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The Management of Peutz–Jeghers Syndrome: European Hereditary Tumour Group (EHTG) Guideline †

Department of Clinical Genetics, Erasmus MC Cancer Institute, University Medical Center Rotterdam, 3000CA Rotterdam, The Netherlands
Institute of Human Genetics, Medical Faculty, University of Bonn, 53127 Bonn, Germany
National Center for Hereditary Tumor Syndromes, University Hospital Bonn, 53127 Bonn, Germany
Department of Obstetrics and Gynecology and Tays Cancer Center, Tampere University Hospital, 33520 Tampere, Finland
Department of Gastroenterology & Hepatology, Erasmus MC Cancer Institute, University Medical Center Rotterdam, 3000CA Rotterdam, The Netherlands
Division of Experimental Oncology, Gastroenterology and Gastrointestinal Endoscopy Unit, Vita-Salute San Raffaele University, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy
Department of Gynecology, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester M13 9WL, UK
Division of Cancer Sciences, Faculty of Biology, Medicine and Health, University of Manchester, St Mary’s Hospital, Manchester M13 9WL, UK
Department of Clinical Genetics, University Hospital of Copenhagen, 2100 Copenhagen, Denmark
Department of Surgery and Cancer, Imperial College London, London SW7 2AZ, UK
Polyposis Registry, St. Marks Hospital, London HA1 3UJ, UK
Department of Gastro-intestinal Oncology, Netherlands Cancer Institute, 1006BE Amsterdam, The Netherlands
Department of Gastroenterology and Hepatology, Leiden University Medical Center, 2300RC Leiden, The Netherlands
Department of Surgery, University Hospital of Helsinki, 00029 Helsinki, Finland
Department of Genomic Medicine, The Royal Melbourne Hospital, University of Melbourne, Melbourne 3052, Australia
Breast Surgery Unit, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy
Center for Hereditary Tumors, Ev. BETHESDA Khs. Duisburg, Academic Hospital University of Düsseldorf, 47053 Duisburg, Germany
Author to whom correspondence should be addressed.
This Guideline is an official statement of the European Hereditary Tumor Group (EHTG). The Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system was adopted to define the strength of recommendations and the quality of evidence.
J. Clin. Med. 2021, 10(3), 473;
Received: 25 December 2020 / Revised: 21 January 2021 / Accepted: 23 January 2021 / Published: 27 January 2021
(This article belongs to the Special Issue Genetic Epidemiology of Inherited Cancers)
The scientific data to guide the management of Peutz–Jeghers syndrome (PJS) are sparse. The available evidence has been reviewed and discussed by diverse medical specialists in the field of PJS to update the previous guideline from 2010 and formulate a revised practical guideline for colleagues managing PJS patients. Methods: Literature searches were performed using MEDLINE, Embase, and Cochrane. Evidence levels and recommendation strengths were assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE). A Delphi process was followed, with consensus being reached when ≥80% of the voting guideline committee members agreed. Recommendations and statements: The only recent guidelines available were for gastrointestinal and pancreatic management. These were reviewed and endorsed after confirming that no more recent relevant papers had been published. Literature searches were performed for additional questions and yielded a variable number of relevant papers depending on the subject addressed. Additional recommendations and statements were formulated. Conclusions: A decade on, the evidence base for recommendations remains poor, and collaborative studies are required to provide better data about this rare condition. Within these restrictions, multisystem, clinical management recommendations for PJS have been formulated. View Full-Text
Keywords: Peutz–Jeghers syndrome; STK11; guideline Peutz–Jeghers syndrome; STK11; guideline
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MDPI and ACS Style

Wagner, A.; Aretz, S.; Auranen, A.; Bruno, M.J.; Cavestro, G.M.; Crosbie, E.J.; Goverde, A.; Jelsig, A.M.; Latchford, A.R.; van Leerdam, M.E.; Lepisto, A.H.; Puzzono, M.; Winship, I.; Zuber, V.; Möslein, G. The Management of Peutz–Jeghers Syndrome: European Hereditary Tumour Group (EHTG) Guideline. J. Clin. Med. 2021, 10, 473.

AMA Style

Wagner A, Aretz S, Auranen A, Bruno MJ, Cavestro GM, Crosbie EJ, Goverde A, Jelsig AM, Latchford AR, van Leerdam ME, Lepisto AH, Puzzono M, Winship I, Zuber V, Möslein G. The Management of Peutz–Jeghers Syndrome: European Hereditary Tumour Group (EHTG) Guideline. Journal of Clinical Medicine. 2021; 10(3):473.

Chicago/Turabian Style

Wagner, Anja, Stefan Aretz, Annika Auranen, Marco J. Bruno, Giulia M. Cavestro, Emma J. Crosbie, Anne Goverde, Anne Marie Jelsig, Andrew R. Latchford, Monique E. van Leerdam, Anna H. Lepisto, Marta Puzzono, Ingrid Winship, Veronica Zuber, and Gabriela Möslein. 2021. "The Management of Peutz–Jeghers Syndrome: European Hereditary Tumour Group (EHTG) Guideline" Journal of Clinical Medicine 10, no. 3: 473.

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