Peutz–Jeghers syndrome and screening for pancreatic cancer

Latchford, Andrew; Greenhalf, William; Vitone, Louis; Neoptolemos, John P.; Lancaster, Gillian; Phillips, R. K. S.

doi:10.1002/bjs.5609

Cited by 65 publications

(34 citation statements)

References 89 publications

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“…There is no consensus or organization-approved guidelines for cancer surveillance in PJS patients. Different protocols are used at Johns Hopkins Hospital [91] , St. Mark's Hospital [92] , the Mayo Clinic [93] , the University of Edinburgh [94] , Danish Polyposis Registry [95] , and the University of Newcastle (Australia) [96] . There are many differences between the protocols, but whichever protocol is used, it should be modified according to available resources, the individual patient's disease manifestations, psychosocial situation, and personal preferences.…”

Section: Screeningmentioning

confidence: 99%

Peutz-Jeghers syndrome: Diagnostic and therapeuticapproach

Kopáčová

Tachecí²,

Rejchrt³

et al. 2009

WJG

193

183

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Peutz-Jeghers syndrome (PJS) is an inherited, autosomal dominant disorder distinguished by hamartomatous polyps in the gastrointestinal tract and pigmented mucocutaneous lesions. Prevalence of PJS is estimated from 1 in 8300 to 1 in 280 000 individuals. PJS predisposes sufferers to various malignancies (gastrointestinal, pancreatic, lung, breast, uterine, ovarian and testicular tumors). Bleeding, obstruction and intussusception are common complications in patients with PJS. Double balloon enteroscopy (DBE) allows examination and treatment of the small bowel. Polypectomy using DBE may obviate the need for repeated urgent operations and small bowel resection that leads to short bowel syndrome. Prophylaxis and polypectomy of the entire small bowel is the gold standard in PJS patients. Intraoperative enteroscopy (IOE) was the only possibility for endoscopic treatment of patients with PJS before the DBE era. Both DBE and IOE facilitate exploration and treatment of the small intestine. DBE is less invasive and more convenient for the patient. Both procedures are generally safe and useful. An overall recommendation for PJS patients includes not only gastrointestinal multiple polyp resolution, but also regular lifelong cancer screening (colonoscopy, upper endoscopy, computed tomography, magnetic resonance imaging or ultrasound of the pancreas, chest X-ray, mammography and pelvic examination with ultrasound in women, and testicular examination in men). Although the incidence of PJS is low, it is important for clinicians to recognize these disorders to prevent morbidity and mortality in these patients, and to perform presymptomatic testing in the first-degree relatives of PJS patients.

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Section: Screeningmentioning

confidence: 99%

Peutz-Jeghers syndrome: Diagnostic and therapeuticapproach

Kopáčová

Tachecí²,

Rejchrt³

et al. 2009

WJG

193

183

View full text Add to dashboard Cite

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“…At the present time it is suggested that CE should be performed, instead of SBFT, at the time of diagnosis and, as a part of surveillance programs, every 2-3 years, but also as a first diagnostic step in patients with symptoms (i.e. abdominal pain or anaemia) [69,72] . Indeed, keeping in mind the limitations of capsule endoscopy in patients with polyposis syndromes, double balloon enteroscopy can become an important tool, to accurately size and locate lesions, but also to remove polyps identified by capsule endoscopy [73] .…”

Section: Other Indicationsmentioning

confidence: 99%

Small bowel capsule endoscopy in 2007: Indications, risks and limitations

Rondonotti¹

2007

WJG

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Capsule endoscopy has revoluzionized the study of the small bowel by providing a reliable method to evaluate, endoscopically, the entire small bowel. In the last six years several papers have been published exploring the possible role of this examination in different clinical conditions. At the present time capsule endoscopy is generally recommended as a third examination, after negative bidirectional endoscopy, in patients with obscure gastrointestinal bleeding. A growing body of evidence suggests also an important role for this examination in other clinical conditions such as Crohn's disease, celiac disease, small bowel polyposis syndromes or small bowel tumors. The main complication of this examination is the retention of the device at the site of a previously unknown small bowel stricture. However there are also some other open issues mainly due to technical limitations of this tool (which is not driven from remote control, is unable to take biopsies, to insufflate air, to suck fluids or debris and sometimes to correctly size and locate lesions).The recently developed double balloon enteroscope, owing to its capability to explore a large part of the small bowel and to take targeted biopsies, although being invasive and time consuming, can overcome some limitations of capsule endoscopy. At the present time, in the majority of clinical conditions (i.e. obscure GI bleeding), the winning strategy seems to be to couple these two techniques to explore the small bowel in a painless, safe and complete way (with capsule endoscopy) and to define and treat the lesions identified (with double balloon enteroscopy).

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“…Peutz-Jeghers polyps can also ulcerate, leading to acute blood loss or chronic anemia (7). Although Peutz-Jeghers polyps are most commonly found in the gastrointestinal system, they can also occur in extraintestinal sites such as the kidney, ureter, gallbladder, bronchial tree, and nasal passages (8).…”

Section: Discussionmentioning

confidence: 99%