GIST and endocrine tumors Flashcards
what is the most common type of sarcoma ?
gastrointestinal stromal tumors
which cells are affected in GIST ?
interstitial cells of cajal
what is the most common site of GIST ?
stomach
what is the gene mutation involved in GIST ?
C-KIT which is the receptor for stem cell factor
where are cells of cajal located ?
muscularis propria
what is the most common presentation of GIST ?
bleeding or abdominal pain
how is a definitive diagnosis of GIST made ?
through histological examination and immunohistochemistry
what is the most specific marker ?
DOG1
what is the most useful marker in GIST?
c-KIT CDK117, which is used in targeted therapy
what is the treatment for GIST ?
surgical resection
gleevec - imatinib
imatinib - gleevec
what are the neuroendocrine tumors that cause chronic diarrhea ?
gastrinoma insulinoma VIPoma Glucagonoma somatostatinoma carcinoid
what is the mediator for gastrinoma and the associated effect ?
gastrin associated with zollinger ellison syndrome
zollinger-ellison syndrome
what is the mediator for insulinoma and the associated effect ?
insulin associated with recurrent hypoglycemia
recurrent hypoglycaemia
what is the mediator for VIPoma and the associated effect ?
vasoactive intestinal peptide associated with vernerr morrison syndrome
Vernner-morrison syndrome
what is the mediator for glucagonoma and the associated effect ?
glucagon
Skin rashes ( migrating necrotizing erythema)
diabetes
what is the mediator for somatostatinoma and the associated effect ?
somatostatin
non-ketotic DM
steatorrhea
gall stones
what is the mediator for carcinoid and the associated effect ?
serotonin, kinins , associated with flushing, wheezing, right sided cardiac valvular disease
flushing , wheezing and right sided valvular heart disease
what is zollinger ellison syndrome ?
peptic ulcer disease due to overproduction of acid , ulcers refractory to treatment
ulcers with unexplained diarrhea
how do we diagnose zollinger ellison syndrome ?
fasting hypergastrinemia , patient must be off PPis and H2 agonists
patient must be off h2 receptor blockers
if the patient is suffering from pulmonary murmur and flushing what should we suspect ?
carcinoid
if the urine 5HIAA is high what should we suspect ?
carcinoid
what is whipples triad ?
symptoms of hypoglycaemia
low blood glucose measure
symptoms revolve when glucose is administered
which is suggestive of insulinoma
what is the 4D syndrome associated with ?
diabetes dermatitis DVT depression are all associated with glucagonoma
what is the prominent symptom inn VIPoma ?
severe diarrhea despite fasting
what is the pentad of somatostatinoma ?
DM cholethiasis weight loss stratorrhea and diarrhea hypochlorydia
what are the features of MEN 1?
3ps
pancreatic tumors
pituitary adenoma
parathyroid hyperplasia
what is verner morrison syndrome ?
watery diarrhea associated with hypokalemia and achlorhydria which is seen in VIPoma
what do all the NET secrete that is common between them ?
chromogranins
what are the instructions that should be followed when taking a gut hormonal panel ?
PPIs should be stopped for 2 weeks
H2 agonists for 72 hrs
sample sent to lab on ice
when should you suspect zollinger ellison syndrome ?
duodenal ulcers not due to h.pylori
ulcers with unexplained diarrhea
recurrent ulcers
ulcers refractory to standard treatment
how is a diagnosis of carcinoid confirmed ?
24 hour urine collection in acidified bottle
