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Endocrinology > Paraneoplastic Syndrome, GN Neoplasms, and MEN > Flashcards

Paraneoplastic Syndrome, GN Neoplasms, and MEN Flashcards

recognize these at part of a differential

1
Q

65% of gastroenteropancreatic neuroendocrine neoplasms are _____ or _____ at time of diagnosis

A

unresectable
metastatic

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2
Q

which genetic disorder are gastroenteropancreatic neuroendocrine neoplasms associated with?

A

MEN 1

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3
Q

what do B cells secrete?

A

insulin

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4
Q

what do D cells secrete? what effect does it have?

A

somatostatin

decreases rate of gastric emptying, inhibits release of glucagon and GH

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5
Q

what do G cells secrete?

A

gastrin

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6
Q

what are B cell adenomas also called?

A

insulinomas

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7
Q

continued insulin secretion, despite hypoglycemic state, and is benign in 90% of cases

A

B cell adenoma

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8
Q

what are the lab results in B cell adenomas?
glucose, insulin, C peptide

A

plasma glucose <45
serum insulin > 6
elevated C peptide

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9
Q

what should we rule out in a case of B cell adenoma?

A

medication cause with sulfonylureas

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10
Q

what diagnostic can we get done for a B cell adenoma?

A

FNA

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11
Q

what is the treatment of choice for a B cell adenoma?

A

surgical resection

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12
Q

excessive gastric causing the stomach to secrete excessive acid

A

G cell gastrinoma

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13
Q

severe acid-related peptic disease and diarrhea associated with G cell gastrinomas

A

Zollinger-Ellison syndrome

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14
Q

what is rare, but one of the more common functional pancreatic neuroendocrine tumors?

A

G cell gastrinoma

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15
Q

what percentage of G cell gastrinomas are malignant?

A

50%

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16
Q

a G cell gastrinoma can be in the _____ or _____

A

pancreas
duodenum

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17
Q

in which patients should we consider a G cell gastrinoma?

A

patients with history of peptic ulcers

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18
Q

which condition are G cell gastrinomas associated with?

A

MEN 1

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19
Q

secrete glucagon, usually malignant with metastases, and cause weight loss, diarrhea, nausea, and DM

A

A cell glucagonomas

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20
Q

do not produce hormones, but grow large prior to detection; causing symptoms due to mass effect

A

nonfunctional tumors

21
Q

what is the treatment for a G cell gastrinoma?

A

surgical resection

22
Q

why is genetic testing in MEN recommended?

A

it is inherited as autosomal dominant

23
Q

causes predisposition to the development of tumors in at least 2 endocrine glands

A

multiple endocrine neoplasia (MEN)

24
Q

what is the life expectancy of MEN 1?

A

less than 55 yo

25
Q

what tumors are common in MEN 1? (3)

A

parathyroid
pancreatic
pituitary

26
Q

what tumor is common in MEN 2A?

A

medullary thyroid carcinoma

27
Q

what tumors are common in MEN 2B? (2)

A

medullary thyroid carcinoma
pheochromocytoma

28
Q

what is MEN 1 also known as?

A

Wermer Syndrome

29
Q

a patient presents with hypercalcemia and hyperparathyroidism. what could they be experiencing?

A

MEN 1
Wermer Syndrome

30
Q

patients with MEN 1 (Werner Syndrome) can present with what 2 other tumors?

A

enteropancreatic tumor
gastrinoma

31
Q

if a patient has MEN 1 (Werner Syndrome) + pituitary adenoma, they could have an elevated _______

A

serum prolactin

32
Q

what 3 types of non-endocrine tumors can be present in a patient with MEN 1 (Werner Syndrome)?

A

facial angiofibroma
lipoma
collagenoma

33
Q

what is MEN S2 also known as?

A

Sipple Syndrome

34
Q

what becomes a significant possibility and concern in patients with MEN 2A?

A

medullary thyroid cancer

35
Q

what is our recommended prophylactic treatment for MEN 2A?

A

total thyroidectomy by 6 yo in children with gene mutation

36
Q

what 4 conditions are common in MEN 2B?

A

pheochromocytoma
medullary thyroid cancer
mucosal neuroma
marfan like habitus

37
Q

what is our recommended prophylactic treatment for MEN 2B?

A

total thyroidectomy by 6 months in babies with gene mutation

38
Q

symptoms and physical findings due to the effects of a neoplasm that is mediated by hormones and cytokines either secreted by the tumor or an immune response from the body to the tumor

A

paraneoplastic syndrome

39
Q

what is the common initial presentation of paraneoplastic syndrome?

A

fever

40
Q

in which cancers is paraneoplastic syndrome most common in? (4)

A

lung
breast
ovarian
lymphatic (hodgkins/non-hodgkin)

41
Q

what is the most common cause of SIADH in paraneoplastic syndromes? why?

A

small cell lung cancer
secretes ADH

42
Q

what does SIADH in paraneoplastic syndromes lead to?

A

hyponatremia

43
Q

what is the most common cause of hypercalcemia in paraneoplastic syndromes? why?

A

bony metastasis from lung cancer
increased osteoclast bone resorption put into blood

44
Q

what is a DDx for hypercalcemia in paraneoplastic syndromes?

A

hyperparathyroidism

45
Q

what are 2 causes of hypoglycemia in paraneoplastic syndromes?

A

insulinoma
hepatocellular carcinoma

46
Q

why does hepatocellular carcinoma cause hypoglycemia in paraneoplastic syndromes?

A

damaged liver cannot meet body’s glucose demand

47
Q

what are 2 causes of cushing syndrome in paraneoplastic syndromes?

A

small cell lung cancer
pancreatic cancer

48
Q

SCLC with associated cushing syndrome has a _____ prognosis than patients without cushing syndrome

A

worse

Endocrinology (17 decks)

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