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Pathology > Pancreatic Neuroendocrine tumors and MEN > Flashcards

Pancreatic Neuroendocrine tumors and MEN Flashcards

1
Q

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

A

Tumor making insulin

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2
Q

Symptoms of hypoglycemia

A

Shakiness, sweating, nervousness

Hunger, weakness, visual disturbances, palpitations

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3
Q

Most common visual disturbance of hypoglycemia

A

Blurred vision

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4
Q

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

A

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

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5
Q

Signs of hypoglycemia

A

diaphoresis, tachycardia, systolic hypertension

Tremulousness, pallor, confusion, unusual behavoir

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6
Q

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

A
  1. Decreased insulin (glucose < 80)
  2. Increased glucagon ( <70)
  3. Increased epinephrine ( < 70)
  4. Increased cortisol ( <70)
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7
Q

What obliterates the bodies first defense against hypoglycemia

A

Exogenous insulin

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8
Q

What impairs the bodies second response to hypoglycemia

A

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

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9
Q

What lowers the threshold for the third defense of hypoglycemia

A

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

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10
Q

General characteristics of pancreatic neuroendocrine tumors

A

Rare

Mostly in middle aged patients

Mostly sporadic

Up to 75% are nonfunctioning

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11
Q

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

A

Serum chromogranin A

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12
Q

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

A

80-100%

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13
Q

What do neuroendocrine tumors look like on histopath

A

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

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14
Q

what is seen here?

A

Pancreatic neuroendocrine tumor with “salt and pepper” chromatin pattern

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15
Q

Architecture of pancreatic NETs

A

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

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16
Q

3 most common sites of pancreatic NETs mets

A

Liver

Retroperitoneal lymph nodes

Bone

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17
Q

Symptoms of pancreatic NETs

A

Most commonly asymptomatic

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

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18
Q

Most common pancreatic NET, features?

A

Insulinoma

Generally single and benign

19
Q

What is seen here?

A

Insulinoma as part of MEN-1

20
Q

What is seen here?

A

Isulinoma on right, non-neoplastic pancreas on right

21
Q

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

A

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

22
Q

Symptoms of glucagonoma syndrome

A

DM, Painful glossitis, cheilitis, anemia

23
Q

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

A

Glucagonoma

24
Q

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

A

DM: opposes actions of insulin

Weight loss: catabolic (lipolytic)

25
Q

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

A

Glucagon

Also first line therapy for treating B-blocker overdoses

26
Q

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

A

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

27
Q

VIPoma symptoms

A

Watery diarrhea, hypokalemia, achlorhydria

28
Q

Best test to dx PNETs

A

Endoscopic ultrasound

29
Q

3 words for MEN-1**

A

Parathyroid, pancreas, pituitary

30
Q

3 words for MEN-2

A

Thyroid, medullary, pheochromocytoma

31
Q

Word for insulinoma**

A

Hypoglycemia

32
Q

Buzzword for glucagonoma**

A

Necrolytic migratory erythema

33
Q

Buzzwords for gastrinoma**

A

Peptic ulcers, diarrhea

34
Q

3 words for somatostainoma**

A

diabetes, cholelithiasis, steatorrhea

35
Q

Buzz words for VIPoma

A

Watery diarrhea, achlorhydria

36
Q

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

A

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

37
Q

Symptoms of chronic hypercalcemia

A

Usually none

If present: vague mild anxiey, depression, cognitive difficulities

38
Q

Most common paraneoplastic syndrome

A

Hypercalcemia

39
Q

Symptoms of acute hypercalcemia

A

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

40
Q

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

A

Interference w/ function of ADH

41
Q

Acute hypercalcemia buzzwords**

A

Confusion, lethargy, obtundation, paraneoplastic syndrome

42
Q

Chronic hypercalcemia buzzwords**

A

Asymptomatic and primary hyperparathyroidism

43
Q

MEN-1 mutation**

A

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

44
Q

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

A

2A: germline mutation in RET protooncogene

2B: germline activating RET mutation, but is point mutation

Pathology (10 decks)

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