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. 1979 Feb;20(2):169–175. doi: 10.1136/gut.20.2.169

Peutz-Jeghers syndrome associated with gastrointestinal carcinoma. Report of two cases in a family.

B Cochet, J Carrel, L Desbaillets, S Widgren
PMCID: PMC1419453  PMID: 428830

Abstract

Patients with the Peutz-Jeghers syndrome carry a slight, though definite, increased risk of gastrointestinal carcinoma. The malignant potentiality of Peutz-Jeghers hamartomatous polyps, generally considered benign, is supported by this report. Two cases of metastasising gastrointestinal carcinoma associated with the Peutz-Jeghers syndrome are described in a 56 year old female and her 29 year old son. Both mother and son died from duodenal and gastric carcinomas respectively, which developed in hamartomatous polyps with extensive metastases. Both cases also showed dysplastic areas within hamartomatous polyps. These features indicate that hamartomatous polyps may, in some cases, be the precursors of digestive tract carcinomas.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. BAILEY D. Polyposis of gastro-intestinal tract: the Peutz syndrome. Br Med J. 1957 Aug 24;2(5042):433–439. doi: 10.1136/bmj.2.5042.433. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. BARTHOLOMEW L. G., DAHLIN D. C., WAUGH J. M. Intestinal polyposis associated with mucocutaneous melanin pigmentation Peutz-Jeghers syndrome; review of literature and report of six cases with special reference to pathologic findings. Gastroenterology. 1957 Mar;32(3):434–451. [PubMed] [Google Scholar]
  3. Bussey H. J. Gastrointestinal polyposis. Gut. 1970 Nov;11(11):970–978. doi: 10.1136/gut.11.11.970. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. DORMANDY T. L. Gastrointestinal polyposis with mucocutaneous pigmentation (Peutz-Jeghers syndrome). N Engl J Med. 1957 Jun 13;256(24):1141–contd. doi: 10.1056/NEJM195706132562405. [DOI] [PubMed] [Google Scholar]
  5. Dodds W. J., Schulte W. J., Hensley G. T., Hogan W. J. Peutz-Jeghers syndrome and gastrointestinal malignancy. Am J Roentgenol Radium Ther Nucl Med. 1972 Jun;115(2):374–377. doi: 10.2214/ajr.115.2.374. [DOI] [PubMed] [Google Scholar]
  6. Dozois R. R., Judd E. S., Dahlin D. C., Bartholomew L. G. The Peutz-Jeghers syndrome. Is there a predisposition to the development of intestinal malignancy? Arch Surg. 1969 Apr;98(4):509–517. doi: 10.1001/archsurg.1969.01340100125017. [DOI] [PubMed] [Google Scholar]
  7. Dozois R. R., Judd E. S., Dahlin D. C., Bartholomew L. G. The Peutz-Jeghers syndrome. Is there a predisposition to the development of intestinal malignancy? Arch Surg. 1969 Apr;98(4):509–517. doi: 10.1001/archsurg.1969.01340100125017. [DOI] [PubMed] [Google Scholar]
  8. HORN R. C., Jr, PAYNE W. A., FINE G. The Peutz-Jeghers syndrome. (Gastrointestinal polyposis with mucocutaneous pigmentation): report of a case terminating with disseminated gastrointestinal cancer. Arch Pathol. 1963 Jul;76:29–37. [PubMed] [Google Scholar]
  9. JEGHERS H., McKUSICK V. A., KATZ K. H. Generalized intestinal polyposis and melanin spots of the oral mucosa, lips and digits; a syndrome of diagnostic significance. N Engl J Med. 1949 Dec 29;241(26):1031–1036. doi: 10.1056/NEJM194912292412601. [DOI] [PubMed] [Google Scholar]
  10. MEGEVAND A. Polypose viscérale et pigmentation mélanique cutanéo-muqueuse (syndrome de Peutz). Arch Fr Pediatr. 1959;16(3):353–361. [PubMed] [Google Scholar]
  11. Payson B. A., Moumgis B. Metastasizing carcinoma of the stomach in Peutz-Jeghers syndrome. Ann Surg. 1967 Jan;165(1):145–151. doi: 10.1097/00000658-196701000-00021. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. RINTALA A. The histological appearance of gastrointestinal polyps in the Peutz-Jeghers syndrome. Acta Chir Scand. 1959 Sep 20;117:366–373. [PubMed] [Google Scholar]
  13. Reid J. D. Intestinal carcinoma in the Peutz-Jeghers syndrome. JAMA. 1974 Aug 12;229(7):833–834. [PubMed] [Google Scholar]
  14. Shibata H. R., Phillips M. J. Peutz-Jeghers syndrome with jejunal and colonic adenocarcinomas. Can Med Assoc J. 1970 Aug 1;103(3):285–287. [PMC free article] [PubMed] [Google Scholar]
  15. WILLIAMS J. P., KNUDSEN A. PEUTZ-JEGHERS SYNDROME WITH METASTASIZING DUODENAL CARCINOMA. Gut. 1965 Apr;6:179–184. doi: 10.1136/gut.6.2.179. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Warren K. W., Kune G. A., Poulantzas J. K. Peutzjeghers syndrome with carcinoma of the duodenum and jejunum. Lahey Clin Found Bull. 1965;14(3):97–102. [PubMed] [Google Scholar]
  17. Wennstrom J., Pierce E. R., McKusick V. A. Hereditary benign and malignant lesions of the large bowel. Cancer. 1974 Sep;34(3):suppl–suppl:857. doi: 10.1002/1097-0142(197409)34:3+<850::aid-cncr2820340711>3.0.co;2-s. [DOI] [PubMed] [Google Scholar]

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