Paraneoplastic Syndrome, GN Neoplasms, and MEN

Question 1

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

Answer 1

unresectable
metastatic

Question 2

which genetic disorder are gastroenteropancreatic neuroendocrine neoplasms associated with?

Answer 2

MEN 1

Question 3

what do B cells secrete?

Answer 3

insulin

Question 4

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

Answer 4

somatostatin

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

Question 5

what do G cells secrete?

Answer 5

gastrin

Question 6

what are B cell adenomas also called?

Answer 6

insulinomas

Question 7

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

Answer 7

B cell adenoma

Question 8

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

Answer 8

plasma glucose <45
serum insulin > 6
elevated C peptide

Question 9

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

Answer 9

medication cause with sulfonylureas

Question 10

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

Answer 10

FNA

Question 11

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

Answer 11

surgical resection

Question 12

excessive gastric causing the stomach to secrete excessive acid

Answer 12

G cell gastrinoma

Question 13

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

Answer 13

Zollinger-Ellison syndrome

Question 14

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

Answer 14

G cell gastrinoma

Question 15

what percentage of G cell gastrinomas are malignant?

Answer 15

50%

Question 16

a G cell gastrinoma can be in the _____ or _____

Answer 16

pancreas
duodenum

Question 17

in which patients should we consider a G cell gastrinoma?

Answer 17

patients with history of peptic ulcers

Question 18

which condition are G cell gastrinomas associated with?

Answer 18

MEN 1

Question 19

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

Answer 19

A cell glucagonomas

Question 20

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

Answer 20

nonfunctional tumors

Question 21

what is the treatment for a G cell gastrinoma?

Answer 21

surgical resection

Question 22

why is genetic testing in MEN recommended?

Answer 22

it is inherited as autosomal dominant

Question 23

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

Answer 23

multiple endocrine neoplasia (MEN)

Question 24

what is the life expectancy of MEN 1?

Answer 24

less than 55 yo

Question 25

what tumors are common in MEN 1? (3)

Answer 25

parathyroid pancreatic pituitary

Question 26

what tumor is common in MEN 2A?

Answer 26

medullary thyroid carcinoma

Question 27

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

Answer 27

medullary thyroid carcinoma pheochromocytoma

Question 28

what is MEN 1 also known as?

Answer 28

Wermer Syndrome

Question 29

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

Answer 29

MEN 1 Wermer Syndrome

Question 30

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

Answer 30

enteropancreatic tumor gastrinoma

Question 31

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

Answer 31

serum prolactin

Question 32

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

Answer 32

facial angiofibroma lipoma collagenoma

Question 33

what is MEN S2 also known as?

Answer 33

Sipple Syndrome

Question 34

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

Answer 34

medullary thyroid cancer

Question 35

what is our recommended prophylactic treatment for MEN 2A?

Answer 35

total thyroidectomy by 6 yo in children with gene mutation

Question 36

what 4 conditions are common in MEN 2B?

Answer 36

pheochromocytoma medullary thyroid cancer mucosal neuroma marfan like habitus

Question 37

what is our recommended prophylactic treatment for MEN 2B?

Answer 37

total thyroidectomy by 6 months in babies with gene mutation

Question 38

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

Answer 38

paraneoplastic syndrome

Question 39

what is the common initial presentation of paraneoplastic syndrome?

Answer 39

fever

Question 40

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

Answer 40

lung breast ovarian lymphatic (hodgkins/non-hodgkin)

Question 41

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

Answer 41

small cell lung cancer secretes ADH

Question 42

what does SIADH in paraneoplastic syndromes lead to?

Answer 42

hyponatremia

Question 43

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

Answer 43

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

Question 44

what is a DDx for hypercalcemia in paraneoplastic syndromes?

Answer 44

hyperparathyroidism

Question 45

what are 2 causes of hypoglycemia in paraneoplastic syndromes?

Answer 45

insulinoma hepatocellular carcinoma

Question 46

why does hepatocellular carcinoma cause hypoglycemia in paraneoplastic syndromes?

Answer 46

damaged liver cannot meet body's glucose demand

Question 47

what are 2 causes of cushing syndrome in paraneoplastic syndromes?

Answer 47

small cell lung cancer pancreatic cancer

Question 48

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

Answer 48

worse