Pancreatic Neuroendocrine tumors and MEN

Question 1

Most common type of pancreatic neuroendocrine tumor to produce endocrine syndrome?

Answer 1

Tumor making insulin

Question 2

Symptoms of hypoglycemia

Answer 2

Shakiness, sweating, nervousness

Hunger, weakness, visual disturbances, palpitations

Question 3

Most common visual disturbance of hypoglycemia

Answer 3

Blurred vision

Question 4

Why do you need family/friends to aid a dx of hypoglycemia

Answer 4

Many patients have amnesia of episodes of hypoglycemia so history will be a false negative (like patients on benzos or with petit mal seiures)

Question 5

Signs of hypoglycemia

Answer 5

diaphoresis, tachycardia, systolic hypertension

Tremulousness, pallor, confusion, unusual behavoir

Question 6

Top 4 things involved in bodies response to hypoglycemia (and when)

Answer 6

1. Decreased insulin (glucose < 80)
2. Increased glucagon ( <70)
3. Increased epinephrine ( < 70)
4. Increased cortisol ( <70)

Question 7

What obliterates the bodies first defense against hypoglycemia

Answer 7

Exogenous insulin

Question 8

What impairs the bodies second response to hypoglycemia

Answer 8

Islet fibrosis or amyloid becuase intra-islet insulin is signal for increased glucagon

Question 9

What lowers the threshold for the third defense of hypoglycemia

Answer 9

Previous episodes of hypoglycemia lower the third defense, sympatho-adrenal response

Question 10

General characteristics of pancreatic neuroendocrine tumors

Answer 10

Rare

Mostly in middle aged patients

Mostly sporadic

Up to 75% are nonfunctioning

Question 11

What is elevated in about 70% of pt’s with pancreatic NETs

Answer 11

Serum chromogranin A

Question 12

Percentage of patients with MEN-1 likely to develop pancreatic neuroendocrine tumor?

Answer 12

80-100%

Question 13

What do neuroendocrine tumors look like on histopath

Answer 13

small, bland and uniform looking cells with “salt and pepper” chromatin

Question 14

what is seen here?

Answer 14

Pancreatic neuroendocrine tumor with “salt and pepper” chromatin pattern

Question 15

Architecture of pancreatic NETs

Answer 15

Nests, trabeculae, stands, islets, glands, or sheets

Question 16

3 most common sites of pancreatic NETs mets

Answer 16

Liver

Retroperitoneal lymph nodes

Bone

Question 17

Symptoms of pancreatic NETs

Answer 17

Most commonly asymptomatic

When present: Ab pain, jaundice, anorexia/nausea, palpable mass, weight loss

Question 18

Most common pancreatic NET, features?

Answer 18

Insulinoma

Generally single and benign

Question 19

What is seen here?

Answer 19

Insulinoma as part of MEN-1

Question 20

What is seen here?

Answer 20

Isulinoma on right, non-neoplastic pancreas on right

Question 21

Second most common cause of pancreatic NETs, what does that cause?

Answer 21

Gastrinomas, cause Zollinger-Ellison syndrome (PUD, Ab pain, and diarrhea)

Question 22

Symptoms of glucagonoma syndrome

Answer 22

DM, Painful glossitis, cheilitis, anemia

Question 23

What is necrolytic migratory erythema seen in (in this block)?

Answer 23

Glucagonoma

Question 24

Why does a glucagonoma cause DM? Why does it cause weight loss?

Answer 24

DM: opposes actions of insulin

Weight loss: catabolic (lipolytic)

Question 25

What can be used in resuscitation of comatose pt's with hypoglycemia if no vascular access? What else is that first line therapy for?

Answer 25

Glucagon Also first line therapy for treating B-blocker overdoses

Question 26

3 things seen in somatostatinoma syndrome? Why is each caused?

Answer 26

1) DM --\> inhibits insulin release from B cells 2) Cholelthiasis --\> inhibits cholecystokinin release (thus gallbladder empying) 3) Steatorrhea --\> inhibits pancreatic secretion and lipid absorption

Question 27

VIPoma symptoms

Answer 27

Watery diarrhea, hypokalemia, achlorhydria

Question 28

Best test to dx PNETs

Answer 28

Endoscopic ultrasound

Question 29

3 words for MEN-1\*\*

Answer 29

Parathyroid, pancreas, pituitary

Question 30

3 words for MEN-2

Answer 30

Thyroid, medullary, pheochromocytoma

Question 31

Word for insulinoma\*\*

Answer 31

Hypoglycemia

Question 32

Buzzword for glucagonoma\*\*

Answer 32

Necrolytic migratory erythema

Question 33

Buzzwords for gastrinoma\*\*

Answer 33

Peptic ulcers, diarrhea

Question 34

3 words for somatostainoma\*\*

Answer 34

diabetes, cholelithiasis, steatorrhea

Question 35

Buzz words for VIPoma

Answer 35

Watery diarrhea, achlorhydria

Question 36

90% of chronic hyper-calcemia due to? Which is usually due to what?

Answer 36

Primary hyperparathyroidism, usually due to parathyroid adenoma (cyclin D)

Question 37

Symptoms of chronic hypercalcemia

Answer 37

Usually none If present: vague mild anxiey, depression, cognitive difficulities

Question 38

Most common paraneoplastic syndrome

Answer 38

Hypercalcemia

Question 39

Symptoms of acute hypercalcemia

Answer 39

Musculoskeletal/nervous system: muscle weakness, decreased muscular reflexes, confusion

Question 40

Why can pt's with acute hypercalcemia have polyuria and polydispia

Answer 40

Interference w/ function of ADH

Question 41

Acute hypercalcemia buzzwords\*\*

Answer 41

Confusion, lethargy, obtundation, paraneoplastic syndrome

Question 42

Chronic hypercalcemia buzzwords\*\*

Answer 42

Asymptomatic and primary hyperparathyroidism

Question 43

MEN-1 mutation\*\*

Answer 43

Germline mutation in MEN-1 tumor suppressor gene, which normally blocks _JunD_

Question 44

MEN-2A mutation? MEN-2B mutation?

Answer 44

2A: germline mutation in _RET protooncogene_ 2B: germline activating _RET_ mutation, but is _point mutation_