Islet Cell Tumors and MEN Syndromes (Nichols) Flashcards Preview

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Flashcards in Islet Cell Tumors and MEN Syndromes (Nichols) Deck (89):
1

the most common type of pancreatic neuroendocrine tumor to produce an endocrine syndrome is a tumor making ....manifested by ....

insulin with hypoglycemia

2

What are the symptoms of hypoglycemia

shaky, sweaty, nervous, hunger, weak, visual disturbances, palpitations

3

most common visual dist seen w/ hypoglycemia

blurred vision

4

Why don't you get a history of an episode of hypoglycemia from the pt?

many of these episodes are assc with amnesia ∴ ask family and friends

5

signs of hypoplgycemia

diaphoresis, tachycardia, systolic hypertension, tremulousness, pallor, confusion, unusual behavior

6

what are the body's defenses against hypoglycemia

at glc below 80...↓ insulin
at glc below 70... ↑glucagon, epi, and cortisol

7

At what blood glc level do symptoms of hypoglycemia manifest (

below 50

8

how does fibrosis and amyloid impair the body's defenses against hypoglycemia

disrupts intra-islet signaling (via insulin) that would cause the pancreas to ↑glucagon secretion

9

previous episodes of hypoglycemia impair the body's defenses against hypoglycemia by ...

lowing the threshold for sympathoadrenal response ∴ glc must fall even lower for epi and cortisol to be released

10

alcohol impairs hepatic (gluconeogeneis or glycogenolysis)

gluconeogenesis

11

in terms of hypoglycemia, what is the effect of a 1 day vs 2 day vs 3 day binge drinking event without eating
(duh, why waste space in your stomach with mac n cheese when you can polish off that 4th bottle of vodka?!?!--sidebar, how many bottles of vodka could one conceivably finish in a 3 day prd... i couldn't decide on a good number, 1/day didn't sound like enough but 5 seemed excessive? so I settled on 4. Aaand I just rewrote the broken-adult version of goldilocks and the 3 bears... anyway, back to science...)

1 day is unlikely to cause hypoglycemia due to liver glycogen stores (glycogenoslysis is fine) but around day 2 or 3, you run out of glycogen stores. Since the alcohol inhibits gluconeogenesis, the pt will become hypoglycemic!

12

serious consequences of hypoglycemia

seizures, arrhythmias, and DEATH (muahahaha)

13

I think Nichols is rubbing off on me... im including colorful writing in my notes...

please don't tell anyone

14

(Better or worse) control of blood sugar leads to more episodes of hypoglycemia

better/tighter

15

when are insulin dept diabetic pts more prone to attacks of hypoglycemia

during sleep

16

What are additional factors that can contribute the the development of hypoglyemia

renal failure (↓Cx insulin)
missed meals
weight loss
exercise

17

the cycle of recurrent iatrogenic hypoglycemia can be reversed in ____ weeks of ...

2 weeks of careful avoidance of hypoglycemia

18

what do you give all comatose pts

glucose

19

Most NETs are (functional or non-functional)

non-functional

20

____ is elevated in 70% of NETs

serum chromogranin A

21

80-100% of pts with MEN-1 will develop...

pancreatic NET with endocrinopathy?

i couldn't decide what he meant by "one" on that slide, pg. 5

22

salt and pepper chromatin arranged in nests or trabeculae

histo of pancreatic NETs, especially islet cells

23

T or F: malignant vs benign pancreatic NETs are easily differentiated under the microscope

F: they look the same

24

What are the most common sites of metastases for pancreatic NETs

liver > retroperitoneal LN > bone

25

grossly, how can you tell if a tumor is benign or malignant

look to see if it is invading nearly organs/tissue

26

most common presentation of a non-functional pancreatic NET

asymptomatic

some abd pan, obstructive jaundice, anorexia/nausea, palpable mass

27

What are 5 pancreatic NETs discussed (ID 2 most common)

1. insulinoma
2. gastrinomas
somatostatinoma
glucagonoma
VIPoma

28

episodic hypoglycemia

insulinoma

29

insulinomas are generally (aggressive or indolent)

indolent (87% are single benign tumors)

30

histo of insulinoma:

trabeculae + immunostain for insulin

31

causes Zollinger-Ellison Syndrome

gastrinomas

32

What is Zollinger-Ellison Syndrome

unrelenting PUD w/ abd pain and diarrhea

33

Can gastrinomas appear outside the pancreas?

yes, 40% do

34

DM, painful glottis, inflammed lip (cheilitis),
+ weight loss, GI dist, normocytic anemia, thromboembolism, neurophychiatric dist,

glucagonoma

35

erythematous, painful, puritic rash that begins as macules which coalesce, develop central bullae and then erode (leaving hyperpigmentation, and crusting at periph)

Necrolytic Migratory Erythema

36

describe the spread of Necrolytic Migratory Erythema

perirectum → perineum, thighs, butt, legs

37

Necrolytic Migratory Erythema is assc with what pancreatic NET

glucagonoma

*but is not specific to glucagonoma

38

Assc with DM and weight loss

glucagonoma

39

Why are glucagonomas assc with DM and weight loss

glucagon opposes the action of insulin (~resistance)
and it is lipolytic

40

use of glucagon in ER

resuscitation of hypoglycemic pt when there is no vascular access

overdose of BB and CCB
*Glucagon activates adenyl cyclase and exerts an inotropic and chronotropic effect (↑cAMP) by a pathway that bypasses the b receptors

41

if glucagon fails to reverse BB and CCB OD, what can be used

insulin + glc

42

DM + Cholelithiasis + steatorrhea

somatostatinoma syndrome (this seen in only 10% of somatostatinoma cases...)

43

explain pathyphys behind the sydrome assc with somatostatinoma syndrome

1. DM: stomatostatin ↓insulin release

2. Cholelithiasis: stomatostatin ↓cholecystokinin release → ↓gallbladder emptying

3. steatorrhea: inhibits pancreatic sectrion and lipid absorption

44

treatment forpancreatic NETs

1. surgical resection
2. sunitinib and everolimus if unresectable
3. octreotide to control symptoms if unresectable

45

parathyroid, pancreas, pituitary

MEN-1:

46

hypoglycemia hypoglycemia hypoglycemia

insulinoma

47

severe watery diarrhea, achlorydria, hypokalemia, acidosis

VIPoma

48

PUD, diarrhea

gastrinoma
*Zollinger-Ellison Syndrome

49

3 words to remember for...
tumor secreting vasoactive intestinal peptide

VIPoma

50

DM, cholelitiasis, steatorrhea

somatostatinoma

51

pancreatic cholera
verner-morrison syndrome

VIPomas

52

most common symptoms of somatostatinomas

abd pain and weight loss

53

thyroid medullary, pheocromocytoma

MEN-2

54

necrolytic migratory erythema

glucagonoma

55

watery diarrhea, achlorhydria

VIPoma

56

DM, painful glottis, inflammed lip (cheilitis),

glucagonoma

57

serum chromogranin A

pancreatic NETs

58

DM and weight loss

glucagonoma

59

how can MEN cause hyperCa?

MEN is a parathyroid adenoma causing hyper PTH which ↑Ca CHRONICALLY

60

urinary pain + endocrinopathy think...

chronic Ca elevation
*if fever, UTI

61

epidemiolgy for primary hyperPTH

inc incidence with age
women > men
blacks and asians

62

hypercalcemia + bilateral hilar lymphadenopathy + non-caseating granulomas

sarcoid

63

why can sarcoid present with hyperCa?

macrophages in granulomas convert vitD to mature form (NO FEEDBACK REG) → ↑bone resorption → ↑Ca

**only 20% of sarcoid pts have hyperCa

64

can mimic islet cell tumor or MEN

paraneoplastic syndrome

65

most common paraneoplastic syndrome

hyper Ca

66

Acute vs chronic hyperCa presentation

chronic: mostly asymptomatic, maybe mild anxiety, depression or cognitive difficulties

acute:
muscle weakness, ↓muscle reflexes,
confusion, lethargy → seizure → coma → death
constipation → nausea and vomiting
polyuria and polydipsia (interfere with ADH)
shortened OT and bradycardia

67

asymptomatic and primary hyperPTH

chronic hyperCa

68

confusion, lethargy, obtundation, and paraneoplastic

acute hyperCa

69

amenorrhea and galactorrhea

prolactinoma

70

prolactinoma is the most common ...

pituitary adenoma

71

lactation failure, amenorrhea, asthenia, apparent pre-mature aging, dryness and hypopigmentation of skin, genital and axillary hair loss
*post-partum

sheehan syndome

72

what is the predomidant manifestation of acute adrenal insufficiency?

shock
*+nonspecific symptoms, weight loss, fatigue, nausea, vom, abd pain, weak, fatigue, fever, confusion

CANNOT HANDLE STRESSES TO BODY

73

what color are adrenal cortical tumors? why?

yellow/gold

Cortisol/cholesterol

74

postpartum hypopit due to necrosis of the gland from peripartum hemorrhagic shock

sheehan syndrome

75

hypertension and hypokalemia

Conn syndrome seen in aldosterone secreting adrenal cortical tumors

76

majority of adrenal cortical tumors are (functional or non-functional)

nonfunctional

77

catecholamine secreting tumor of the adrenal medulla

pheochromocytoma

78

what are 2 substances adrenal cortical tumors can secrete

cortisol and aldosterone

79

HTN, episodic headaches, sweating, tachycardia

pheochromocytoma

80

A 35-year-old black female presents with the acute onset of painful urination associated with increased urination, constipation, anorexia, fatigue, depressed mood and confusion. She most likely has...

hypercalcemia

81

symptoms of acute hyperCa and hypoCa

Hyper: Stones, (abdominal) Moans, (psychic), Groans, Bones, constipation

hypo:parasthesias (esp lips), muscle cramps, irritability, depression

82

symptoms of acute hyperglycemia and hypoclycemia

hyper: polyuria, polydipsia,polyphagia, fatigue, weight loss

Hypoglycemia: shakiness, sweatiness,nervousness, hunger, weakness

83

symptoms of acute hyperK and hypoK

Hyperkalemia: fatigue, weakness, nausea,
parathesias, confusion

Hypokalemia: fatigue, weakness, constipation,
irritability, myalgias

84

symptoms of acute hyperNa and hypoNa

Hypernatremia: lethargy, confusion,
irritability, seizures, coma


Hyponatremia: lethargy, confusion,
irritability, seizures, coma*

85

Urolithiasis is suggestive of (acute or chronic) hypercalcemia

chronic

86

what makes an presentation of hypercalcemia more severe?

↑# symptoms and more severe neurological impariment

87

how can you differentiates parathyroid adenoma from hyperplasia

hyperplasia is in more than one glad
adenomas are uni-focal

88

pre-mature menopause in a person wil DM for 15+ years

accelerated aging

89

tumors of parathyroid, pancreas, pituitary

MEN-1