TY - JOUR
T1 - The Pediatric Choroidal and Ciliary Body Melanoma Study
T2 - A Survey by the European Ophthalmic Oncology Group
AU - Al-Jamal, Rana'a T
AU - Cassoux, Nathalie
AU - Desjardins, Laurence
AU - Damato, Bertil
AU - Konstantinidis, Lazaros
AU - Coupland, Sarah E
AU - Heimann, Heinrich
AU - Petrovic, Aleksandra
AU - Zografos, Leonidas
AU - Schalenbourg, Ann
AU - Velazquez-Martin, Juan P
AU - Krema, Hatem
AU - Bogdali, Anna
AU - Markiewicz, Anna
AU - Romanowska-Dixon, Bozena
AU - Metz, Claudia H D
AU - Biewald, Eva
AU - Bornfeld, Norbert
AU - Kiratli, Hayyam
AU - Bronkhorst, Inge H G
AU - Jager, Martine J
AU - Marinkovic, Marina
AU - Fili, Maria
AU - Seregard, Stefan
AU - Frenkel, Shahar
AU - Pe'er, Jacob
AU - Salvi, Sachin M
AU - Rennie, Ian G
AU - Rospond-Kubiak, Iwona
AU - Kociecki, Jaroslaw
AU - Kiilgaard, Jens Folke
AU - Heegaard, Steffen
AU - Cohen, Victoria M L
AU - Sagoo, Mandeep S
AU - Amiryan, Anush
AU - Saakyan, Svetlana
AU - Eide, Nils
AU - Krohn, Jørgen
AU - Midena, Edoardo
AU - Parrozzani, Raffaele
AU - Grange, Jean-Daniel
AU - Kilic, Emine
AU - Blasi, Maria Antonietta
AU - Saornil, Maria Antonia
AU - Kivelä, Tero T
N1 - Copyright © 2016 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.
PY - 2016/4/1
Y1 - 2016/4/1
N2 - 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.
AB - 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.
KW - Adolescent
KW - Child
KW - Child, Preschool
KW - Choroid Neoplasms
KW - Ciliary Body
KW - Europe
KW - Eye Enucleation
KW - Female
KW - Health Surveys
KW - Humans
KW - Male
KW - Medical Oncology
KW - Melanoma
KW - Neoplasm Recurrence, Local
KW - Ophthalmologic Surgical Procedures
KW - Ophthalmology
KW - Photochemotherapy
KW - Radiotherapy
KW - Retrospective Studies
KW - Survival Rate
KW - Uveal Neoplasms
KW - Young Adult
KW - Journal Article
KW - Multicenter Study
KW - Observational Study
KW - Research Support, Non-U.S. Gov't
U2 - 10.1016/j.ophtha.2015.12.024
DO - 10.1016/j.ophtha.2015.12.024
M3 - Journal article
C2 - 26854035
SN - 0161-6420
VL - 123
SP - 898
EP - 907
JO - Ophthalmology
JF - Ophthalmology
IS - 4
ER -