INTRODUCTION: Advanced intraocular retinoblastoma can be cured by enucleation, but spread of retinoblastoma cells beyond the natural limits of the eye is related to a high mortality. Adjuvant therapy after enucleation has been shown to prevent metastasis in children with risk factors for extraocular retinoblastoma. However, histological criteria and adjuvant treatment regimens vary and there is no unifying consensus on the optimal choice of treatment. METHOD: Data on guidelines for adjuvant treatment in European retinoblastoma referral centres were collected in an online survey among all members of the European Retinoblastoma Group (EURbG) network. Extended information was gathered via personal email communication. RESULTS: Data were collected from 26 centres in 17 countries. Guidelines for adjuvant treatment were in place at 92.3% of retinoblastoma centres. There was a consensus on indication for and intensity of adjuvant treatment among more than 80% of all centres. The majority of centres use no adjuvant treatment for isolated focal choroidal invasion or prelaminar optic nerve invasion. Patients with massive choroidal invasion or postlaminar optic nerve invasion receive adjuvant chemotherapy, while microscopic invasion of the resection margin of the optic nerve or extension through the sclera are treated with combined chemo- and radiotherapy. CONCLUSION: Indications and adjuvant treatment regimens in European retinoblastoma referral centres are similar but not uniform. Further biomarkers in addition to histopathological risk factors could improve treatment stratification. The high consensus in European centres is an excellent foundation for a common European study with prospective validation of new biomarkers.

Amsterdam UMC Location VU University Medical Centre Amsterdam The Netherlands

Birmingham Children's Hospital Birmingham England

Centre for Pediatric Oncology and Hematology Vilnius University Vilnius Lithuania

Centro Hospitalar e Universitário de Coimbra University of Coimbra Coimbra Portugal

Charles University Prague 2nd Faculty of Medicine and University Hospital Motol Prague Czech Republic

Children's Hospital University of Tuebingen Tuebingen Germany

Department of Ophthalmology Amsterdam UMC Vrije Universiteit Amsterdam Cancer Center Amsterdam Amsterdam The Netherlands

Department of Ophthalmology Semmelweis University Budapest Hungary

Department of Ophthalmology University Duisburg Essen University Hospital Essen Essen Germany

Department of Particle Therapy University Hospital Essen West German Proton Therapy Centre Essen Essen Germany

Department of Pediatric Hematology and Oncology University Duisburg Essen University Hospital Essen Essen Germany

Department of Pediatric Hematology and Oncology University Hospital CHUV University of Lausanne Lausanne Switzerland

Department of Pediatric Hematology Oncology IRCCS Ospedale Pediatrico Bambino Gesù Rome Italy

Department of Pediatric Oncology and Hematology Charité Universitätsmedizin Berlin Berlin Germany

Department of Pediatric Oncology and Hematology University Children's Hospital of Krakow Krakow Poland

Division of Pediatric Hematology Oncology Medical University of Graz Graz Austria

East Tallinn Central Hospital Tallinn Estonia

Filatov Eye Institute Odessa Odessa Ukraine

Gazi University School of Medicine Ankara Turkey

German Consortium for Translational Cancer Research Heidelberg Germany

Hacettepe University Hospital Ankara Turkey

Hospital La Paz Madrid Spain

Hospital Sant Joan de Deu Barcelona Spain

Hospital Virgen Macarena Sevilla Spain

Institut Curie PSL Research University and University of Paris Paris France

Jules Gonin Eye Hospital Fondation Asile des Aveugles University of Lausanne Lausanne Switzerland

Ocular Oncology Siena University Hospital Siena Italy

Pediatric Hematology and Oncology University Hospital Vall d'Hebron Barcelona Spain

Pediatric Hematology Oncology and Stem Cell Transplant Division Padua University Hospital Padua Italy

Pediatric Oncology Sheba Medical Center Tel Aviv University Tel Aviv Israel

Royal London Hospital and Great Ormond Street Hospital London England

The Children's Memorial Health Institute Warsaw Poland

The Goldschleger eye institute Sheba Medical Center Tel Aviv University Tel Aviv Israel

University Hospital Brno and St Anna University Hospital ICRC Masaryk University Brno Czech Republic

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