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Incidence and Survival of Patients With Conjunctival Melanoma in Europe

Educational Objective
To estimate incidence and survival of patients with conjunctival melanoma in Europe.
1 Credit CME
Key Points

Question  What are the incidence and survival of patients with conjunctival melanoma in Europe?

Findings  In this cohort study of 724 patients with conjunctival melanoma from population-based data of 41 cancer registries, the overall incidence was 0.46 cases per 1 000 000 person-years. Overall, 5-year relative survival was 83.5% and varied among European geographic areas, with the highest in the UK and Ireland (89.1%) and lowest in Southern Europe (65.7%).

Meaning  Geographical differences in survival indicate room for outcome improvement in Southern, Northern, and Eastern European countries.

Abstract

Importance  Conjunctival melanoma (CM) is a rare ocular tumor. Estimates of incidence and survival of patients with CM are important to researchers and policy makers.

Objective  To estimate incidence and survival of patients with CM in Europe.

Design, Setting, and Participants  This population-based cohort study used data from 41 European cancer registries adhering to the RARECAREnet project. All individuals diagnosed as having malignant CM from January 1995 to December 2007 coded according to the International Classification of Diseases for Oncology, Third Edition codes C69.0 (conjunctiva) and 8720-8780 (melanoma) were included. Analysis began March 2019.

Main Outcomes and Measures  Trend estimates for incidence and for 5-year relative survival (the ratio of the measured survival of patients to the expected survival in the general population for the same country, age, sex, and calendar year). Crude, age-standardized, and bayesian incidence rates were calculated. Five-year relative survival was calculated by the Ederer II method with the cohort and period approach.

Results  A total of 724 patients 15 years or older (512 [70.7%] were 55 years or older; 366 [50.6%] were female) were analyzed with an overall crude incidence of CM (per 1 000 000 person/y) of 0.46 (95% CI, 0.42-0.49). Crude incidence was similar in men and women (0.48; 95% CI, 0.44-0.54 and 0.46; 95% CI, 0.41-0.51, respectively) and increased with age. Age-standardized incidence increased over time only in men and was the highest in Norway and the Netherlands (more than 0.70). Only 1 case in 14 years was estimated to occur in Iceland vs about 20 cases per year in large countries such as France and Germany. Percentage of 5-year survival (83.5 overall; 95% CI, 78.6-87.3) was not different between adult and elderly patients but showed large geographical disparities across European regions (range, 66-89) and improved markedly in male patients (from 76 in 1995-1998 to 86 in 2003-2007, with a difference of 10.2 [95% CI, 1.3-19.2]; P < .05) becoming similar to that of women in the last period.

Conclusions and Relevance  Although these data are only available through 2007 and based on registries not uniformly covering the European population, the study provides the first Europe-wide estimates of the incidence and relative survival of patients with CM using population-based data. Geographical differences in survival indicate room for outcome improvement in Southern, Northern, and Eastern European countries.

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Article Information

Accepted for Publication: February 2, 2020.

Corresponding Author: Mariacristina Parravano, MD, IRCCS-Fondazione Bietti, Via Livenza 3, Rome 00198, Italy (mcparravano@gmail.com).

Published Online: March 26, 2020. doi:10.1001/jamaophthalmol.2020.0531

Author Contributions: Drs Botta and Capocaccia had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

Concept and design: Virgili, Parravano, Gatta, Mallone, Botta.

Acquisition, analysis, or interpretation of data: Virgili, Gatta, Capocaccia, Mazzini, Mallone, Botta.

Drafting of the manuscript: Virgili, Mazzini.

Critical revision of the manuscript for important intellectual content: Virgili, Parravano, Gatta, Capocaccia, Mallone, Botta.

Statistical analysis: Virgili, Capocaccia, Mazzini, Mallone, Botta.

Obtained funding: Gatta.

Administrative, technical, or material support: Virgili, Gatta.

Supervision: Virgili, Parravano, Gatta, Capocaccia, Mallone.

Conflict of Interest Disclosures: Dr Parravano reports personal fees from Allergan, Bayer, and Novartis outside the submitted work. No other disclosures were reported.

Funding/Support: This research was funded by the European Commission through the Consumers, Health, Agriculture and Food Executive Agency (grant 2000111201). We also recognize the contribution of IRCCS Fondazione G. B. Bietti in this study supported by the Italian Ministry of Health and by Fondazione Roma.

Role of the Funder/Sponsor: The funders had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Group Information: The RARECARENet Working Group members are Monika Hackl, MSc (Austrian National Cancer Registry); Elizabeth Van Eycken, MD (Belgian Cancer Registry); Kris Henau (Belgian Cancer Registry); Nadya Dimitrova, PhD (Bulgaria Cancer Registry); Zdravka Valerianova (Bulgaria Cancer Registry); Mario Sekerija (Croatian Cancer Registry); Ladislav Dušek (Czech National Cancer Registry); Margit Mägi (Estonian Cancer Registry); Keiu Paapsi (National Institute for Health Develpoment, Estonia); Nea Malila (Finnish Cancer Registry); Maarit Leinonen, PhD (Finnish Cancer Registry); Michel Velten (Bas Rhin Cancer Registry); Xavier Troussard (Basse Normandie Haematological Malignancies Registry); Veronique Bouvier (Calvados Digestive Tract Registry); Anne-Valérie Guizard (Calvados, Registre Gèneral des Tumeurs); Anne-Marie Bouvier (Côte d’Or Digestive Tract Registry, Burgundy); Patrick Arveux (Côte d’Or Gynaecologic Cancer registry); Marc Maynadié, MD (Côte d’Or Haematological Malignancies Cancer Registry); Anne-Sophie Woronoff (Doubs Cancer Registry); Michel Robaszkiewicz (Finistère Digestive Tract Registry); Isabelle Baldi (Gironde CNS Tumour Registry); Alain Monnereau (Gironde Haematological Malignancies); Brigitte Tretarre (Hérault Cancer Registry); Marc Colonna, MSc (Isère Cancer Registry) Florence Molinié (Loire-Atlantique/Vendée Cancer Registry); Anne Cowppli-Bony (Loire-Atlantique/Vendée Cancer Registry); Simona Bara (Manche Cancer Registry); Claire Schvartz (Marne & Ardennes, Thyroid); Bénédicte Lapôtre-Ledoux (Somme Cancer Registry); Pascale Grosclaude, MSc (Tarn Cancer Registry); Roland Stabenow (Berlin; Brandenburg; Mecklenburg-West Pomerania; Saxony; Saxony-Anhalt; Thüringen Cancer Registry); Sabine Luttmann (Bremen Cancer Registry, Leibniz Institute for Prevention Research and Epidemiology); Alice Nennecke (Hamburg Cancer Registry); Jutta Engel (Munich Cancer Registry); Gabriele Schubert-Fritschle (Munich Cancer Registry); Jan Heidrich (North Rhine-Westphalia Cancer Registry); Bernd Holleczek (Saarland Cancer Registry); Jón Gunnlaugur Jónasson (Icelandic Cancer Registry); Helgi Birgisson (Icelandic Cancer Registry); Kerri Clough-Gorr (National Cancer Registry Ireland); Harry Comber, PhD (National Cancer Registry Ireland); Guido Mazzoleni, MD (Alto Adige Cancer Registry); Adriano Giacomin, MD (Biella Cancer Registry); Antonella Sutera Sardo, MD (Catanzaro Cancer Registry); Alessandro Barchielli, MD (Firenze-Prato Cancer Registry); Diego Serraino, MD (Friuli Venezia Giulia, CRO Aviano National Cancer Institute); Paolo Collarile (Friuli Venezia Giulia, CRO Aviano National Cancer Institute); Roberta De Angelis, MSc (Istituto Superiore di Sanità, Rome); Silvia Francisci, PhD (Istituto Superiore di Sanità, Rome); Arnold Knijn (Istituto Superiore di Sanità, Rome); Sandra Mallone, MSc (Istituto Superiore di Sanità, Rome); Daniela Pierannunzio, MSc (Istituto Superiore di Sanità, Rome); Andrea Tavilla, MSc (Istituto Superiore di Sanità, Rome); Silvia Rossi, MSc (Istituto Superiore di Sanità, Rome); Mariano Santaquilani, IT (Istituto Superiore di Sanità, Rome); Fabio Pannozzo, MD (Latina Cancer Registry); Paolo Ricci, MD (Mantova Cancer Registry); Mariangela Autelitano, MD (Milano Cancer Registry); Antonio Giampiero Russo, MD (Milano Cancer Registry); Gianbattista Spagnoli (Modena Cancer Registry); Mario Fusco, MD (Napoli 3 South Cancer Registry); Mario Usala, (Nuoro Cancer Registry); Francesco Vitale, MD (Palermo Cancer Registry); Maria Michiara, MD (Parma Cancer Registry); Rosario Tumino, MD (Ragusa Cancer Registry); Lucia Mangone, MD (Reggio Emilia Cancer Registry); Fabio Falcini, MD (Romagna Cancer Registry, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori [IRST], IRCCS, Meldola, Forlì, Italy-Azienda Usl della Romagna, Forlì, Italy); Rosa Vattiato, IT (Romagna Cancer Registry); Stefano Ferretti, MD (Ferrara Cancer Registry); Rosa Angela Filiberti (RTR Liguria, IRCCS AOU SM-IST); Enza Marani, MD (RTR Liguria, IRCCS AOU SM-IST); Arturo Iannelli, IT (Salerno Cancer Registry); Anna Luisa Caiazzo (Salerno Cancer Registry); Rosaria Cesaraccio (Sassari Cancer Registry); Silvano Piffer, MD (Servizio Epidemiologia Clinica e Valutativa, Trento); Maria Gentilini, MD (Servizio Epidemiologia Clinica e Valutativa, Trento); Anselmo Madeddu, MD (Siracusa Cancer Registry); Antonino Ziino Colanino (Siracusa Cancer Registry); Sergio Maspero (Sondrio Cancer Registry); Pina Candela, MD (Trapani Cancer Registry); Fabrizio Stracci, MD (Umbria Cancer Registry); Giovanna Tagliabue, MD (Varese Province Cancer Registry, Fondazione IRCCS Istituto Nazionale dei Tumori); Massimo Rugge, MD (Azienda Zero, SER-Registro Tumori del Veneto); Paolo Baili, MSc (Fondazione IRCCS, National Cancer Institute, Milan); Laura Botta, MSc (Fondazione IRCCS, National Cancer Institute, Milan); Riccardo Capocaccia, MSc (Fondazione IRCCS, National Cancer Institute, Milan); Roberto Foschi, MSc (Fondazione IRCCS, National Cancer Institute, Milan); Gemma Gatta, MD (Fondazione IRCCS, National Cancer Institute, Milan); Pamela Minicozzi, MSc (Fondazione IRCCS, National Cancer Institute, Milan); Annalisa Trama, PhD (Fondazione IRCCS, National Cancer Institute, Milan); Carmen Tereanu, MD (Fondazione IRCCS, National Cancer Institute, Milan); Milena Sant, MD (Fondazione IRCCS, National Cancer Institute, Milan); Santa Pildava (Latvian Cancer Registry); Giedre Smailyte (Lithuanian Cancer Registry); Neville Calleja (Malta National Cancer Registry, Health Information and Research); Dominic Agius (Malta National Cancer Registry, Health Information and Research); Tom Børge Johannesen (Norwegian Cancer Registry); Jadwiga Rachtan (Cracow Cancer Registry); Stanisław Góźdź (Kielce Cancer Registry); Jerzy Błaszczyk (Lower Silesia Cancer Registry, Wroclaw); Kamila Kępska (Lower Silesia Cancer Registry, Wroclaw); Gonçalo Forjaz de Lacerda (Açores Cancer Registry); Maria José Bento (Northern Portugal Cancer Registry); Ana Miranda (Southern Portugal Cancer Registry); Chakameh Safaei Diba (Slovakian National Cancer Registry); Maja Primic-Žakelj, MD (Cancer Registry of Republic of Slovenia); Tina Žagar (Cancer Registry of Republic of Slovenia); Enrique Almar (Albacete Castilla-La Mancha Cancer Registry); Nerea Larrañaga (Basque Country Cancer Registry); Arantza Lopez de Munain (Basque Country Cancer Registry); Ana Torrella-Ramos (Castellón-Valencia [breast] Cancer Registry); José María Díaz García (Cuenca Cancer Registry); Rafael Marcos-Gragera (Girona Cancer Registry); Maria Josè Sanchez (Granada Cancer Registry, CIBERESP, ibs.Granada); Mª Dolores Chirlaque (Murcia Cancer Registry, CIBERESP, IMIB-Arrixaca, Murcia University); Diego Salmerón (Murcia Cancer Registry, CIBERESP, IMIB-Arrixaca, Murcia University); Eva Ardanaz, PhD (Navarra Cancer Registry, CIBERESP); Marcela Guevara (Navarra Cancer Registry, CIBERESP); Jaume Galceran (Tarragona Cancer Registry); Marià Carulla (Tarragona Cancer Registry); Mohsen Mousavi (Basel Cancer Registry); Christine Bouchardy (Geneva Cancer Registry); Silvia M. Ess (Grisons-Glarus, St Gallen Cancer Registry); Andrea Bordoni, MD (Ticino Cancer Registry); Isabelle Konzelmann (Valais Cancer Registry); Jem Rashbass (Public Health England); Anna Gavin (Northern Ireland Cancer Registry); David H Brewster (Scotland Cancer Registry); Dyfed Wyn Huws (Welsh Cancer Intelligence and Surveillance Unit); Otto Visser, PhD (The Netherlands Cancer Registry); Jan Maarten van der Zwan, PhD (The Netherlands Cancer Registry); Sabine Siesling, PhD (The Netherlands Cancer Registry); Ellen Benhamou, PhD (Institut de Cancérologie Gustave Roussy, Villejuif, France); Renèe Otter, PhD (Scientific Institute of Public Health, Brussels).

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