Endo - Pathology (Malignancy)

Question 1

Is carcinoid syndrome common or rare? What causes/defines it, and what is secreted in it?

Answer 1

Rare syndrome caused by carcinoid tumors (neuroendocrine cells), especially metastatic small bowel tumors, which secrete high levels of serotonin (5-HT)

Question 2

In what setting is carcinoid syndrome not seen, and why?

Answer 2

Not seen if tumor is limited to GI tract (5-HT undergoes first-pass metabolism in liver)

Question 3

What are 4 symptoms/conditions that result from carcinoid syndrome?

Answer 3

Results in (1) recurrent diarrhea, (2) cutaneous flushing, (3) asthmatic wheezing, and (4) right-sided valvular disease.

Question 4

What are 2 lab findings associated with carcinoid syndrome?

Answer 4

(1) High 5-hydroxyindoleacetic (5-HIAA) in urine (2) Niacin deficiency (pellagra)

Question 5

What are 2 treatments for carcinoid syndrome?

Answer 5

Treatment: resection, somatostatin analog (e.g., octreotide)

Question 6

What is the rule associated with carcinoid syndrome?

Answer 6

Rule of 1/3’s: 1/3 metastasize, 1/3 present with 2nd malignancy, 1/3 are multiple

Question 7

What is the most common malignancy in the small intestine?

Answer 7

Carcinoid (tumor) syndrome

Question 8

What is pathophysiology of Zollinger-Ellison syndrome? Where does it arise versus damage?

Answer 8

Gastrin-secreting tumor of pancreas or duodenum. Acid hypersecretion causes recurrent ulcers in distal duodenum and jejunum.

Question 9

What are 2 symptoms with which Zollinger-Ellison syndrome presents?

Answer 9

Presents with (1) abdominal pain (peptic ulcer disease, distal ulcers), (2) Diarrhea (malabsorption).

Question 10

With what condition may Zolinger-Ellison syndrome be associated?

Answer 10

May be associated with MEN 1

Question 11

What does MEN stand for? What are the types of MEN?

Answer 11

Multiple endocrine neoplasias; (1) MEN 1 (Wermer syndrome) (2) MEN 2A (Sipple syndrome) (3) MEN 2B

Question 12

What is another name for MEN 1? What 3 kinds of tumors characterize it?

Answer 12

MEN 1 (Wermer syndrome); (1) Parathyroid tumors (2) Pituitary tumors (prolactin or GH) (3) Pancreatic endocrine tumors - Zollinger-Ellison syndrome, Insulinomas, VIPomas, Glucagonomas (rare)

Question 13

What are 4 examples of pancreatic endocrine tumors that may characterize MEN 1? Which of these is particularly rare?

Answer 13

Pancreatic endocrine tumors - Zollinger-Ellison syndrome, Insulinomas, VIPomas, Glucagonomas (rare)

Question 14

Besides tumors, what are 2 additional conditions/symptoms with which MEN 1 commonly presents?

Answer 14

Commonly presents with kidney stones and stomach ulcers

Question 15

What is another name for MEN 2A? What are 3 tumors associated with it?

Answer 15

MEN 2A (Sipple syndrome); (1) Medullary thyroid carcinoma (secretes calcitonin) (2) Pheochromocytoma (3) Parathyroid hyperplasia

Question 16

What are 3 tumors associated with MEN 2B?

Answer 16

(1) Medullary thyroid carcinoma (secretes calcitonin) (2) Pheochromocytoma (3) Oral/intestinal ganglioneuromatosis (mucosal neuromas)

Question 17

Besides tumors, with what condition is MEN 2B associated?

Answer 17

Associated with marfanoid habitus

Question 18

What does medullary thyroid carcinoma secrete? With which type(s) of MEN is it associated?

Answer 18

Medullary thyroid carcinoma (secretes calcitonin); MEN 2A (Sipple syndrome) & 2B

Question 19

What are the 2 kinds of pituitary tumors associated with a MEN? Which MEN is it?

Answer 19

Pituitary tumors (prolactin or GH); MEN 1 (Wermer syndrome)

Question 20

What is the learning/memorization method for distinguishing the characteristic tumors that define each type of MEN?

Answer 20

(1) MEN 1 = 3 P’s (from cepahlad to caudad: Pituitary, Parathyroid, and Pancreas); remember by drawing a diamond (2) MEN 2A = 2 P’s (Parathyroids & Pheochromocytoma); remember by drawing a square (3) MEN 2B = 1 P (Pheochromocytoma); remember by drawing a triangle; See p. 329 in First Aid 2014 at bottom of page for visuals

Question 21

What is the mode of inheritance for the MEN syndromes?

Answer 21

All MEN syndromes are autosomal dominant inheritance; Think: “All MEN are DOMINANT (or so they think)”

Question 22

What is the gene mutation associated with 2 of the MEN syndromes? Which 2 are these?

Answer 22

Associated with ret gene mutation in 2A and 2B