What is Peutz Jeghers syndrome?
inactivating mutation of LKB1
-intestinal hamartomatous polyps and freckle like macular lesions which fade at puberty
Which cancers does Peutz jeghers syndrome predispose someone to?
breast, pancreas, testis, ovary
Where in the small intestine would adenocarcinomas occur?
duodenum and jejunum
What diseases inc risk for adenocarcinoma of the small intestine?
- crohn disease
- FAP
- HNPCC
- Lynch syndrome
- Celiac disease
Compare and contrast mediterannean vs western type intestinal lymphoma?
-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
Where is the most common location of a carcinoid tumor?
appendix
Where is the location that a carcinoid tumor is known to be most aggressive?
ileum
When would carcinoid syndrome be present with a carcinoid tumor?
ONLY when metastasis present in the liver
-otherwise 5H1AA is not secreted
(liver has MAO to breakdown serotonin)
What are the symptoms of carcinoid syndrome?
bronchospasm, diarrhea, flushing of the skin
Right sided valvular fibrosis of the heart
-tricuspid regurg and pulm valve stenosis
What is the most common neoplasm of the small intestine?
metastatic tumors
-lung, female genital organs, melanomas
