Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network

Gonzague de Pinieux , Marie Karanian , Francois Le Loarer , Sophie Le Guellec , Sylvie Chabaud , Philippe Terrier , Corinne Bouvier , Maxime Batistella , Agnès Neuville , Yves-Marie Robin , Jean-Francois Emile , Anne Moreau , Frederique Larousserie , Agnes Leroux , Nathalie Stock , Marick Lae , Francoise Collin , Nicolas Weinbreck , Sébastien Aubert , Florence Mishellany (1) , Celine Charon-Barra , Sabrina Croce , Laurent Doucet , Isabelle Quintin-Rouet , Marie-Christine Chateau , Celine Bazille , Isabelle Valo , Bruno Chetaille , Nicolas Ortonne , Anne Brouchet , Philippe Rochaix , Anne Demuret , Jean-Pierre Ghnassia , Lenaig Mescam , Nicolas Macagno (2, 3) , Isabelle Birtwisle-Peyrottes , Christophe Delfour , Emilie Angot , Isabelle Pommepuy , Dominique Ranchere , Claire Chemin-Airiau , Myriam Jean-Denis , Yohan Fayet , Jean-Baptiste Courrèges , Nouria Mesli , Juliane Berchoud , Maud Toulmonde , Antoine Italiano , Axel Le Cesne , Nicolas Penel , Francoise Ducimetiere , Francois Gouin , Jean-Michel Coindre , Jean-Yves Blay

1 INSERM U1240 IMoST, Université Clermont Auvergne, Clermont-Ferrand, France; Centre d'Investigation Clinique UMR 501, Clermont-Ferrand, France; Departement de recherche clinique, Délégation Recherche Clinique et Innovation, Centre Jean PERRIN, Clermont-Ferrand, France
2 MMG - Marseille medical genetics - Centre de génétique médicale de Marseille
3 ACPNP - Service d'Anatomo-Cyto-Pathologie et de NeuroPathologie [Hôpital de la Timone - APHM]

Background Since 2010, nationwide networks of reference centers for sarcomas (RREPS/NETSARC/RESOS) collected and prospectively reviewed all cases of sarcomas and connective tumors of intermediate malignancy (TIM) in France. Methods The nationwide incidence of sarcoma or TIM (2013–2016) was measured using the 2013 WHO classification and confirmed by a second independent review by expert pathologists. Simple clinical characteristics, yearly variations and correlation of incidence with published clinical trials are presented and analyzed. Results Over 150 different histological subtypes are reported from the 25172 patients with sarcomas (n = 18712, 74,3%) or TIM (n = 6460, 25.7%), with n = 5838, n = 6153, n = 6654, and n = 6527 yearly cases from 2013 to 2016. Over these 4 years, the yearly incidence of sarcomas and TIM was therefore 70.7 and 24.4 respectively, with a combined incidence of 95.1/10 6 /year, higher than 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 10/10 6 /year. There were respectively 30, 64 and 66 different histological subtypes of sarcomas or TIM with an incidence ranging from 10 to 1/10 6 , 1–0.1/10 6 , or < 0.1/10 6 /year respectively. The 2 latter incidence groups represented 21% of the patients with 130 histotypes. Published phase III and phase II clinical trials (p<10 −6 ) are significantly higher with sarcomas subtypes with an incidence above 1/10 6 per. Conclusions This nationwide registry of sarcoma patients, with exhaustive histology review by sarcoma experts, shows that the incidence of sarcoma and TIM is higher than reported, and that tumors with a very low incidence (1<10 6 /year) are less likely to be included in clinical trials.

Mots clés: Sarcoma; Metastatic tumors; Cancer epidemiology; Angiosarcoma; Neurological tumors; Skin tumors; Gastrointestinal cancers; Malignant tumors

Domaines: Sciences du Vivant [q-bio]; Cancer; Médecine humaine et pathologie

Gonzague de Pinieux, Marie Karanian, Francois Le Loarer, Sophie Le Guellec, Sylvie Chabaud, et al.. Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network. PLoS ONE, 2021, 16 (2), pp.e0246958. ⟨10.1371/journal.pone.0246958⟩. ⟨hal-03208671⟩