Title: Peutz-Jeghers syndrome. A clinicopathologic study of a large family with a 27-year follow-up
Abstract: CancerVolume 50, Issue 10 p. 2139-2146 ArticleFree Access Peutz-Jeghers syndrome. A clinicopathologic study of a large family with a 27-year follow-up Daniel Burdick MD, Daniel Burdick MD Departments of Surgery, Syracuse, New YorkSearch for more papers by this authorJohn T. Prior MD, Corresponding Author John T. Prior MD Departments of Pathology, State University of New York, Upstate Medical Center, Syracuse, New YorkCommunity General Hospital, Broad Road, Syracuse, NY 13215===Search for more papers by this author Daniel Burdick MD, Daniel Burdick MD Departments of Surgery, Syracuse, New YorkSearch for more papers by this authorJohn T. Prior MD, Corresponding Author John T. Prior MD Departments of Pathology, State University of New York, Upstate Medical Center, Syracuse, New YorkCommunity General Hospital, Broad Road, Syracuse, NY 13215===Search for more papers by this author First published: 15 November 1982 https://doi.org/10.1002/1097-0142(19821115)50:10<2139::AID-CNCR2820501028>3.0.CO;2-KCitations: 68AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Abstract A family group of ten patients with the Peutz-Jeghers syndrome has been followed for a 27-year period. Eight members of the family had one or more manifestations of the syndrome. One member of the family died following a bypass procedure of a nonresectable carcinoma of the jejunum. Postmortem examination revealed this to be an adenocarcinoma of the jejunum arising in a Peutz-Jeghers polyp and metastases were present in the mesenteric lymph nodes. Two members of the family developed breast carcinomas, one arising in a fibroadenoma; both patients died, one of metastatic breast carcinoma, the other of a second primary malignancy (adenocarcinoma of the jejunum arising in a Peutz-Jeghers polyp). Three family members had benign ovarian tumors, one patient had a benign breast tumor and another patient had a benign colloid thyroid nodule. While the authors of this report believe that they have added a documented case of an adenocarcinoma of the jejunum arising in a Peutz-Jeghers polyp to the literature, the exact risk of intestinal cancer in the Peutz-Jeghers syndrome is unknown, but probably very small. Gastrointestinal surgery should continue to be performed in symptomatic patients and all patients should be followed closely at regular intervals. References 1 Burdick D, Prior JT, Scanlon GT. Peutz-Jeghers syndrome: A clinical-pathological study of a large family with a 10-year follow-up. Cancer 1963; 16: 854– 867. 2 Jancu J. Peutz-Jeghers syndrome: Involvement of gastrointestinal and upper respiratory tracts. Am J Gastroenterol 1971; 56: 545– 549. 3 Scully RE. Sex cord tumor with annular tubules: A distinctive ovarian tumor with Peutz-Jeghers syndrome. Cancer 1970; 25: 1107– 1121. 4 Williams JP, Knudsen A. Peutz-Jeghers syndrome with metastasizing duodenal carcinoma. Gut 1965; 6: 179– 184. 5 Gannon PG, Dablin DC, Bartholomew LG, Beahrs OH. Polypoid glandular tumors of the small intestine. Surg Gynecol Obstet 1962; 114: 666– 672. 6 Hart WR, Kumar N, Crissman, JD, Ovarian neoplasms resembling sex cord tumors with annular tubules. Cancer 1980; 45: 2352– 2363. 7 Cantu JM, Rivera H, Ocampo-Campos R et al. Peutz-Jeghers syndrome with feminizing Sertoli cell tumor. Cancer 1980; 46: 223– 228. 8 Buzanowski-Konakry K, Harrison EG Jr, Payne, WS. Lobular carcinoma arising in fibroadenoma of the breast. Cancer 1975; 35: 450– 456. 9 Riley E, Swift M. A family with Peutz-Jeghers syndrome and bilateral breast cancer. Cancer 1980; 46: 815– 817. 10 Sommerhaug RG, Mason T. Peutz-Jeghers syndrome and ureteral polyposis. JAMA 1970; 211: 120– 122. Citing Literature Volume50, Issue1015 November 1982Pages 2139-2146 ReferencesRelatedInformation