Peutz-Jeghers Syndrome - JMI Articles

Articles from our collection about Peutz-Jeghers Syndrome published during the 1980s

Title: Peutz-Jeghers syndrome: a clinicopathologic survey of the 'Harrisburg family' with a 49-year follow-up

Authors: Foley TR;McGarrity TJ;Abt AB;

Journal: Gastroenterology Date: 1988 Dec
Volume: 95 Issue: 6
Pages: 1535 - 1540

Abstract: Of the original Peutz-Jeghers families reported by Jeghers, the 'Harrisburg Family' has now been followed for 49 yr. Their 12 affected family members comprise the largest Peutz-Jeghers kindred reported. The course of this family illustrates that Peutz-Jeghers syndrome is not a benign disease. One family member developed a duodenal carcinoma in a hamartoma with adenomatous changes; this progression in the duodenum has not previously been reported. Ten patients underwent 75 polypectomies. One patient developed short bowel syndrome. Three patients died in young adulthood. The development of gastrointestinal malignancy in 2 of 12 affected patients suggests that Peutz-Jeghers syndrome may be a premalignant condition. Consequently, even asymptomatic gastric, duodenal, and colonic polyps should be removed endoscopically. If surgical intervention is necessary, intraoperative endoscopy with polypectomy may prevent the development of a short bowel syndrome. Colonoscopic screening of patients and their family members may be beneficial and surveillance for extraintestinal malignancy appears to be warranted

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Title: [The men behind the syndrome: Johannes Peutz and Harold Jeghers. The explorers of an underdiagnosed condition with intestinal polyps and mucocutaneous pigmentations]

Authors: Alm T;

Journal: Lakartidningen Date: 1987 Jul 8
Volume: 84 Issue: 28-29
Pages: 2351 - 2353

Abstract:

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Title: Localization of Peutz-Jeghers macules to psoriatic plaques

Authors: Banse-Kupin LA;Douglass MC;

Journal: Arch Dermatol Date: 1986 Jun
Volume: 122 Issue: 6
Pages: 679 - 683

Abstract: We report a case of the Peutz-Jeghers syndrome in which the characteristic pigmented macules developed within preexisting psoriatic plaques in sites extremely unusual for the syndrome. To our knowledge, ours is the first report of such a case in the English literature

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Title: Epithelioid leiomyosarcoma originating in a hamartomatous polyp from a patient with Peutz-Jeghers syndrome

Authors: Patterson MJ;Kernen JA;

Journal: Gastroenterology Date: 1985 Apr
Volume: 88 Issue: 4
Pages: 1060 - 1064

Abstract: A case of Peutz-Jeghers syndrome with long history and multiple resections is presented. Despite the large number of intestinal polyps in these patients, malignant transformations are rare. In this case, an epithelioid leiomyosarcoma developed within a single polyp. At the time of resection this tumor had already metastasized to the liver. The histology and ultrastructure of this tumor is presented. Reviews of the clinical syndrome and leiomyosarcoma are given, with discussion of the possible interaction of these two processes as presented in this patient

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Title: Does Peutz-Jeghers syndrome predispose to gastrointestinal malignancy? A later look

Authors: Linos DA;Dozois RR;Dahlin DC;Bartholomew LG;

Journal: Arch Surg Date: 1981 Sep
Volume: 116 Issue: 9
Pages: 1182 - 1184

Abstract: Several reports have recently suggested an association between the development of intestinal carcinoma and the presence of Peutz-Jeghers syndrome. During the 45-year period between 1935 and 1979, 48 patients with Peutz-Jeghers syndrome were seen at the Mayo Clinic, Rochester, Minn, and followed up for a median period of 33 years. Carcinoma of the small or large intestine did not develop in any patient, with possibly one exception. Survival of patients with Peutz-Jeghers syndrome also was found to be similar to that of the general population. Therefore, skepticism continues about the premalignant potential of this rare syndrome, especially in the small intestine, and surgical conservatism should be practiced in its management

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Title: Peutz-Jeghers syndrome

Authors: Griffith CD;Bisset WH;

Journal: Arch Dis Child Date: 1980 Nov
Volume: 55 Issue: 11
Pages: 866 - 869

Abstract:

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Title: Gall-bladder polyps in Peutz-Jeghers syndrome

Authors: Foster DR;Foster DB;

Journal: Postgrad Med J Date: 1980 May
Volume: 56 Issue: 655
Pages: 373 - 376

Abstract: A case of Peutz-Jeghers syndrome with polyps in the gall bladder is presented. No previous description of this finding has been reported. The literature regarding known sites of polyposis in this condition is reviewed

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