Screening and surveillance practices for Multiple Endocrine Neoplasia type 1-related Neuroendocrine Tumours in European Neuroendocrine Tumor Society Centers of Excellence (ENETS CoE)-An ENETS MEN1 task force questionnaire study

Carolina R C Pieterman¹, Simona Grozinsky-Glasberg², Dermot O'Toole³, James R Howe⁴, Valentina Ambrosini^{5

6}, Susana H Belli⁷, Mikkel Andreassen⁸, Nehara Begum⁹, Timm Denecke¹⁰, Antongiulio Faggiano¹¹, Massimo Falconi¹², Jo Grey¹³, Ulrich P Knigge¹⁴, Teodora Kolarova¹⁵, Bruno Niederle¹⁶, Els Nieveen van Dijkum¹⁷, Stefano Partelli¹⁸, Andreas Pascher¹⁹, Guido Rindi^{20

21

22}, Philippe Ruszniewski²³, Stefan Stättner²⁴, Timon Vandamme^{25

26}, Juan W Valle^{27

28}, Marie-Pierre Vullierme^{29

30}, Staffan Welin³¹, Aurel Perren³², Detlef K Bartsch³³, Gregory K Kaltsas³⁴, Gerlof D Valk¹

Affiliations

¹ Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, the Netherlands.
² Neuroendocrine Unit, ENETS Center of Excellence, Division of Medicine, Hadassah Medical Center and Faculty of Medicine, The Hebrew University, Jerusalem, Israel.
³ Department of Clinical Medicine, St James Hospital, Trinity College Dublin & National Centre for Neuroendocrine Tumours, St Vincent's University Hospital, Dublin, Ireland.
⁴ University of Iowa Carver College of Medicine, Iowa City, Iowa, USA.
⁵ Nuclear Medicine, Alma Mater Studiorum, University of Bologna, Bologna, Italy.
⁶ Nuclear Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.
⁷ Endocrinology Instituto Alexander Fleming, Buenos Aires, Argentina.
⁸ Department of Endocrinologt, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark.
⁹ Department of General-, Visceral-, Thoracic- and Endocrine Surgery, Johannes Wesling Klinikum Minden, University Hospital of the Ruhr-University Bochum (RUB), Minden, Germany.
¹⁰ Klinik für Diagnostische und Interventionelle Radiologie, Universitätsklinikum Leipzig, Leipzig, Germany.
¹¹ Endocrinology Unit, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, ENETS Center of Excellence, Sapienza University of Rome, Rome, Italy.
¹² Pancreatic and Transplantation Surgical Unit, IRCCS San Raffaele Hospital, Università Vita-Salute, Milan, Italy.
¹³ AMEND (Association for Multiple Endocrine Neoplasia Disorder), Tornbridge, UK.
¹⁴ Department of Endocrinology and Department of Surgery and Transplantation, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
¹⁵ Executive Director International Neuroendocrine Cancer Alliance (INCA), Boston, Massachusetts, USA.
¹⁶ Division of Visceral Surgery, Department of General Surgery, Medical University, Vienna, Austria.
¹⁷ Department of Surgery, Cancer Center Amsterdam, University of Amsterdam, Amsterdam, The Netherlands.
¹⁸ Vita-Salute San Raffaele University, IRCCS San Raffaele Scientific Institute, Milan, Italy.
¹⁹ Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Universitätsklinikum Münster, Münster, Germany.
²⁰ Department of Life Sciences and Public Health, Università Cattolica del Sacro Cuore, Rome, Italy.
²¹ Department of Woman and Child Health Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
²² European NeuroEndocrine Tumor Society (ENETS) Center of Excellence Roma-Gemelli, Roma, Italy.
²³ Université de Paris, Beaujon Hospital, Clichy, France.
²⁴ Department of General, Visceral and Vascular Surgery, Salzkammergut Klinikum Vöcklabruck, Oberösterreichische Gesundheitsholding OÖG, Vöcklabruck, Austria.
²⁵ NETwerk, Department of Oncology, Antwerp University Hospital, Antwerp, Belgium.
²⁶ Integrated Personalized and Precision Oncology Network (IPPON), Center for Oncological Research (CORE), University of Antwerp, Antwerp, Belgium.
²⁷ Division of Cancer Sciences, University of Manchester, Manchester, UK.
²⁸ Cholangiocarcinoma Foundation, Herriman, Utah, USA.
²⁹ Université Paris Diderot, Paris, France.
³⁰ Service de Radiologie, AP-HP Hôpital Beaujon, Clichy, France.
³¹ Endocrine Oncology, Department of Medical sciences Uppsala University, Uppsala, Sweden.
³² Institute of Tissue Medicine and Pathology, University of Bern, Bern, Switzerland.
³³ Department of Visceral-, Thoracic and Vascular Surgery, Philipps-University Marburg, Marburg, Germany.
³⁴ 1st Propaedeutic Department of Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece.

PMID: 39587981
PMCID: PMC11750319
DOI: 10.1111/jne.13468

Screening and surveillance practices for Multiple Endocrine Neoplasia type 1-related Neuroendocrine Tumours in European Neuroendocrine Tumor Society Centers of Excellence (ENETS CoE)-An ENETS MEN1 task force questionnaire study

Carolina R C Pieterman et al. J Neuroendocrinol. 2025 Jan.

. 2025 Jan;37(1):e13468.

doi: 10.1111/jne.13468. Epub 2024 Nov 26.

Authors

Affiliations

¹ Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, the Netherlands.
² Neuroendocrine Unit, ENETS Center of Excellence, Division of Medicine, Hadassah Medical Center and Faculty of Medicine, The Hebrew University, Jerusalem, Israel.
³ Department of Clinical Medicine, St James Hospital, Trinity College Dublin & National Centre for Neuroendocrine Tumours, St Vincent's University Hospital, Dublin, Ireland.
⁴ University of Iowa Carver College of Medicine, Iowa City, Iowa, USA.
⁵ Nuclear Medicine, Alma Mater Studiorum, University of Bologna, Bologna, Italy.
⁶ Nuclear Medicine, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.
⁷ Endocrinology Instituto Alexander Fleming, Buenos Aires, Argentina.
⁸ Department of Endocrinologt, Copenhagen University Hospital Rigshospitalet, Copenhagen, Denmark.
⁹ Department of General-, Visceral-, Thoracic- and Endocrine Surgery, Johannes Wesling Klinikum Minden, University Hospital of the Ruhr-University Bochum (RUB), Minden, Germany.
¹⁰ Klinik für Diagnostische und Interventionelle Radiologie, Universitätsklinikum Leipzig, Leipzig, Germany.
¹¹ Endocrinology Unit, Department of Clinical and Molecular Medicine, Sant'Andrea Hospital, ENETS Center of Excellence, Sapienza University of Rome, Rome, Italy.
¹² Pancreatic and Transplantation Surgical Unit, IRCCS San Raffaele Hospital, Università Vita-Salute, Milan, Italy.
¹³ AMEND (Association for Multiple Endocrine Neoplasia Disorder), Tornbridge, UK.
¹⁴ Department of Endocrinology and Department of Surgery and Transplantation, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
¹⁵ Executive Director International Neuroendocrine Cancer Alliance (INCA), Boston, Massachusetts, USA.
¹⁶ Division of Visceral Surgery, Department of General Surgery, Medical University, Vienna, Austria.
¹⁷ Department of Surgery, Cancer Center Amsterdam, University of Amsterdam, Amsterdam, The Netherlands.
¹⁸ Vita-Salute San Raffaele University, IRCCS San Raffaele Scientific Institute, Milan, Italy.
¹⁹ Klinik für Allgemein-, Viszeral- und Transplantationschirurgie, Universitätsklinikum Münster, Münster, Germany.
²⁰ Department of Life Sciences and Public Health, Università Cattolica del Sacro Cuore, Rome, Italy.
²¹ Department of Woman and Child Health Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
²² European NeuroEndocrine Tumor Society (ENETS) Center of Excellence Roma-Gemelli, Roma, Italy.
²³ Université de Paris, Beaujon Hospital, Clichy, France.
²⁴ Department of General, Visceral and Vascular Surgery, Salzkammergut Klinikum Vöcklabruck, Oberösterreichische Gesundheitsholding OÖG, Vöcklabruck, Austria.
²⁵ NETwerk, Department of Oncology, Antwerp University Hospital, Antwerp, Belgium.
²⁶ Integrated Personalized and Precision Oncology Network (IPPON), Center for Oncological Research (CORE), University of Antwerp, Antwerp, Belgium.
²⁷ Division of Cancer Sciences, University of Manchester, Manchester, UK.
²⁸ Cholangiocarcinoma Foundation, Herriman, Utah, USA.
²⁹ Université Paris Diderot, Paris, France.
³⁰ Service de Radiologie, AP-HP Hôpital Beaujon, Clichy, France.
³¹ Endocrine Oncology, Department of Medical sciences Uppsala University, Uppsala, Sweden.
³² Institute of Tissue Medicine and Pathology, University of Bern, Bern, Switzerland.
³³ Department of Visceral-, Thoracic and Vascular Surgery, Philipps-University Marburg, Marburg, Germany.
³⁴ 1st Propaedeutic Department of Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece.

PMID: 39587981
PMCID: PMC11750319
DOI: 10.1111/jne.13468

Abstract

Multiple Endocrine Neoplasia type 1 (MEN1) Clinical Practice Guidelines (2012) are predominantly based on expert opinion due to limited available evidence at the time, leaving room for interpretation and variation in practices. Evidence on the natural course of MEN1-related neuroendocrine tumours (NET) and the value of screening programs has increased and new imaging techniques have emerged. The aim of this study is to provide insight in the current practices of screening and surveillance for MEN1-related NETs in ENETS Centers of Excellence (CoEs). A clinical practice questionnaire was distributed among all 65 ENETS CoEs. Response rate was 91% (59/65). In 14% of CoEs <10 patients, in 50% 10-49, in 31% 50-100 and in 3 centres (5%) >100 patients with MEN1 are seen. Practices with regard to screening and surveillance of NETs were markedly heterogeneous. Differences between countries were noted in the use of gut hormones for biochemical screening and the choice for imaging modality for screening/surveillance of pancreatic NETs (PanNETs). Magnetic resonance imaging (MRI) is the preferred modality for screening and surveillance of PanNETs, whereas this is computed tomography (CT) for thoracic NETs. Practices regarding screening for thoracic NETs were more homogeneous among larger volume CoEs, with longer screening intervals. The majority of CoEs tailored the surveillance of small pancreatic and lung NETs to observed growth rate. 68% of CoEs advise patients with clinical MEN1 with negative genetic testing to undergo periodic screening like mutation-positive patients. In conclusion, there is still marked heterogeneity in practice, although there are also common trends. Differences were sometimes associated with volume or country, but often no association was found. This underscores the need for clear and evidence-based practice recommendations.

Keywords: multiple endocrine neoplasia type 1; neuroendocrine tumour; screening; surveillance; survey.

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Conflict of interest statement

Dermot O'Toole reports previous educational grants from Novartis, Ipsen, Wyeth Leferle and AstraZenca. Valentina Ambrosini reports personal fees from AAA outside the submitted work. Guido Rindi reports past activities for AAA speakers bureau and Bracco Imaging Suisse, consultant. The other authors have no conflict of interest to declare.

Figures

**FIGURE 1**
Flow chart of survey responses. CoE Center of Excellence, MEN1 Multiple Endocrine Neoplaisa Type 1.

See this image and copyright information in PMC

References

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Screening and surveillance practices for Multiple Endocrine Neoplasia type 1-related Neuroendocrine Tumours in European Neuroendocrine Tumor Society Centers of Excellence (ENETS CoE)-An ENETS MEN1 task force questionnaire study

Affiliations

Screening and surveillance practices for Multiple Endocrine Neoplasia type 1-related Neuroendocrine Tumours in European Neuroendocrine Tumor Society Centers of Excellence (ENETS CoE)-An ENETS MEN1 task force questionnaire study

Authors

Affiliations

Abstract

Conflict of interest statement

Figures

References

MeSH terms

LinkOut - more resources

Full Text Sources