Pancreatic Neuroendocrine Tumors and Multiple Endocrine Neoplasia: Gupta Flashcards
Bona fide carcinomas are dx’d on the basis of…
local invasion and distant metastasis.
Pancreatic neuroendocrine tumor syndromes at a glance:
Glucagonoma clinical features:
VERY RARE -alpha cell tumor \::*T2DM* \::Necrolytic migratory erythema (NME)- *blisters on high friction areas such as gluteal cleft, perineum, and groin.* \::Anemia \::High plasma glucagon levels
Pancreatic neuroendocrine tumor syndromes at a glance:
Insulinoma clinical features:
MOST COMMON -Beta cell tumor *Hypoglycemia* Confusion Muscle weakness Blurred vision Sweating- ^ SNS response to stress of hypogly. Palpitations- ^ SNS response to stress of hypogly.
Remember, it is essentially presenting as hypoglycemia. The person may appear to be retarded. But give them some 50% dextrose and BAM!! not retarded. Then, get that damn tumor out of there, +/- octreotide.
Pancreatic neuroendocrine tumor syndromes at a glance:
Somatostatinoma clinical features:
VERY RARE
-delta cell tumor
::Cholelithiasis- inhibits cholecystokinin release–> no GB emptying
::Diabetes- inhibits insulin release from B cells
::Steatorrhea- inhibits pancreatic secretion and lipid absorption
::Achlorhydria (low HCl production)
Pancreatic neuroendocrine tumor syndromes at a glance:
Gastrinoma clinical features:
Zollinger-Ellison Syndrome - unrelenting peptic ulcer dz, hypersecretion of acid and pancretic islet cell lesion.
Peptic ulcers
25% are in pts with MEN1 syndrome
Pancreatic neuroendocrine tumor syndromes at a glance:
VIPoma (vasoactive intestinal peptide) clinical features:
VERY RARE
Vermer-Morrison Syndrome (pancreatic cholera)
Achlorhydria (low HCl production) (WDHA syndrome)
Severe diarrhea (WDHA syndrome)
Hypokalemia (WDHA syndrome)
Acidosis
Hypovolemia
In at pt with severe secretory diarrhea, a VIP assay should be ordered
The most common type of pancreatic neuroendocrine tumor to produce and endocrine syndrome is:
Insulinoma- generally indolent tumors 87% single benign tumors 7% multiple benign tumors 10% malignant tumors 8% part of MEN1 syndrome
Describe the histology of an insulinoma.
May reveal abundant amyloid. Trabecular pattern.
Describe epidemiology and other key findings in Multiple Endocrine Neoplasia (MEN) syndromes- (morphology, timing of presentation, aggression, recurrence).
Inherited dz of multiple endocrine organs
Can be synchronous or metachronous
Are often multifocal
Typically occur in younger pts
Typically have hyperplasia of cell before neoplasm
Typically more aggressive and recur (vs. sporadic)
What are the 3 P’s of MEN-1?
Pituitary- Most freq. Prolactin secreting adenoma.
Pancreas- This is what kills the pt. Often multiple, functional, and aggressive.
Parathyroid- most common manifestation (this is usually how they present), primary hyperparathyroidism
What is the gene involved in MEN-1 and what is its normal function?
MEN-1: encodes menin- tumor suppressor
Describe what is going on in MEN-2 mutations
MEN-2 is a gain of function mutation of RET (encodes an RTK)
Two groups of disorders Type 2A and Type 2B
Strong genotype-phenotype correlation
MEN-2 is autosomal dominant
What malignancy to MEN-2A pts get 100% of the time?
Medullary carcinoma (usually in first 2 decades of life)
50% get pheochromocytomas
10-20% get hyperparathyroidism due to hyperplasia of PT gland
What do you stain medullary carcinoma with?
Stain for Calcitonin, bc this is a cancer of C cells
What malignancy to MEN-2B get 100% of the time?
Describe some other key features differentiating it from 2A.
Medullary carcinoma
Also get pheochromocytomas
Differentiate 2B from 2A: no hyperparathyroidism in 2B
Extra clinical manifestations: neuromas/ganglioneuromas, marfanoid habitus
