Endocrine pancreas syndromes Flashcards
Are PNETs more commonly functional or non functional?
non functional
what percent of PNETs are malignant?
75%
where is the MC site of mets for PNETs?
liver
what is octreotide useful for?
effective for symptoms with insulinoma, gastronome, glucagonoma, VIPoma
Most common PNETs in the head of the pancreas?
gastronome, somatostatinoma
which PNETs should have cholecystectomy?
glucagonoma and VIPoma to avoid gallstones with prolonged octreotide use. Also use for somatostatinoma for mets use
what is the MC functional PNET?
insulinoma
what is the Whipple triad? (for insulinoma)
fasting hypoglycemia (<55), symptoms of hypoglycemia (confusion, LOC, seizures, increase HR, diaphoresis), relief with glucose admin
are most insulinomas malignant or benign?
benign
where are insulinomas found
throughout the pancreas
If insulin and C peptide are not elevated after 72 hr fast, what would you suspect?
Manchausen’s syndrome
what imaging localizes the insulinoma?
triple phase CT (or MRI) and EUS
If cannot locate insulinoma with CT or MRI or EUS what is the next step?
selective intra-arterial calcium injection with hepatic venous sampling for insulin
which PNET is somatostatin scintigraphy not helpful for?
insulinoma
what is the treatment for insulinoma
enucleate if <2 cm or formal resection if >2 cm
what is the treatment for insulinoma with mets?
5FU and streptozocin, octreotide
Diazoxide for symptoms
are gastrinomas more commonly spontaneous or from MEN 1?
spontaneous
what percent of gastrinomas are found in the triangle?
75%
what is a tip off to gastrinomas?
ulcers past 1st portion of duodenum
what are the tests for gastrinomas?
fasting gastrin >1000 is diagnostic, secretin test (positive will have decreased gastrin, normal will have increased gastrin)
what is the best imaging for gastrinomas?
somatostatin scintigraphy (get second if not found on CT or MRI)
what is the first imaging for PNETs?
CT triple phase or MRI
what is the treatment for gastrinomas?
enucleation (<2 cm), formal resection with lymph nodes (>2 cm)
what is the treatment for metastatic gastrinomas?
50% of pts. focus on symptom control
what are the symptoms of glucagonoma?
4D’s: diabetes, dermatitis (necrolytic migratory erythema), depression, DVT, weight loss
mostly malignant PNET
glucagonoma, 90% (distal pancreas)
treatment for glucagonoma
formal resection w/regional lymph node dissection for all (high malignancy rate) + cholecystectomy
what can you give to help the rash with glucagonomas?
zinc, amino acids, fatty acids
what are the symptoms with VIPoma
watery diarrhea, hypokalemia, achlorhydria (WDHA)
are VIPomas mostly benign or malignant?
malignant
where are VIPomas found usually
distal pancreas
what is the treatment for VIPomas
formal resection with lymph node dissection + cholecystectomy
What are the symptoms of somatostatinoma?
diabetes, gallstones, steatorrhea
how do you dx somatostatinoma?
fasting somatostatin level
are somatostatinomas mostly benign or malignant?
malignant
what is the treatment for somatostatinomas?
formal resection with lymph node dissection + cholecystectomy
glucagon is made where
alpha cells
insulin is made where
beta cells (at center of islets)
somatostatin is made where
D cells
pancreatic polypeptide is made where
PP or F cells
VIP and serotonin are made where
islet cells
what does enterokinase do
released by duodenum and activates trypsinogen to trypsin
what does trypsin do
activates other pancreatic enzymes including trypsinogen
what does secretin do
increase HCO3 mostly
what does CCK do?
increase pancreatic enzymes
what does acetylcholine do
increase HCO3 and enzymes
what does somatostatin and glucagon do
decrease exocrine function
where does CCK and secretin come from
most released by cells in duodenum
what is the affect of CCK and glucagon on the sphincter of Oddi?
relaxes the sphincter
what are the exocrine enzymes of the pancreas?
amylase, lipase, trypsinogen, chymotrypsinogen, carboxypeptidase, HCO3
what is the only pancreatic enzyme that is released in its active form?
amylase - hydrolyzes alpha 1-4 linkages of glucose chains
