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Endo > Islet Cell Tumors and MEN Syndromes (Nichols) > Flashcards

Islet Cell Tumors and MEN Syndromes (Nichols) Flashcards

1
Q

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

A

insulin with hypoglycemia

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2
Q

What are the symptoms of hypoglycemia

A

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

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3
Q

most common visual dist seen w/ hypoglycemia

A

blurred vision

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4
Q

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

A

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

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5
Q

signs of hypoplgycemia

A

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

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6
Q

what are the body’s defenses against hypoglycemia

A

at glc below 80…↓ insulin

at glc below 70… ↑glucagon, epi, and cortisol

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7
Q

At what blood glc level do symptoms of hypoglycemia manifest (

A

below 50

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8
Q

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

A

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

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9
Q

previous episodes of hypoglycemia impair the body’s defenses against hypoglycemia by …

A

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

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10
Q

alcohol impairs hepatic (gluconeogeneis or glycogenolysis)

A

gluconeogenesis

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11
Q

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…)

A

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!

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12
Q

serious consequences of hypoglycemia

A

seizures, arrhythmias, and DEATH (muahahaha)

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13
Q

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

A

please don’t tell anyone

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14
Q

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

A

better/tighter

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15
Q

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

A

during sleep

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16
Q

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

A

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

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17
Q

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

A

2 weeks of careful avoidance of hypoglycemia

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18
Q

what do you give all comatose pts

A

glucose

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19
Q

Most NETs are (functional or non-functional)

A

non-functional

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20
Q

____ is elevated in 70% of NETs

A

serum chromogranin A

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21
Q

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

A

pancreatic NET with endocrinopathy?

i couldn’t decide what he meant by “one” on that slide, pg. 5

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22
Q

salt and pepper chromatin arranged in nests or trabeculae

A

histo of pancreatic NETs, especially islet cells

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23
Q

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

A

F: they look the same

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24
Q

What are the most common sites of metastases for pancreatic NETs

A

liver > retroperitoneal LN > bone

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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

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