Characterization, Prognosis, and Treatment of Patients With Locally Advanced or Metastatic Thymic Neuroendocrine Tumor: A Retrospective Study of the French GTE, ENDOCAN RENATEN, and RYTHMIC Networks
Year: 2025
Type: article
Study design: Observational Study
Abstract: INTRODUCTION: Thymic neuroendocrine tumors (Thy-NETs) constitute a poorly characterized ultrarare subgroup of NET. To characterize locally advanced or metastatic Thy-NET, to evaluate prognostic factors for survival, and to provide an overview of their current therapeutic management in two dedicated French national networks. METHODS: Retrospective multicenter study of two French ENDOCAN-RENATEN and RYTHMIC networks. RESULTS: A total of 74 patients (median age, 46 y) followed in 20 French centers between 1988 and 2020 were included. Main characteristics were as follows: male over female ratio of 2.7, multiple endocrine neoplasia type 1 inherited syndrome in 25 patients (34%), NET G2 atypical carcinoids in 31 patients (48%), 20 patients (31%) with NET G3 highly proliferative atypical carcinoids, and 11 patients (15%) with ectopic Cushing syndrome. Metastatic sites were as follows: lymph nodes (54 patients, 73%), bone (42 patients, 65%), lung (31 patients, 42%), and pleura (31 patients, 42%). A total of 64 patients (87%) had locally advanced Thy-NET and eight (11%) had synchronous metastases, at diagnosis. Pleural metastases were almost exclusively metachronous. Positive results for 18F-FDG or somatostain receptor imaging was found in 88% or 74% of cases, respectively. The 5-year overall survival was 85%. Univariate analysis on overall survival reveals a trend toward worse prognosis in case of female gender and bone metastases and better prognosis for patients with multiple endocrine neoplasia type 1. Partial responses were more frequently observed in patients treated with cytotoxic chemotherapy. CONCLUSION: This large series identifies specific characteristics of patients with advanced Thy-NET. Prognosis was better than previously reported. Perioperative pleural dissemination is suspected. Partial responses were mainly observed with chemotherapy.
Source: Journal of Thoracic Oncology
Authors: Sarah Fodil-Cherif, Julien Hadoux, Olaf Mercier, Jose Carlos Benitez, Alice Durand, Helene Lasolle, Marine Perrier, Arnaud Jannin, Sandrine Laboureau, Maelle Le Bras, Olivier Chabre, Sébastien Gaujoux, Lionel Groussin Rouiller, Olivia Hentic, Hélène Du Boullay, Magalie Haissaguerre, Hervé Kinn, Thierry Lecomte, Pierre Goudet, Nicolas Girard, Jean-Yves Scoazec, Benjamin Besse, Matthieu Faron, Eric Baudin
Institutions: Hôpital Lapeyronie, Institut Gustave Roussy, Université Paris-Saclay, Hôpital Marie Lannelongue, Instituto de Investigación Biomédica de Málaga, Hospices Civils de Lyon, Centre Hospitalier Universitaire de Reims, Centre National de la Recherche Scientifique, Inserm, Université de Lille, Centre Hospitalier Universitaire de Lille, CANTHER - Hétérogénéité, Plasticité et Résistance aux Thérapies des Cancers, Centre Hospitalier Universitaire d'Angers, Centre Hospitalier Universitaire de Nantes, Institut du Thorax, Nantes Université, Commissariat à l'Énergie Atomique et aux Énergies Alternatives, Université Grenoble Alpes, Assistance Publique – Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Université Paris Cité, Hôpital Cochin, Institut Cochin, Hôpital Beaujon, Centre Hospitalier Métropole Savoie, Université de Bordeaux, Hôpital Cardiologique du Haut-Lévêque, Centre Hospitalier Régional Universitaire de Brest, Université de Tours, Centre Hospitalier Universitaire de Tours, Niche, Nutrition, Cancer et métabolisme oxydatif, CHU Dijon Bourgogne, Centre de recherche Translationnelle en Médecine moléculaire, Université de Versailles Saint-Quentin-en-Yvelines, Institut Curie, Fondation Gustave Roussy
Language: English
Subfield: Epidemiology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $4,100
OpenAlex ID: W4410074873