Peutz-Jeghers Syndrome

Question 1

What is peutz-jeghers syndrome?

Answer 1

Freckle like lesions of the hands, periooral skin and oral mucosa

Question 2

What occurs in conjuction with the freckle-like lesions in peutz-jeghers syndrome?

Answer 2

Intestinal polyposis and predisposition for affected pateints to develop cancer

Question 3

Where does peutz-jeghers syndrome lesions occur most often?

Answer 3

labial mucosa and buccal mucosa

occurs occasionally on tongue and palate

Question 4

What part of intestine is most affected by polyps?

Answer 4

Jejunum and ileum

Question 5

What kind of cancer developers in a significant % of patients with peutz-jeghers syndrome?

Answer 5

GI adenocarcinoma

Question 6

GI polyps in peutz-jeghers syndrome is a benign overgrowth of what?

Answer 6

Benign overgrowth of intestinal glandular epithelium supported by a core of smooth muscle

Question 7

Tx for peutz-jeghers syndrome?

Answer 7

Should be monitored for development of intussusceptions or tumor formation

Genetic counseling