Intestinal Neoplasmas

Question 1

What is Peutz Jeghers syndrome?

Answer 1

inactivating mutation of LKB1

-intestinal hamartomatous polyps and freckle like macular lesions which fade at puberty

Question 2

Which cancers does Peutz jeghers syndrome predispose someone to?

Answer 2

breast, pancreas, testis, ovary

Question 3

Where in the small intestine would adenocarcinomas occur?

Answer 3

duodenum and jejunum

Question 4

What diseases inc risk for adenocarcinoma of the small intestine?

Answer 4

• crohn disease
• FAP
• HNPCC
• Lynch syndrome
• Celiac disease

Question 5

Compare and contrast mediterannean vs western type intestinal lymphoma?

Answer 5

-both originate in nodules of MALT

Mediterranean:

• poor countries in young men
• duodenum and proximal jejunum

Western

• adults older than 40, kids <10
• immunosuppression inc risk
• ileum

Question 6

Where is the most common location of a carcinoid tumor?

Answer 6

appendix

Question 7

Where is the location that a carcinoid tumor is known to be most aggressive?

Answer 7

ileum

Question 8

When would carcinoid syndrome be present with a carcinoid tumor?

Answer 8

ONLY when metastasis present in the liver
-otherwise 5H1AA is not secreted
(liver has MAO to breakdown serotonin)

Question 9

What are the symptoms of carcinoid syndrome?

Answer 9

bronchospasm, diarrhea, flushing of the skin
Right sided valvular fibrosis of the heart
-tricuspid regurg and pulm valve stenosis

Question 10

What is the most common neoplasm of the small intestine?

Answer 10

metastatic tumors

-lung, female genital organs, melanomas