RT Journal Article
SR Electronic
T1 Cancer and the Peutz-Jeghers syndrome.
JF Gut
JO Gut
FD BMJ Publishing Group Ltd and British Society of Gastroenterology
SP 1588
OP 1590
DO 10.1136/gut.30.11.1588
VO 30
IS 11
A1 A D Spigelman
A1 V Murday
A1 R K Phillips
YR 1989
UL http://gut.bmj.com/content/30/11/1588.abstract
AB Among 72 patients with the Peutz-Jeghers syndrome malignant tumours have developed in 16 (22%) of whom all but one have died. There were nine gastrointestinal and seven nongastrointestinal tumours. The relative risks of death from gastrointestinal cancer and all cancers were 13 (95% CI 2.7-38.1) and 9 (95% CI 4.2-17.3) respectively. The chance of dying of cancer by the age of 57 was 48%. There is evidence for a hamartoma/carcinoma sequence in the Peutz-Jeghers syndrome, suggesting that the gene locus involved is relevant to the development of malignancy in general.