Neuroendocrine (O'Dorisio)

Question 1

this is the most important peptide in the system of the disseminated neuroendocrine cells and their tumors

Answer 1

somatostatin

Question 2

what are the top three most common tumors of the neuroendocrine system?

Answer 2

1. small bowel carcinoid 2. bronchial carcinoid 3. pancreatic neuroendocrine tumors

Question 3

_____ is a protein that serves as an excellent marker for tumor cell growth, and can be detected by a special staining known as _______

Answer 3

Ki67; MIB-1

Question 4

what is one of the hallmark clinical signs of patients with MEN-1, a subset of a group of disorders that involves tumors in at least two endocrine glands?

Answer 4

MEN-1 most often first involves the parathyroid gland (90%), so these patients present with hyperparathyroidism or hypercalcemia; may then progress to the pituitary (25% of cases) and/or the pancreas (66%).

Question 5

Which subtype of multiple endocrine neoplasia (MEN) is found in ~10% of sporadic hyperPTH cases and 25% of gastrinomas?

Answer 5

MEN-1

Question 6

what type of tumor is often associated with MEN-1 that affects the pituitary?

Answer 6

prolactinoma

Question 7

most common functioning tumor of MEN-1 and presents with severe peptic ulcer disease and Zollinger-Ellison syndrome

Answer 7

gastrinoma (in the pancreas or duodenum); also presents with gastric ph 1000

*this is the most common functioning tumor, but non-functioning tumors are by far the most common tumor types associated with MEN-1 (40%)

Question 8

MEN-1 is found in ~10% of these tumors which are generally benign and may be implicated by the presence of Whipple’s triad: 1) symptoms of hypogycemia and 2) low plasma concentration of glc, which is 3) relieved with glc administration

Answer 8

insulinoma

Question 9

patients with this type of tumor seen in MEN-1 often present with dermatitis, diabetes, weight loss and glossitis

Answer 9

glucagonoma

Question 10

a mutation in this tumor suppressor gene is what accounts for 75% of MEN-1 cases

Answer 10

menin

Question 11

Which of the following statements regarding MEN is FALSE?

A. 75% of MEN-1 results from a mutation in a tumor suppressor gene called menin

B. MEN-1 characteristically presents as tumors in the pituitary, parathyroid, and pancreas

C. Most MEN-2 are classified as MEN-2A (90%) though symptoms often do not develop before the age of 70

D. MEN-2B is associated with poor prognosis and routinely involves the parathyroid

E. Classical presentation in MEN2 is often asymptomatic, though the patient may have flushing or fat malabsorptive diarrhea

Answer 11

D. While MEN-2B is associated with poor prognosis, there is NO parathyroid involvement in this subtype. It routinely affects the thyroid.

Question 12

What is the screening test for MEN2?

Answer 12

calcitonin and CEA

Question 13

Tumors in these 3 locations are most commonly associated with MEN-1

Answer 13

THE 3 Ps of MEN-1: REMEMBER THIS! Parathyroid, pituitary, and pancreas

Question 14

MEN2 tumors are associated with marfanoid habitus and mutations in what gene?

Answer 14

RET gene

Question 15

epinephrine-secreting tumor that is characteristic of MEN2

Answer 15

pheochromocytoma

Question 16

a form of thyroid carcinoma which originates from the parafollicular cells (C cells), which produce the hormone calcitonin, and is seen in MEN2 along with pheochromocytoma

Answer 16

medullary thyroid carcinoma