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Peutz-Jeghers Syndrome - JMI ArticlesArticles from our collection about Peutz-Jeghers Syndrome published during the 2000sTitle: Skin manifestations of internal malignancy Authors: Braverman IM; Journal: Clin Geriatr Med Date: 2002 Feb Pages: 1 - 19 Abstract: This article concentrates on the major signs and syndromes that are associated with internal malignancies in the geriatric population. Included are cutaneous metastases, ectopic adrenocorticotropic hormone-producing syndromes, and disorders arising from APUD cell tumors. The major paraneoplastic disorders of dermatomyositis, generalized pruritus, Bazex's syndrome, and acanthosis nigricans also are discussed. Also included are Bowen's disease of skin; arsenical toxicity; and the Peutz-Jeghers', Gardner's, and Torre's syndromes, which are indicative of systemic or organ-related carcinogens Link: Click for Pubmed record Title: Skin spots and heart tumors Authors: Reddy TD;Eccles DM;Theaker J;Keeton BR;Temple K; Journal: J Pediatr Date: 2001 Dec Pages: 901 - 902 Abstract: Link: Click for Pubmed record Title: Solitary hamartomatous polyp of Peutz-Jeghers type in the jejunum resected endoscopically Authors: Sone Y;Nakano S;Takeda I;Kumada T;Kiriyama S;Hisanaga Y; Journal: Gastrointest Endosc Date: 2000 May Pages: 620 - 622 Abstract: Link: Click for Pubmed record Title: Peutz-Jeghers syndrome Authors: McGarrity TJ;Kulin HE;Zaino RJ; Journal: Am J Gastroenterol Date: 2000 Mar Pages: 596 - 604 Abstract: Peutz-Jeghers syndrome (PJS) is an unusual polyposis syndrome that has enjoyed a rich and somewhat confusing history. Mucocutaneous pigmentation and diffuse gastrointestinal hamartomas are the hallmark features of this autosomal dominant inherited condition. Peutz-Jeghers syndrome is now also recognized as a cancer predisposition syndrome. In this review, we highlight the historical aspects of PJS polyposis with special emphasis on its extraintestinal manifestations, particularly genital tract tumors. A PJS management scheme for clinicians is included Link: Click for Pubmed record Title: Small bowel polyps in Peutz-Jeghers syndrome: management by combined push enteroscopy and intraoperative enteroscopy Authors: Pennazio M;Rossini FP; Journal: Gastrointest Endosc Date: 2000 Mar Pages: 304 - 308 Abstract: BACKGROUND: Polyps occur throughout the GI tract in Peutz-Jeghers syndrome; the major problem in the management of the syndrome lies in the small bowel. METHODS: From January 1979 to January 1998, seven patients with Peutz-Jeghers syndrome underwent surveillance. Between 1979 and 1992 they were managed with upper and lower endoscopy every 2 to 3 years and surgery when intestinal obstruction occurred. From 1993 they also underwent enteroclysis and, on the basis of radiologic findings, push enteroscopy and/or intraoperative enteroscopy. Push enteroscopy was then performed every 2 years in all patients. RESULTS: During the first period, 5 of 7 patients underwent emergency small bowel resection (2 operated twice). The patients were divided into 2 groups based on enteroclysis findings; the first comprised 4 patients with multiple polyps throughout the small bowel, and the second included 3 patients with polyps only in the proximal small bowel. Three of the 4 patients with diffuse polyposis underwent intraoperative enteroscopy during which on average 16 polyps per patient were removed (range 10 to 25 polyps; mean diameter 16 mm, range 3 to 50 mm). The remaining patient with diffuse polyposis had a single 25 mm polyp in the terminal ileum removed by retrograde ileoscopy; the more proximal polyps were removed by push enteroscopy. The patients with diffuse polyposis remained asymptomatic during follow-up (mean 50 months, range 47 to 57 months) and also underwent periodic push enteroscopy (mean 2.25 enteroscopies per patient, range 2 to 3) at which a mean of 8.5 polyps per patient (range 4 to 13 polyps) were removed (mean diameter 7.2 mm, range 3 to 15 mm). The 3 patients of the second group underwent periodic push enteroscopy alone (mean 3 per patient) during which a mean of 11.7 polyps per patient were removed (range 7 to 15 polyps: mean diameter 10.9 mm, range 3 to 40 mm). Enteroclysis was not repeated in these patients, who remained asymptomatic during follow-up (mean 47 months, range 46 to 48 months). CONCLUSIONS: More effective clearance of small bowel polyps via enteroscopy will help reduce the need for emergency surgery with extensive intestinal resection in patients with Peutz-Jeghers syndrome Link: Click for Pubmed record Title: Gastric polyps: an update of their pathology and biological significance Authors: Oberhuber G;Stolte M; Journal: Virchows Arch Date: 2000 Dec Pages: 581 - 590 Abstract: Gastric polyps are clinically important lesions that are frequently encountered in routine pathology (2-3% of all gastroscopies). Polyps may occur sporadically or in polyposis syndromes, such as familial adenomatous polyposis coli (FAP), Peutz-Jeghers syndrome, juvenile polyposis, Cowden's disease and Cronkhite-Canada syndrome. In biopsy specimens taken during routine gastroscopic examinations, it is almost always possible to differentiate between neoplastic and non-neoplastic polyps and to type polyps. In this review, we focus on the morphological spectrum of gastric polyps in an attempt to assist the pathologist and the gastroenterologist in recognising the lesion and in treating patients with gastric polyps, respectively. Further, we propose that the World Health Organization (WHO) classification should be modified to include the following categories: non-neoplastic polyps (WHO: tumour-like lesion), hamartomatous polyps/polyps of polyposis syndromes (WHO: tumour-like lesion), heterotopic tissue polyps (WHO: tumour-like lesion), neoplastic polyps (WHO: epithelial, non-epithelial and endocrine tumours) and reactive polypoid lesions Link: Click for Pubmed record Title: Hereditary colorectal cancer: risk assessment and management Authors: Hampel H;Peltomaki P; Journal: Clin Genet Date: 2000 Aug Pages: 89 - 97 Abstract: There are at least nine major cancer susceptibility syndromes that infer an increased risk for colorectal cancer and/or colorectal polyposis; hereditary nonpolyposis colorectal cancer syndrome, Muir-Torre syndrome, Turcot syndrome, the I1307K polymorphism of the APC gene, familial adenomatous polyposis, attenuated familial adenomatous polyposis, Peutz Jeghers syndrome, juvenile polyposis, and the PTEN hamartoma tumor syndrome. As a result, the differential diagnosis of hereditary colorectal cancer can be complex. In addition, there has been a dramatic increase in the knowledge available regarding risk assessment and management of hereditary colorectal cancer syndromes. The literature was reviewed to develop this concise review of the hereditary colorectal cancer syndromes to facilitate the accurate diagnosis of each syndrome and the appropriate medical care for individuals with these diagnoses. Referral to a qualified Clinical Cancer Genetics program is appropriate if any of these syndromes is suspected and they will ensure the most up-to-date information is available to the patient, their family, and their health care professionals Link: Click for Pubmed record Title: Hereditary intestinal cancer Authors: Aaltonen LA; Journal: Semin Cancer Biol Date: 2000 Aug Pages: 289 - 298 Abstract: Mechanisms involved in hereditary intestinal cancer are likely to play a role in sporadic tumorigenesis as well. Studies focusing on the molecular biology underlying these syndromes has contributed considerably to our knowledge on molecular bases of malignant transformation. It can be concluded, that there are two aspects to the importance of studies on intestinal cancer predisposition. First, the families suffering from cancer proneness need help which can, to some extent, be provided through molecular genetic studies. Second, the resources appointed to such research have produced scientific advances with outstanding importance to our understanding of common malignant diseases Link: Click for Pubmed record |
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Jeghers Medical Index, St. Elizabeth Health Center, 1044 Belmont Ave., Youngstown, OH 44501
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