JOURNAL OF KAOHSIUNG MEDICAL ASSOCIATION 2013. vol.21, No.

Peutz-Jeghers

20

XX20
4

6
4

(6/4)

(caf-au-lait pigmentation spots,

)6

Peutz-Jeghers
Peutz
Jeghers (Peutz-Jeghers syndrome)
(hemoglobin)9.3 g/dL

(hematocrit) 29.9%

(hamartomatous
polyps)

Peutz-Jeghers

Peutz-Jeghers

Peutz ( c a f - a u - l a i t
Jeghers
pigmentation spots)

152

Peutz-Jeghers

(arborizing muscular bundles)

(A)
(B)
(C)228
T2N2bM0
UICC

(FOLFOX4
regimen)

(carcinoembryonic antigen)7.27 ng/

mL6

()

Peutz-Jeghers

Peutz-Jeghers

(hamartomatous

153

 JOURNAL OF KAOHSIUNG MEDICAL ASSOCIATION 2013. vol.21, No.2

polyps)

(
78%)(38%)
(42%)(28%)

(adenoma)
1-5

30PeutzJeghers

19q13.3serine
threonine kinase 11
(germline mutation)50-70%

Peutz-Jeghers

(918)

70
87%15%

Peutz-Jeghers

Peutz-Jeghers

1. Riegert-Johnson DL, Westra W, Roberts M:

High cancer risk and increased mortality in
patients with Peutz-Jeghers syndrome. Gut
2012; 61:322-323.
2. Lim W, Olschwang S, Keller JJ et al.:
Relative frequency and morphology of
cancers in STK 11 mutation carriers.
Gastroenterology 2004; 126:1788-1794.
3. Giardiello FM, Brensinger JD, Tersmette
AC et al. : Very high risk of cancer
in familial Peutz-Jeghers syndrome.
Gastroenterology 2000; 119:1447-1453.
4. Derici H, Peker Y, Tatar F et al. : Multiple
malignant gastrointestinal polyps and rectal
carcinoma in a young patients with PeutzJeghers syndrome. Int J Colorectal Dis
2007; 22:85-86.
5. Hearle N, Schumacher V, Menko FH et al.:
Frequency and spectrum of cancers in the
Peutz-Jeghers syndrome. Clin Cancer Res

154

Peutz-Jeghers

(arborizing muscular bundles) (A, )

(B, )
(C)

2006; 12:3209-3215.
6. You YN, Wolff BG, Boardman LA et al. :
Peutz-Jeghers syndrome: a study of longterm surgical morbidity and causes of
mortality. Familial Cancer 2010; 9:609-616.
7. Dunlop MG: Guidance on gastrointestinal
surveillance for hereditary non-polyposis
colorectal cancer, familial adenomatous
polypolis, juvenile polyposis, and PeutzJeghers syndrome. Gut 2002; 51 Supple
5:V21-7.

155