Cancer and the Peutz-Jeghers syndrome.
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.
Spigelman
A D
AD
St Mark's Hospital, London.
Murday
V
V
Phillips
R K
RK
D000328:Adult; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000401:mortality; D006801:Humans; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000401:mortality; D010580:Peutz-Jeghers Syndrome / Q000235:genetics / Q000401:mortality; D012306:Risk