Rana'a T Al-Jamal1, Nathalie Cassoux2, Laurence Desjardins2, Bertil Damato3, Lazaros Konstantinidis3, Sarah E Coupland4, Heinrich Heimann3, Aleksandra Petrovic5, Leonidas Zografos5, Ann Schalenbourg5, Juan P Velazquez-Martin6, Hatem Krema6, Anna Bogdali7, Anna Markiewicz7, Bozena Romanowska-Dixon7, Claudia H D Metz8, Eva Biewald8, Norbert Bornfeld8, Hayyam Kiratli9, Inge H G Bronkhorst10, Martine J Jager10, Marina Marinkovic10, Maria Fili11, Stefan Seregard11, Shahar Frenkel12, Jacob Pe'er12, Sachin M Salvi13, Ian G Rennie13, Iwona Rospond-Kubiak14, Jaroslaw Kociecki14, Jens Folke Kiilgaard15, Steffen Heegaard15, Victoria M L Cohen16, Mandeep S Sagoo16, Anush Amiryan17, Svetlana Saakyan17, Nils Eide18, Jørgen Krohn19, Edoardo Midena20, Raffaele Parrozzani21, Jean-Daniel Grange22, Emine Kilic23, Maria Antonietta Blasi24, Maria Antonia Saornil25, Tero T Kivelä26. 1. Ocular Oncology Service, Department of Ophthalmology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland. Electronic address: ranaa.aljamal@hus.fi. 2. Department of Ophthalmology, Institute Curie, Paris, France. 3. Ocular Oncology Service, Royal Liverpool University Hospital, Liverpool, United Kingdom. 4. Ocular Oncology Service, Royal Liverpool University Hospital, Liverpool, United Kingdom; Department of Cellular Pathology, Royal Liverpool University Hospital, Liverpool, United Kingdom. 5. Department of Ophthalmology, University of Lausanne, Jules-Gonin Eye Hospital, Fondation Asile des Aveugles (FAA), Lausanne, Switzerland. 6. Department of Ocular Oncology, Princess Margaret Hospital, University of Toronto, Toronto, Canada. 7. Department of Ophthalmology and Ocular Oncology, Jagiellonian University, Collegium Medicum, Krakow, Poland. 8. Department of Ophthalmology, Faculty of Medicine, University Duisburg-Essen, Essen, Germany. 9. Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey. 10. Department of Ophthalmology, Leiden University Medical Center, Leiden, The Netherlands. 11. Department of Ophthalmic Oncology, St. Erik's Eye Hospital, Stockholm, Sweden. 12. Department of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel. 13. Department of Ophthalmology, Royal Hallamshire Hospital, Sheffield, United Kingdom. 14. Department of Ophthalmology, Poznán University of Medical Sciences, Poznán, Poland. 15. Department of Ophthalmology, Copenhagen University Hospital Glostrup, Copenhagen, Denmark. 16. Ocular Oncology Service, St Bartholomew's and Moorfields Eye Hospital, London, United Kingdom. 17. Department of Ophthalmic Oncology and Radiology, Helmholtz Institute, Moscow, Russia. 18. Department of Ophthalmology, Oslo University Hospital-HF and University of Oslo, Oslo, Norway. 19. Department of Clinical Medicine, Section of Ophthalmology, University of Bergen, Bergen, Norway. 20. Department of Ophthalmology, University of Padova, Padova, Italy. 21. G. B. Bietti Foundation, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Ocular Oncology and Toxicology Research Unit, Rome, Italy. 22. Department of Ophthalmology, Croix-Rousse Hospital, Lyon, France. 23. Department of Ophthalmology, Erasmus University Medical Center, Rotterdam, The Netherlands. 24. Department of Ophthalmology, Catholic University of Rome, Rome, Italy. 25. Department of Ophthalmology, Ocular Oncology Unit, Valladolid University Hospital, Valladolid, Spain. 26. Ocular Oncology Service, Department of Ophthalmology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Abstract
PURPOSE: To collect comprehensive data on choroidal and ciliary body melanoma (CCBM) in children and to validate hypotheses regarding pediatric CCBM: children younger than 18 years, males, and those without ciliary body involvement (CBI) have more favorable survival prognosis than young adults 18 to 24 years of age, females, and those with CBI. DESIGN: Retrospective, multicenter observational study. PARTICIPANTS: Two hundred ninety-nine patients from 24 ocular oncology centers, of whom 114 were children (median age, 15.1 years; range, 2.7-17.9 years) and 185 were young adults. METHODS: Data were entered through a secure website and were reviewed centrally. Survival was analyzed using Kaplan-Meier analysis and Cox proportional hazards regression. MAIN OUTCOME MEASURES: Proportion of females, tumor-node-metastasis (TNM) stage, cell type, and melanoma-related mortality. RESULTS: Cumulative frequency of having CCBM diagnosed increased steadily by 0.8% per year of age between 5 and 10 years of age and, after a 6-year transition period, by 8.8% per year from age 17 years onward. Of children and young adults, 57% and 63% were female, respectively, which exceeded the expected 51% among young adults. Cell type, known for 35% of tumors, and TNM stage (I in 22% and 21%, II in 49% and 52%, III in 30% and 28%, respectively) were comparable for children and young adults. Melanoma-related survival was 97% and 90% at 5 years and 92% and 80% at 10 years for children compared with young adults, respectively (P = 0.013). Males tended to have a more favorable survival than females among children (100% vs. 85% at 10 years; P = 0.058). Increasing TNM stage was associated with poorer survival (stages I, II, and III: 100% vs. 86% vs. 76%, respectively; P = 0.0011). By multivariate analysis, being a young adult (adjusted hazard rate [HR], 2.57), a higher TNM stage (HR, 2.88 and 8.38 for stages II and III, respectively), and female gender (HR, 2.38) independently predicted less favorable survival. Ciliary body involvement and cell type were not associated with survival. CONCLUSIONS: This study confirms that children with CCBM have a more favorable survival than young adults 18 to 25 years of age, adjusting for TNM stage and gender. The association between gender and survival varies between age groups.
PURPOSE: To collect comprehensive data on choroidal and ciliary body melanoma (CCBM) in children and to validate hypotheses regarding pediatric CCBM: children younger than 18 years, males, and those without ciliary body involvement (CBI) have more favorable survival prognosis than young adults 18 to 24 years of age, females, and those with CBI. DESIGN: Retrospective, multicenter observational study. PARTICIPANTS: Two hundred ninety-nine patients from 24 ocular oncology centers, of whom 114 were children (median age, 15.1 years; range, 2.7-17.9 years) and 185 were young adults. METHODS: Data were entered through a secure website and were reviewed centrally. Survival was analyzed using Kaplan-Meier analysis and Cox proportional hazards regression. MAIN OUTCOME MEASURES: Proportion of females, tumor-node-metastasis (TNM) stage, cell type, and melanoma-related mortality. RESULTS: Cumulative frequency of having CCBM diagnosed increased steadily by 0.8% per year of age between 5 and 10 years of age and, after a 6-year transition period, by 8.8% per year from age 17 years onward. Of children and young adults, 57% and 63% were female, respectively, which exceeded the expected 51% among young adults. Cell type, known for 35% of tumors, and TNM stage (I in 22% and 21%, II in 49% and 52%, III in 30% and 28%, respectively) were comparable for children and young adults. Melanoma-related survival was 97% and 90% at 5 years and 92% and 80% at 10 years for children compared with young adults, respectively (P = 0.013). Males tended to have a more favorable survival than females among children (100% vs. 85% at 10 years; P = 0.058). Increasing TNM stage was associated with poorer survival (stages I, II, and III: 100% vs. 86% vs. 76%, respectively; P = 0.0011). By multivariate analysis, being a young adult (adjusted hazard rate [HR], 2.57), a higher TNM stage (HR, 2.88 and 8.38 for stages II and III, respectively), and female gender (HR, 2.38) independently predicted less favorable survival. Ciliary body involvement and cell type were not associated with survival. CONCLUSIONS: This study confirms that children with CCBM have a more favorable survival than young adults 18 to 25 years of age, adjusting for TNM stage and gender. The association between gender and survival varies between age groups.
Authors: Mohamed H Abdel-Rahman; Klarke M Sample; Robert Pilarski; Tomas Walsh; Timothy Grosel; Daniel Kinnamon; Getachew Boru; James B Massengill; Lynn Schoenfield; Ben Kelly; David Gordon; Peter Johansson; Meghan J DeBenedictis; Arun Singh; Silvia Casadei; Frederick H Davidorf; Peter White; Andrew W Stacey; James Scarth; Ellie Fewings; Marc Tischkowitz; Mary-Claire King; Nicholas K Hayward; Colleen M Cebulla Journal: Ophthalmology Date: 2019-11-18 Impact factor: 12.079
Authors: Christine D M Roelofsen; Annemijn P A Wierenga; Sjoerd van Duinen; Robert M Verdijk; Jaco Bleeker; Marina Marinkovic; Gregorius P M Luyten; Martine J Jager Journal: Ocul Oncol Pathol Date: 2020-12-15
Authors: Anna Markiewicz; Anna A Brożyna; Ewa Podgórska; Martyna Elas; Krystyna Urbańska; Anton M Jetten; Andrzej T Slominski; Wojciech Jóźwicki; Jolanta Orłowska-Heitzman; Grzegorz Dyduch; Bożena Romanowska-Dixon Journal: Sci Rep Date: 2019-06-24 Impact factor: 4.379
Authors: Natasha M van Poppelen; Daniël P de Bruyn; Tolga Bicer; Rob Verdijk; Nicole Naus; Hanneke Mensink; Dion Paridaens; Annelies de Klein; Erwin Brosens; Emine Kiliҫ Journal: Int J Mol Sci Date: 2020-12-30 Impact factor: 5.923
Authors: Laurien E Houtzagers; Annemijn P A Wierenga; Aleid A M Ruys; Gregorius P M Luyten; Martine J Jager Journal: Int J Mol Sci Date: 2020-09-28 Impact factor: 5.923