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James C Yao University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Jonathan Strosberg Department of GI Oncology, Moffitt Cancer Center, Tampa, Florida, USA

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Nicola Fazio European Institute of Oncology, IEO, IRCCS, Milan, Italy

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Marianne E Pavel Department of Medicine 1, Friedrich Alexander University Erlangen-Nuremberg, Erlangen, Germany

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Emily Bergsland UCSF Helen Diller Family Comprehensive Cancer Center, San Francisco, California, USA

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Philippe Ruszniewski Hôpital Beaujon, University of Paris, Paris, France

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Daniel M Halperin University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Daneng Li City of Hope Comprehensive Cancer Center and Beckman Research Institute, Duarte, California, USA

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Salvatore Tafuto Sarcomas and Rare Tumours Unit, Istituto Nazionale Tumori, IRCCS Fondazione G. Pascale, Naples, Italy

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Nitya Raj Memorial Sloan Kettering Cancer Center, New York, New York, USA

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Davide Campana Department of Clinical Medicine, Sant’Orsola-Malpighi Hospital, University of Bologna, ENETS Center of Excellence, Bologna, Italy

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Susumu Hijioka National Cancer Center Japan Tsukiji Campus, Department of Hepatobiliary and Pancreatic Oncology, Tokyo, Japan

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Markus Raderer Clinical Division of Oncology, Medical University of Vienna, Vienna, Austria

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Rosine Guimbaud CHU de Toulouse, Toulouse, France

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Pablo Gajate Hospital Universitário Ramón y Cajal, Clinical Oncology Department, Madrid, Spain

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Sara Pusceddu Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

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Albert Reising Novartis Pharmaceuticals Corporation, East Hanover, New Jersey, USA

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Evgeny Degtyarev Novartis Pharmaceuticals Corporation, East Hanover, New Jersey, USA

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Mark Shilkrut Novartis Pharmaceuticals Corporation, East Hanover, New Jersey, USA

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Simantini Eddy Novartis Pharmaceuticals Corporation, East Hanover, New Jersey, USA

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Simron Singh Sunnybrook Health Sciences Centre, Toronto, Canada

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. Immunotherapy has been studied to a limited extend in NENs, demonstrating low objective response rates ( Mehnert et al. 2020 ). Spartalizumab (PDR001) is a high-affinity, ligand-blocking, humanized IgG4 antibody directed against PD-1 receptor, that blocks

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Anna Angelousi 1st Department of Internal Medicine, Unit of Endocrinology, Laikon Hospital, Athens, Greece

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Ploutarchos Tzoulis Department of Metabolism & Experimental Therapeutics, Division of Medicine, University College London, London, UK

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Marina Tsoli 1st Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Eleftherios Chatzellis 251 HAF and VA Hospital, Athens, Greece

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Anna Koumarianou Fourth Department of Internal Medicine, Hematology Oncology Unit, Attikon University Hospital, National and Kapodistrian University of Athens, Greece

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Gregory Kaltsas 1st Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece

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alone in ATC ( Capdevila et al. 2020 , De Leo et al. 2020 , Boudin et al. 2022 , Hatashima et al. 2022 ). The first, a phase II study evaluating spartalizumab, a monoclonal antibody against PD-1 receptor, in patients with locally advanced

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A Mohamed Division of Hematology and Medical Oncology, UH Seidman Cancer Center, Case Comprehensive Cancer Center, Case Western Reserve University, Cleveland, Ohio, USA

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M Trybula Division of Hematology and Medical Oncology, UH Seidman Cancer Center, Case Comprehensive Cancer Center, Case Western Reserve University, Cleveland, Ohio, USA

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S L Asa Department of Medicine, UH Seidman Cancer Center Case Comprehensive Cancer Center, Case Western Reserve University, Cleveland, Ohio, USA

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T R Halfdanarson Division of Medical Oncology, Department of Oncology, Mayo Clinic Comprehensive Cancer Center, Rochester, Minnesota, USA

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M B Sonbol Division of Hematology and Medical Oncology, Mayo Clinic Cancer Center, Phoenix, Arizona, USA

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-institution experiences ( Table 3 ). A phase II multicenter trial for spartalizumab in patients with both GEP and thoracic NENs showed a low ORR (7.4% in well-differentiated G3 GEP NETs and 4.8% in GEP NECs) ( Yao et al. 2021 ). Although Ki-67 was > 20% in some of the

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Clotilde Sparano Nuclear Medicine and Endocrine Oncology, Gustave Roussy, Université Paris Saclay, Villejuif, France
Endocrinology Unit, Department of Experimental and Clinical Biomedical Sciences ‘Mario Serio’, University of Florence, Florence, Italy

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Yann Godbert Nuclear Medicine, and Thyroid Oncology Department, Institut Bergonié, Bordeaux, France

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Marie Attard Radiology, Gustave Roussy and Université Paris Saclay, Villejuif, France

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Christine Do Cao Endocrinology, Diabetology and Metabolism, CHRU Lille, Lille, France

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Slimane Zerdoud Nuclear medicine, Claudius-Regaud Institute, Oncology University Institute-IUCT-Oncopole, Toulouse, France

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Nathalie Roudaut Department of Endocrinology, University Hospital of Brest, Brest, France

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Charlotte Joly Department of Oncology, Henri-Mondor Hospital, Créteil, France

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Amandine Berdelou Nuclear Medicine and Endocrine Oncology, Gustave Roussy, Université Paris Saclay, Villejuif, France

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Julien Hadoux Nuclear Medicine and Endocrine Oncology, Gustave Roussy, Université Paris Saclay, Villejuif, France

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Livia Lamartina Nuclear Medicine and Endocrine Oncology, Gustave Roussy, Université Paris Saclay, Villejuif, France

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Martin Schlumberger Nuclear Medicine and Endocrine Oncology, Gustave Roussy, Université Paris Saclay, Villejuif, France

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Sophie Leboulleux Nuclear Medicine and Endocrine Oncology, Gustave Roussy, Université Paris Saclay, Villejuif, France

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II trial with spartalizumab showed promising and durable results in advanced ATC, with an overall response rate of 19% and also three cases of complete response, regardless of BRAF gene molecular status ( Capdevila et al. 2020 ). If early phase

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Pedro Iglesias Department of Endocrinology, Hospital Universitario Puerta de Hierro Majadahonda, Majadahonda, Madrid, Spain
Instituto de Investigación Sanitaria Puerta de Hierro Segovia de Arana (IDIPHISA), Majadahonda, Madrid, Spain

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Inmaculada Peiró Clinical Nutrition Unit, Catalan Institute of Oncology, L’Hospitalet de Llobregat, Barcelona, Spain
Unit of Nutrition and Cancer-IDIBELL, L’Hospitalet de Llobregat, Barcelona, Spain

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Betina Biagetti Department of Endocrinology, Universitari Vall d’Hebron, Barcelona, Spain

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Miguel Paja-Fano Department of Endocrinology, Hospital Universitario de Basurto, Bilbao, Spain

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Diana Ariadel Cobo Department of Endocrinology, Complejo Asistencial Universitario de León, León, Spain

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Carlos García Gómez Department of Endocrinology, Hospital Universitario Puerta de Hierro Majadahonda, Majadahonda, Madrid, Spain

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Manuel Mateu-Salat Department of Endocrinology, Hospital Sant Pau, Hospital Sant Pau, Barcelona, Spain
Department of Medicine/Endocrinology, IIB-Sant Pau, Research Center for Pituitary Diseases, Barcelona, Spain

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Idoia Genua Department of Endocrinology, Hospital Sant Pau, Hospital Sant Pau, Barcelona, Spain
Department of Medicine/Endocrinology, IIB-Sant Pau, Research Center for Pituitary Diseases, Barcelona, Spain

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Margarita Majem Department of Oncology, Hospital Sant Pau, Barcelona, Spain

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Mariona Riudavets Department of Medical Oncology, Gustave Roussy Cancer Campus, Villejuif, France

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Javier Gavira Department of Oncology, Hospital Sant Pau, Barcelona, Spain

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Cristina Lamas Department of Endocrinology, Hospital General Universitario de Albacete, Albacete, Spain

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Antía Fernández Pombo Department of Endocrinology, Hospital Clínico Universitario de Santiago, Santiago de Compostela, A Coruña, Spain

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Fernando Guerrero-Pérez Department of Endocrinology, Hospital Universitari de Bellvitge, L’Hospitalet de Llobregat, Barcelona, Spain

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Carles Villabona Department of Endocrinology, Hospital Universitari de Bellvitge, L’Hospitalet de Llobregat, Barcelona, Spain

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José Manuel Cabezas Agrícola Department of Endocrinology, Hospital Clínico Universitario de Santiago, Santiago de Compostela, A Coruña, Spain

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Susan M Webb Department of Endocrinology, Hospital Sant Pau, Hospital Sant Pau, Barcelona, Spain
Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER, Unidad 747), ISCIII, Universitat Autònoma de Barcelona, Barcelona, Spain

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Juan J Díez Department of Endocrinology, Hospital Universitario Puerta de Hierro Majadahonda, Majadahonda, Madrid, Spain
Instituto de Investigación Sanitaria Puerta de Hierro Segovia de Arana (IDIPHISA), Majadahonda, Madrid, Spain
Department of Medicine, Universidad Autónoma de Madrid, Madrid, Spain

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.7)  Atezolizumab 1 (2.7)  Tremelimumab 1 (2.7)  Spartalizumab 1 (2.7)  Others 3 (8.1) ICI regimen, n (%)  Monotherapy 27 (73.0)  Combined therapy 10 (27.0) Time to develop IAD after starting ICI (months

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Philipp Melhorn Department of Medicine I, Division of Oncology, Medical University of Vienna, Vienna, Austria

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Peter Mazal Department of Pathology, Medical University of Vienna, Vienna, Austria

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Ladislaia Wolff Department of Medicine I, Division of Oncology, Medical University of Vienna, Vienna, Austria

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Petar Popov Department of Medicine I, Division of Oncology, Medical University of Vienna, Vienna, Austria

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Elisabeth Kretschmer-Chott Department of Biomedical Imaging and Image-guided Therapy, Division of Nuclear Medicine, Medical University of Vienna, Vienna, Austria

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Alexander Haug Department of Biomedical Imaging and Image-guided Therapy, Division of Nuclear Medicine, Medical University of Vienna, Vienna, Austria

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Marius E Mayerhoefer Department of Radiology, Memorial Sloan Kettering Cancer Center, New York, New York, USA
Weill Cornell Medical College, Cornell University, New York, New York, USA

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Markus Raderer Department of Medicine I, Division of Oncology, Medical University of Vienna, Vienna, Austria

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Barbara Kiesewetter Department of Medicine I, Division of Oncology, Medical University of Vienna, Vienna, Austria

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, mediastinum, pleura, and psoas muscle; b Lanreotide/temozolomide, pembrolizumab, streptozotocin/doxorubicin, topotecan, ACO, EPICO, FOLFOX, spartalizumab, and investigational medicinal product. Treatment specifics The median number of TEMCAP

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Andrew McDonald Winship Cancer Institute at Emory University, Atlanta, Georgia, USA

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Vaidehi Avadhani Grady Memorial Hospital, Atlanta, Georgia, USA

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Gabriela Oprea-Ilies Grady Memorial Hospital, Atlanta, Georgia, USA

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Katerina Zakka Winship Cancer Institute at Emory University, Atlanta, Georgia, USA

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Gregory B Lesinski Winship Cancer Institute at Emory University, Atlanta, Georgia, USA

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Olumide B Gbolahan Winship Cancer Institute at Emory University, Atlanta, Georgia, USA

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Olatunji Alese Winship Cancer Institute at Emory University, Atlanta, Georgia, USA

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DM Li D Tafuto S Raj N , et al. 2021 Spartalizumab in metastatic, well/poorly differentiated neuroendocrine neoplasms . Endocrine-Related Cancer 28 161 – 172 . ( https://doi.org/10.1530/ERC-20-0382 ) Ye L Ye H Zhou Q Li Z Lin Q Tan

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Tim J Takkenkamp Department of Surgery, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

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Mathilde Jalving Department of Medical Oncology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

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Frederik J H Hoogwater Department of Surgery, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

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Annemiek M E Walenkamp Department of Medical Oncology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

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NECs (GEP-NECs) ( n  = 21). The study analysed spartalizumab, a PD-1 inhibitor, and found that in the well-differentiated cohort, there were seven partial responses (7%) and 55% had stable disease, while 31% had progressive disease. The confirmed

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Mairéad G McNamara Department of Medical Oncology, The Christie NHS Foundation Trust, Manchester, UK
Division of Cancer Sciences, University of Manchester, Manchester, UK

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Jean-Yves Scoazec Department of Pathology, Gustave Roussy Cancer Campus, Villejuif, France
Université Paris Sud, Faculté de Médecine de Bicêtre, Le Kremlin-Bicêtre, France

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Thomas Walter Department of Gastroenterology and Medical Oncology, Edouard Herriot Hospital, Hospices Civils de Lyon, Lyon, France

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studies, thus far, have been conflicting. In preliminary analysis of a phase II study (NCT 02955069), the activity and safety of spartalizumab (PDR001), a high-affinity, humanised, anti-PD-1 IgG4 antibody that blocks PD-L1 and PD-L2 binding to PD-1, was

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Halfdan Sorbye Department of Oncology, Haukeland University Hospital, Bergen, Norway
Department of Clinical Sciences, University of Bergen, Bergen, Norway

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Grace Kong Department of Molecular Imaging and Therapeutic Nuclear Medicine, Peter MacCallum Cancer Centre, Melbourne, Australia
Sir Peter MacCallum Department of Oncology, The University of Melbourne, Melbourne, Australia

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Simona Grozinsky-Glasberg Neuroendocrine Tumor Unit, ENETS Center of Excellence, Department of Endocrinology and Metabolism, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

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Bergsland E , et al . 2018 Activity and safety of spartalizumab (PDR001) in patients with advanced neuroendocrine tumors (NET) of pancreatic (Pan), gastrointestinal (GI), or thoracic (T) origin, and gastroenteropancreatic neuroendocrine carcinoma (GEP

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