Cancer and the Peutz-Jeghers syndrome.
Open Access
- 1 November 1989
- Vol. 30 (11), 1588-1590
- https://doi.org/10.1136/gut.30.11.1588
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.Keywords
This publication has 17 references indexed in Scilit:
- Epithelial Misplacement in Peutz-Jeghers PolypsThe American Journal of Surgical Pathology, 1987
- Increased Risk of Cancer in the Peutz–Jeghers SyndromeNew England Journal of Medicine, 1987
- Mixed familial polyposis syndromesInternational Journal of Colorectal Disease, 1987
- Peutz-Jeghers syndrome and metastasising colonic adenocarcinoma.Gut, 1984
- A distinctive ovarian sex cord-stromal tumor causing sexual precocity in the Peutz-Jeghers syndrome*The American Journal of Surgical Pathology, 1983
- Feminizing sex cord tumor with annular tubules in a boy with Peutz-Jeghers syndromeThe Journal of Pediatrics, 1982
- Long term survival analysis: The curability of breast cancerStatistics in Medicine, 1982
- Intestinal Carcinoma in the Peutz-Jeghers SyndromeJAMA, 1974
- Maximum utilization of the life table method in analyzing survivalJournal of Chronic Diseases, 1958
- Generalized Intestinal Polyposis and Melanin Spots of the Oral Mucosa, Lips and DigitsNew England Journal of Medicine, 1949