title: The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  Retrospective, multicenter observational study.
  
  
  METHODS
  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.
  
  
  METHODS
  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: Ocular Oncology Service, Department of Ophthalmology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland. Electronic address: ranaa.aljamal@hus.fi.
authors: Rana'a T
authors: RT
authors: Al-Jamal
authors: Department of Ophthalmology, Institute Curie, Paris, France.
authors: Nathalie
authors: N
authors: Cassoux
authors: Department of Ophthalmology, Institute Curie, Paris, France.
authors: Laurence
authors: L
authors: Desjardins
authors: Ocular Oncology Service, Royal Liverpool University Hospital, Liverpool, United Kingdom.
authors: Bertil
authors: B
authors: Damato
authors: Ocular Oncology Service, Royal Liverpool University Hospital, Liverpool, United Kingdom.
authors: Lazaros
authors: L
authors: Konstantinidis
authors: Ocular Oncology Service, Royal Liverpool University Hospital, Liverpool, United Kingdom; Department of Cellular Pathology, Royal Liverpool University Hospital, Liverpool, United Kingdom.
authors: Sarah E
authors: SE
authors: Coupland
authors: Ocular Oncology Service, Royal Liverpool University Hospital, Liverpool, United Kingdom.
authors: Heinrich
authors: H
authors: Heimann
authors: Department of Ophthalmology, University of Lausanne, Jules-Gonin Eye Hospital, Fondation Asile des Aveugles (FAA), Lausanne, Switzerland.
authors: Aleksandra
authors: A
authors: Petrovic
authors: Department of Ophthalmology, University of Lausanne, Jules-Gonin Eye Hospital, Fondation Asile des Aveugles (FAA), Lausanne, Switzerland.
authors: Leonidas
authors: L
authors: Zografos
authors: Department of Ophthalmology, University of Lausanne, Jules-Gonin Eye Hospital, Fondation Asile des Aveugles (FAA), Lausanne, Switzerland.
authors: Ann
authors: A
authors: Schalenbourg
authors: Department of Ocular Oncology, Princess Margaret Hospital, University of Toronto, Toronto, Canada.
authors: Juan P
authors: JP
authors: Velazquez-Martin
authors: Department of Ocular Oncology, Princess Margaret Hospital, University of Toronto, Toronto, Canada.
authors: Hatem
authors: H
authors: Krema
authors: Department of Ophthalmology and Ocular Oncology, Jagiellonian University, Collegium Medicum, Krakow, Poland.
authors: Anna
authors: A
authors: Bogdali
authors: Department of Ophthalmology and Ocular Oncology, Jagiellonian University, Collegium Medicum, Krakow, Poland.
authors: Anna
authors: A
authors: Markiewicz
authors: Department of Ophthalmology and Ocular Oncology, Jagiellonian University, Collegium Medicum, Krakow, Poland.
authors: Bozena
authors: B
authors: Romanowska-Dixon
authors: Department of Ophthalmology, Faculty of Medicine, University Duisburg-Essen, Essen, Germany.
authors: Claudia H D
authors: CH
authors: Metz
authors: Department of Ophthalmology, Faculty of Medicine, University Duisburg-Essen, Essen, Germany.
authors: Eva
authors: E
authors: Biewald
authors: Department of Ophthalmology, Faculty of Medicine, University Duisburg-Essen, Essen, Germany.
authors: Norbert
authors: N
authors: Bornfeld
authors: Ocular Oncology Service, Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey.
authors: Hayyam
authors: H
authors: Kiratli
authors: Department of Ophthalmology, Leiden University Medical Center, Leiden, The Netherlands.
authors: Inge H G
authors: IH
authors: Bronkhorst
authors: Department of Ophthalmology, Leiden University Medical Center, Leiden, The Netherlands.
authors: Martine J
authors: MJ
authors: Jager
authors: Department of Ophthalmology, Leiden University Medical Center, Leiden, The Netherlands.
authors: Marina
authors: M
authors: Marinkovic
authors: Department of Ophthalmic Oncology, St. Erik's Eye Hospital, Stockholm, Sweden.
authors: Maria
authors: M
authors: Fili
authors: Department of Ophthalmic Oncology, St. Erik's Eye Hospital, Stockholm, Sweden.
authors: Stefan
authors: S
authors: Seregard
authors: Department of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors: Shahar
authors: S
authors: Frenkel
authors: Department of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors: Jacob
authors: J
authors: Pe'er
authors: Department of Ophthalmology, Royal Hallamshire Hospital, Sheffield, United Kingdom.
authors: Sachin M
authors: SM
authors: Salvi
authors: Department of Ophthalmology, Royal Hallamshire Hospital, Sheffield, United Kingdom.
authors: Ian G
authors: IG
authors: Rennie
authors: Department of Ophthalmology, Poznán University of Medical Sciences, Poznán, Poland.
authors: Iwona
authors: I
authors: Rospond-Kubiak
authors: Department of Ophthalmology, Poznán University of Medical Sciences, Poznán, Poland.
authors: Jaroslaw
authors: J
authors: Kociecki
authors: Department of Ophthalmology, Copenhagen University Hospital Glostrup, Copenhagen, Denmark.
authors: Jens Folke
authors: JF
authors: Kiilgaard
authors: Department of Ophthalmology, Copenhagen University Hospital Glostrup, Copenhagen, Denmark.
authors: Steffen
authors: S
authors: Heegaard
authors: Ocular Oncology Service, St Bartholomew's and Moorfields Eye Hospital, London, United Kingdom.
authors: Victoria M L
authors: VM
authors: Cohen
authors: Ocular Oncology Service, St Bartholomew's and Moorfields Eye Hospital, London, United Kingdom.
authors: Mandeep S
authors: MS
authors: Sagoo
authors: Department of Ophthalmic Oncology and Radiology, Helmholtz Institute, Moscow, Russia.
authors: Anush
authors: A
authors: Amiryan
authors: Department of Ophthalmic Oncology and Radiology, Helmholtz Institute, Moscow, Russia.
authors: Svetlana
authors: S
authors: Saakyan
authors: Department of Ophthalmology, Oslo University Hospital-HF and University of Oslo, Oslo, Norway.
authors: Nils
authors: N
authors: Eide
authors: Department of Clinical Medicine, Section of Ophthalmology, University of Bergen, Bergen, Norway.
authors: Jørgen
authors: J
authors: Krohn
authors: Department of Ophthalmology, University of Padova, Padova, Italy.
authors: Edoardo
authors: E
authors: Midena
authors: G. B. Bietti Foundation, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), Ocular Oncology and Toxicology Research Unit, Rome, Italy.
authors: Raffaele
authors: R
authors: Parrozzani
authors: Department of Ophthalmology, Croix-Rousse Hospital, Lyon, France.
authors: Jean-Daniel
authors: JD
authors: Grange
authors: Department of Ophthalmology, Erasmus University Medical Center, Rotterdam, The Netherlands.
authors: Emine
authors: E
authors: Kilic
authors: Department of Ophthalmology, Catholic University of Rome, Rome, Italy.
authors: Maria Antonietta
authors: MA
authors: Blasi
authors: Department of Ophthalmology, Ocular Oncology Unit, Valladolid University Hospital, Valladolid, Spain.
authors: Maria Antonia
authors: MA
authors: Saornil
authors: Ocular Oncology Service, Department of Ophthalmology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
authors: Tero T
authors: TT
authors: Kivelä
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002830:Choroid Neoplasms / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy; D002924:Ciliary Body / Q000473:pathology*; D005060:Europe / Q000453:epidemiology; D015353:Eye Enucleation; D005260:Female; D006306:Health Surveys; D006801:Humans; D008297:Male; D008495:Medical Oncology / Q000458:organization & administration; D008545:Melanoma / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis; D013508:Ophthalmologic Surgical Procedures; D009885:Ophthalmology / Q000458:organization & administration; D010778:Photochemotherapy; D011878:Radiotherapy; D012189:Retrospective Studies; D015996:Survival Rate; D014604:Uveal Neoplasms / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy; D055815:Young Adult; D000098943:Uveal Melanoma