Cancer and the Peutz-Jeghers syndrome. Issue 11 (November 1989)

• Record Type: Journal Article
• Authors: Spigelman, A D; Murday, V; Phillips, R K
• Is Part Of: Gut. Volume 30:Issue 11(1989)
• Page Start: 1588
• Page End: 1590
• Publication Date: 1989-11
• Subjects: Gastroenterology -- Periodicals; 616.33
• DOI: 10.1136/gut.30.11.1588
• Languages: English
• ISSNs: 0017-5749

Abstract

Abstract : 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.