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MEN1-Related Pancreatic Nets: Identification of Unmet Clinical 27 8 Endocrine-Related C R C Pieterman, Future directives in 27:8 T9–T25 Cancer S M Sadowski et al. MEN1-related PanNETs THEMATIC REVIEW HEREDITARY ENDOCRINE TUMOURS: CURRENT STATE-OF-THE-ART AND RESEARCH OPPORTUNITIES MEN1-related pancreatic NETs: identifcation of unmet clinical needs and future directives C R C Pieterman1,2,*, S M Sadowski3,*, J E Maxwell4, M H G Katz4, K E Lines5, C M Heaphy6,†, A Tirosh7, J E Blau8, N D Perrier1, M A Lewis9,10, J P Metzcar11,12, D M Halperin13, R V Thakker5 and G D Valk2 1Section of Surgical Endocrinology, Department of Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA 2Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands 3Endocrine Surgery, Surgical Oncology Program, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA 4Department of Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA 5Academic Endocrine Unit, Radclife Department of Medicine, OCDEM, University of Oxford, Oxford, UK 6Departments of Pathology and Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA 7Neuroendocrine Tumors Service, Division of Endocrinology, Metabolism and Diabetes, The Chaim Sheba Medical Center, and Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel 8Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland, USA 9Gastrointestinal Oncology, Intermountain Healthcare, Murray, Utah, USA 10American Multiple Endocrine Neoplasia Support (AMENSupport), Maryville, Tennessee, USA 11Association of Multiple Endocrine Neoplasia Disorders (AMEND), Bloomington, Indiana, USA 12Departments of Intelligent Systems Engineering and Informatics, Indiana University, Bloomington, Indiana, USA 13Department of Gastrointestinal Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA Correspondence should be addressed to D M Halperin: [email protected] *(C R C Pieterman and S M Sadowski contributed equally to this work) †(C M Heaphy is now at Department of Medicine, Boston University School of Medicine, Boston, Massachusetts, USA) This paper is part of a thematic section on current knowledge and future research opportunities in hereditary endocrine tumours, as discussed at MEN2019: 16th International Workshop on Multiple Endocrine Neoplasia, 27–29 March 2019, Houston, TX, USA. This meeting was sponsored by Endocrine-Related Cancer Abstract The PanNET Working Group of the 16th International Multiple Endocrine Neoplasia Key Words Workshop (MEN2019) convened in Houston, TX, USA, 27–29 March 2019 to discuss key f MEN1 unmet clinical needs related to PanNET in the context of MEN1, with a special focus on f multiple endocrine non-functioning (nf)-PanNETs. The participants represented a broad range of medical neoplasia type 1 scientists as well as representatives from patient organizations, pharmaceutical industry f pancreatic neuroendocrine tumor and research societies. In a case-based approach, participants addressed early detection, f conference proceeding surveillance, prognostic factors and management of localized and advanced disease. f unmet clinical needs For each topic, after a review of current evidence, key unmet clinical needs and future f research infrastructure research directives to make meaningful progress for MEN1 patients with nf-PanNETs f management were identifed. International multi-institutional collaboration is needed for adequately f risk stratifcation sized studies and validation of fndings in independent datasets. Collaboration between f surveillance basic, translational and clinical scientists is paramount to establishing a translational f management science approach. In addition, bringing clinicians, scientists and patients together f treatment improves the prioritization of research goals, assures a patient-centered approach https://erc.bioscientifca.com © 2020 Society for Endocrinology https://doi.org/10.1530/ERC-19-0441 Published by Bioscientifca Ltd. Printed in Great Britain Downloaded from Bioscientifica.com at 07/30/2020 09:08:56PM via free access -19-0441 Endocrine-Related C R C Pieterman, Future directives in 27:8 T10 Cancer S M Sadowski et al. MEN1-related PanNETs and maximizes patient involvement. It was concluded that collaboration, research infrastructure, methodologic and reporting rigor are essential to any translational science efort. The highest priority for nf-PanNETs in MEN1 syndrome are (1) the development of a data and biospecimen collection architecture that is uniform across all MEN1 centers, (2) unifed strategies for diagnosis and follow-up of incident and prevalent nf-PanNETs, (3) non-invasive detection of individual nf-PanNETs that have an increased risk of metastasis, (4) chemoprevention clinical trials driven by basic research studies and (5) therapeutic targets for advanced disease based on biologically plausible mechanisms. Endocrine-Related Cancer (2020) 27, T9–T25 Introduction For each topic, after a review of the current clinical understanding, the key unmet clinical needs and future Multiple endocrine neoplasia type 1 (MEN1), caused by research directives to make meaningful progress for pathogenic variants in the MEN1 gene on chromosome MEN1 patients with nf-PanNETs were identifed. 11q13, is a rare neuroendocrine tumor (NET) susceptibility syndrome with an approximate prevalence of 1 in 30,000 (Chandrasekharappa et al. 1997). By the age of Clinical case 80 more than 80% of the patients will have developed a duodenopancreatic NET (de Laat et al. 2016) and The patient is a 42-year-old female. She has recently multifocality is common. Non-functional pancreatic been diagnosed with MEN1 by genetic screening NETs (nf-PanNETs) are the most common type of through her 46-year-old brother, the index case. She PanNET in patients with MEN1, followed by gastrinoma, has an unremarkable medical history. She denies insulinoma and other rarer functional tumors. Metastatic any symptoms of hypoglycemia (insulinoma), duodenopancreatic NETs are the most frequent cause of acid refux, diarrhea or peptic ulcer disease disease-related death in patients with MEN1 (hazard ratio (gastrinoma) or newly developed diabetes and skin (HR) for death = 3.43 for nf-PanNETs; 95% CI 1.71–6.88) rash (glucagonoma), other functional PanNETs or (Goudet et al. 2010). Early detection or prevention are ideal, mechanical symptoms. Her vital signs and general as surgery is the only curative treatment, feasible only for physical examination are unremarkable. localized NETs. Continued systematic duodenopancreatic NET screening and follow-up are warranted, especially since MEN1 patients frequently develop additional Diagnosis and follow-up of nf-PanNETs in primaries in remnant duodenopancreatic tissue (Dralle patients with MEN1 et al. 2004, Thakker et al. 2012). Question 1: Do we need new blood-based biomarkers The PanNET Working Group of the 16th International for the diagnosis of nf-PanNETs in MEN1 and how Multiple Endocrine Neoplasia Workshop (MEN2019) should we study this? convened in Houston, TX, USA, 27–29 March 2019 to discuss key unmet clinical needs related to PanNET in State of evidence the context of MEN1, with a special focus on nf-PanNETs. Current clinical practice guidelines, published in 2012, The participants represented a broad range of medical balanced expert opinion with scarce scientifc evidence scientists including basic, translational and clinical to provide the framework of care for MEN1 patients. scientists from the felds of Endocrinology, Medical The guidelines recommended that annual screening Oncology, Surgical Oncology, Surgical Endocrinology for nf-PanNET should include fasting glucagon, and Genetic Counseling. Representatives from the chromogranin A (CgA) and pancreatic polypeptide (PP) North American and UK MEN patient advocacy groups which would expand evidence regarding their diagnostic (AMENsupport, AMEND USA and UK) were present, as value (Thakker et al. 2012). A recent systematic review were representatives from the pharmaceutical industry on the diagnosis of nf-PanNETs in MEN1, with strict and the Neuroendocrine Tumor Research Foundation defnitions of research quality and risk of bias, has (NETRF). In a case-based approach, participants addressed summarized evidence from multiple studies (van Treijen early detection, surveillance, prognostic factors and et al. 2018b). Most studies had signifcant risk of bias. Two management of localized and advanced disease. studies with the highest quality of evidence and a low https://erc.bioscientifca.com © 2020 Society for Endocrinology https://doi.org/10.1530/ERC-19-0441 Published by Bioscientifca Ltd. Printed in Great Britain Downloaded from Bioscientifica.com at 07/30/2020 09:08:56PM via free access Endocrine-Related C R C Pieterman, Future directives in 27:8 T11 Cancer S M Sadowski et al. MEN1-related PanNETs risk of bias showed low accuracy of the tumor markers function impairment. Furthermore, routine blood work (de Laat et al. 2013, Qiu et al. 2016), and annual use may already be a part of the patient’s life, more accessible in of CgA, PP and glucagon for the diagnosis of nf-PanNETs terms of travel, and less costly, all of which might facilitate in MEN1 was not recommended (van Treijen et al. 2018b). more consistent monitoring as warranted by disease stage Therefore, there are currently no available biomarkers to and quality of life (QoL).
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