen in VIPoma

28
Q

what do all the NET secrete that is common between them ?

A

chromogranins

29
Q

what are the instructions that should be followed when taking a gut hormonal panel ?

A

PPIs should be stopped for 2 weeks
H2 agonists for 72 hrs
sample sent to lab on ice

30
Q

when should you suspect zollinger ellison syndrome ?

A

duodenal ulcers not due to h.pylori
ulcers with unexplained diarrhea
recurrent ulcers
ulcers refractory to standard treatment

31
Q

how is a diagnosis of carcinoid confirmed ?

A

24 hour urine collection in acidified bottle

ICC2 (42 decks)

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