What is Whipple's triad?
2) low plasma glucose measured when symptomatic
3) relief of symptoms with administration of glucose
The presence of what type of ulcer should raise your suspicion for gastrinoma?
Jejunal
What is WDHA syndrome?
Watery diarrhea, hypokalemia, achlorhydria
Which biomarker is useful in not only diagnosis confirmation but also post-treatment surveillance?
Chromogranin A
What is the endocrine pancreas' primary physiologic function?
Glucose and insulin regulation
What is the average age of diagnosis?
45 years old
What are the boundaries of the gastrinoma triangle?
Cystic duct, junction between 2nd & 3rd portions of duodenum, junction between neck & body of pancreas
What are the 4D's associated with glucagonoma?
Diabetes, dermatitis, DVTs, depression
Capture of this particular phase in CT is important in distinguishing PNETs from other pancreatic tumors
Vascular blush in arterial phase
Pancreatic islet cells are composed of these 4 types of cells
A (alpha), B (beta), D and F cells
What is the gold standard for diagnosis?
72 hour monitored fast
More than this percentage of patients have metastatic disease at the time of diagnosis
50%
What is the standard treatment for these tumors?
Complete anatomic resection, if possible
What is the next step if unable to localize the tumor on CT or MRI?
EUS (90% sensitivity for tumors of all sizes)
Biochemical screening for patients suspected of having MEN1 includes...
Gastrin, insulin, proinsulin, PP (pancreatic polypeptide), glucagon, and chromogranin A
What is the mainstay of treatment?
Surgical resection - the only curative option
What is the best predictor of survival?
Presence of liver mets
(lymph node mets are NOT predictive)
Somatostatinomas are associated with these 2 diseases/tumors
von Recklinghausen's disease and pheochromocytomas
What does ghrelin ultimately do to the body?
Causes hyperglycemia and impairs glucose tolerance
What is the percentage of benign insulinomas? Malignant?
95%, 5%
How does pancreaticoduodenectomy improve disease-free survival in patients with MEN1?
Following local excision, recurrent tumors are often found in the duodenum
How much diarrhea is considered watery diarrhea (in terms of volume)?
3-5L per day
less than 700 mL per day is less likely VIPoma
What can be used instead of oral contrast to identify small duodenal gastrinomas?
Water
What percentage of islet cells need to be removed before endocrine dysfunction becomes apparent?
85%