Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network
Author:
de Pinieux Gonzague, Karanian-Philippe Marie, Loarer Francois Le, Guellec Sophie Le, Chabaud Sylvie, Terrier Philippe, Bouvier Corinne, Battistella Maxime, Neuville Agnès, Robin Yves-Marie, Emile Jean-Francois, Moreau Anne, Larousserie Frederique, Leroux Agnes, Stock Nathalie, Lae Marick, Collin Francoise, Weinbreck Nicolas, Aubert Sebastien, Mishellany Florence, Charon-Barra Céline, Croce Sabrina, Doucet Laurent, Quintin-Rouet Isabelle, Chateau Marie-Christine, Bazille Celine, Valo Isabelle, Chetaille Bruno, Ortonne Nicolas, Gomez-Brouchet Anne, Rochaix Philippe, Muret Anne De, Ghnassia Jean-Pierre, Mescam-Mancini Lenaig, Macagno Nicolas, Birtwisle-Peyrottes Isabelle, Delfour Christophe, Angot Emilie, Pommepuy Isabelle, Ranchere-Vince Dominique, Chemin-Airiau Claire, Jean-Denis Myriam, Fayet Yohan, Courrèges Jean-Baptiste, Mesli Nouria, Berchoud Juliane, Toulmonde Maud, Italiano Antoine, Cesne Axel Le, Penel Nicolas, Ducimetiere Francoise, Gouin Francois, Coindre Jean-Michel, Blay Jean-YvesORCID
Abstract
AbstractBackgroundSince 2010, NETSARC and RREPS collected and reviewed prospectively all cases of sarcomas and tumors of intermediate malignancy (TIM) nationwide.MethodsThe nationwide incidence of sarcoma or TIM (2013-2016), confirmed by expert pathologists using WHO classification are presented. Yearly variations and correlation with published clinical trials was analyzed.Results139 histological subtypes are reported among the 25172 patients with sarcomas (n=18710, 64%) or TIM (n=6460, 36%), respectively n=5838, n=6153, n=6654, and n=6527 yearly from 2013 to 2016. Over these 4 years, the yearly incidence of sarcomas and TIM was therefore 79.7, 24.9 and 95.1/106/year, above that previously reported. GIST, liposarcoma, leiomyosarcomas, undifferentiated sarcomas represented 13%, 13%, 11% and 11% of tumors. Only GIST, as a single entity had a yearly incidence above 10/million/year. There were respectively 30, 63 and 66 different histological subtypes of sarcomas or TIM with an incidence ranging from 10 to 1/106, 1-0.1/106, or < 0.1/106/year respectively. The 2 later “incidence groups” included 21% of the patients. The incidence of 8 histotypes varied significantly over this 4 years. Patients with tumors with an incidence above 1/106per year have significantly higher numbers of dedicated published phase III and phase II clinical trials (p<10−6).ConclusionsThis nationwide registry of sarcoma patients with histology reviewed by sarcoma experts shows that the incidence of sarcoma and TIM is higher than reported, and that tumors with an incidence<106/year have a much lower access to clinical trials.
Publisher
Cold Spring Harbor Laboratory
Reference27 articles.
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