Survival and Health Care Burden of Children With Retinoblastoma in Europe
Authors: Virgili Gianni , Capocaccia Riccardo , Botta Laura , Bennett Damien , Hadjistilianou Theodora , Innos Kaire , Karim-Kos Henrike , Kuehni Claudia E. , Kuhnel Ursula , Mazzini Cinzia , Canete Nieto Adela , Paapsi Keiu , Parravano Mariacristina , Ronckers Cécile M. , Rossi Silvia , Stiller Charles , Vicini Giulio , Visser Otto , Gatta Gemma ,   EUROCARE-6 Working Group, Hackl Monica , Van Eycken Elisabeth , Van Damme Nancy , Valerianova Zdravka , Sekerija Mario , Gregoriu Ioanna , Demetriou Anna , Dušek Ladislav , Krejici Denisa , Storm Hans , Mägi Margit , Pitkäniemi Janne , Clavel Jacqueline , Poulalhon Claire , Lacour Brigitte , Desandes Emmanuel , Jooste Valerie , Monnereau Alain , Ressing Meike , Katalinic Alexander , Petridou Eleni , Markozannes Georgios , Garami Miklos , Jakab Zsusanna , Birgisson Helgi , Murray Deirdre , Mazzoleni Guido , Vittadello Fabio , Cuccaro Francesco , Galasso Rocco , Sampietro Giuseppe , Rosso Stefano , Gasparotti Cinzia , Maifredi Giovanni , Ferrante Margherita , Torrisi Antonina , Sutera Sardo Antonella , Gambino Maria Letizia , Lanzoni Monica , Ballotari Paola , Giacomazzi Erica , Ferretti Stefano , Caldarella Adele , Manneschi Gianfranco , Sant Milena , Baili Paolo , Berrino Franco , Trama Annalisa , Lillini Roberto , Bernasconi Alice , Bonfarnuzzo Simone , Vener Claudia , Didonè Fabio , Lasalvia Paolo , Buratti Lucia , Tagliabue Giovanna , Serraino Diego , Taborelli Martina , De Angelis Roberta , Demuru Elena , Cerza Francesco , Di Mari Fabrizio , Di Benedetto Corrado , Santaquilani Mariano , Venanzi Serenella , Tallon Marco , Boni Luca , Iacovacci Silvia , Russo Antonio Giampiero , Gervasi Federico , Spagnoli Gianbattista , Cavalieri d'Oro Luca , Fusco Mario , Vitale Maria Francesca , Pinna Pasquale , Mazzucco Walter , Michiara Maria , Chiranda Giorgio , Sacerdote Caelotta , Maule Milena , Cascone Giorgio , Spata Eugenia , Mangone Lucia , Falcini Fabio , Cavallo Rossella , Piras Daniela , Y. Dinaro Ylenia , Castaing Marine , Fanetti Anna Clara , Minerba Sante , Candela Giuseppina , Scuderi Tiziana , Mantovani William , Gentilini Maria Adalgisa , Stracci Fabirizio , Zorzi Manuel , Ferrarini Nicola , Liepina Eliana , Smailyte Giedre , Azzopardi Mirima , Calleja Neville , Johannesen Tom Borge , Didkowska Joanna , Wojciechowska Urszula , Bielska-Lasota Magdalena , Pais Ana , Bento Maria Josè , Calisto Rita , Lourenço Antonio , Mayer Alexandra , Safaei Diba Chakameh , Zadnik Vesna , Zagar Tina , Lopez de Munain Arantza , De-La-Cruz Marta , Garrido Montserrat , Vizcaino Ana , Almela Fernando , Marcos-Gragera Rafael , Sanvisens Arantza , Sanchez Maria Josè , Redondo Daniel , Chirlaque Lopez Maria Dolores , Sanchez-Gil Antonia , Guevara Marcela , Ardanaz Eva , Peris-Bonet Rafael , Galceran Jaume , Carulla Mia , Stevens Saarah , Gavin Anna , Morrison David , Huws Dyfed , Smits Stephanie 
Published: October 2024

ImportanceStudies on the epidemiology of retinoblastoma (RB) could lead to improvement in management.ObjectiveTo estimate the incidence and survival of RB in European children and the occurrence of second primary tumors (other than RB) in these patients.Design, Setting, and ParticipantsThis cohort study used population-based data from 81 cancer registries in 31 European countries adhering to the European Cancer Registries (EUROCARE-6) project. Data collection took place between January 2000 and December 2013. European children aged 0 to 14 years diagnosed with RB were included. Data were analyzed from May to November 2023.ExposuresDiagnosis of RB with International Classification of Diseases for Oncology, Third Edition (ICD-O-3), morphology coded 9510-9514 (retinoblastoma) and malignant behavior (fifth digit of morphology code, 3).Main Outcome and MeasuresAnnual incidence (per million children aged 0-14 years), 5-year survival (%), and the standardized incidence ratio (SIR) of subsequent malignant neoplasms.ResultsThe study included 3262 patients (mean [SD] age, 1.27 [1.63] years; 1706 [52%] male and 1556 [48%] female) from 81 registries. Of these, 3098 patients were considered in trend analysis after excluding registries with incomplete time coverage: 940 in 2000 to 2003, 703 in 2004 to 2006, 744 in 2007 to 2009, and 856 in 2010 to 2013. The estimated overall European incidence rate was 4.0 (95% CI, 3.9-4.1). Rates among countries varied from less than 2 million to greater than 6 million per year. No time trend of incidence was observed in any area. The overall European 5-year survival was 97.8% (95% CI, 95.5-98.9; 3180 cases). Five-year survival was lower in Estonia and Bulgaria (&amp;lt;80%) and 100% in several countries. Twenty-five subsequent malignant neoplasms were recorded during follow-up (up to 14 years), with an SIR of 8.2 and with cases occurring at mean ages between 1.3 and 8.9 years across different sites. An increased risk was found for hematological tumors (SIR, 5) and bone and soft tissue sarcomas (SIR, 29).Conclusions and RelevanceThis study showed RB incidence remained stable at 4.0 per 1 000 000 European children aged 0 to 14 years from 2000 to 2013, but estimates varied among countries and differences in survival across countries persist. These data might be used to monitor RB management and occurrences of second tumors. The findings suggest future registry studies should aim to collect standardized RB stage at diagnosis and treatment to interpret disparities and potentially improve surveillance.