Neuroendocrine Tumors Flashcards
NET’s
Neoplasms that arise from endocrine and nervous tissue.
MEN Syndromes
Multiple Endocrine Neoplasms
Cause overactivity and enlargement, tumors of certain endocrine glands.
Usually inherited
Autosomal dominant
One type of MEN sticks to a single family
MEN-1
ParaT tumors
Pancreatic tumors
Pituitary tumors
MEN-2a
Medullary thyroid cancers
Pheochromocytoma
Parathyroid
Very rare
MEN-2b
Medullary thyroid cancers
Pheochromocytomas
Neuromas
MEN-1 Manifestation
Almost all MEN-1 patients develop overactivity of parathyroid (hyperparathyroidism)
Pancreas is next: gastrin and insulin overproduction
Pituitary adenoma occurs in 42% of pt’s
MEN-1 tumors are usually?
Benign
MEN-2a will mosr certainly develop which type of cancer?
Medullary thyroid cancer
Very aggressive
Begins early on in life
Should have thyroid surgically removed
Testing for MEN-2a
ret protooncogene mutation
MEN-2b
Medullary thyroid cancers
Pheochromocytoma
Mucosal neuromas
Marfanoid Habitus
Seen in MEN-2b Long fingers and extremities Tall, long legs and arms Pectus Excavatum Spinal abnormalities
Insulinoma
Rare beta cell tumor that secretes insulin Evenly distributed Hyperinsulinemia Persistant hypoglycemia More common in women 90% benign
Insulinoma S/S
Polyphagia, weight gain
CNS glusoce lack
Whipples triad
Insulinoma Dx
Insulin to glucose ratio <.3
Increased levels of C-peptide
Insulinoma Tx
Surgery
DIazoxide
Octreotide
Continuous SQ glucagon
Gastrinoma
Zollinger-Ellison Syndrome
Tumor of pancreas and duedenum
Excess levels of gastrin
25% part of MEN-1
Gastrinoma S/S
Frequent peptic ulcers (absence of NSAIDS and H.Pylori
esophagitis
diarrhea
Gastrinoma Dx
Fasting gastrin > 1000
Somatostatin receptor scintigraphy
Endoscopic ultrasound
Gastrinoma Tx
Proton pump inhibitors
Surgical removal
VIPoma
Verner Morrison Syndrome
Pancreatic tumor secreting Vasoactive intestinal polypeptide (VIP)
Tail of pancreas
VIPoma S/S
Severe, intermittent watery diarrhea
Dehydration and weakness
Decreased gastrin production
Flushing
VIPoma Dx
Serum VIP levels 75 - 190
CT, US
VIPoma Tx
Fluid correction
Surgical rescection
Glucagonoma
Pancreatic tumor that secretes glucagon Increased levels of glucagon in blood Produced rash 80% are cancerous Grows slowly 80% women
