The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group

Rana'a T Al-Jamal; Nathalie Cassoux; Laurence Desjardins; Bertil Damato; Lazaros Konstantinidis; Sarah E Coupland; Heinrich Heimann; Aleksandra Petrovic; Leonidas Zografos; Ann Schalenbourg; Juan P Velazquez-Martin; Hatem Krema; Anna Bogdali; Anna Markiewicz; Bozena Romanowska-Dixon; Claudia H D Metz; Eva Biewald; Norbert Bornfeld; Hayyam Kiratli; Inge H G Bronkhorst; Martine J Jager; Marina Marinkovic; Maria Fili; Stefan Seregard; Shahar Frenkel; Jacob Pe'er; Sachin M Salvi; Ian G Rennie; Iwona Rospond-Kubiak; Jaroslaw Kociecki; Jens Folke Kiilgaard; Steffen Heegaard; Victoria M L Cohen; Mandeep S Sagoo; Anush Amiryan; Svetlana Saakyan; Nils Eide; Jørgen Krohn; Edoardo Midena; Raffaele Parrozzani; Jean-Daniel Grange; Emine Kilic; Maria Antonietta Blasi; Maria Antonia Saornil; Tero T Kivelä

doi:10.1016/j.ophtha.2015.12.024

The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group

Rana'a T Al-Jamal, Nathalie Cassoux, Laurence Desjardins, Bertil Damato, Lazaros Konstantinidis, Sarah E Coupland, Heinrich Heimann, Aleksandra Petrovic, Leonidas Zografos, Ann Schalenbourg, Juan P Velazquez-Martin, Hatem Krema, Anna Bogdali, Anna Markiewicz, Bozena Romanowska-Dixon, Claudia H D Metz, Eva Biewald, Norbert Bornfeld, Hayyam Kiratli, Inge H G BronkhorstMartine J Jager, Marina Marinkovic, Maria Fili, Stefan Seregard, Shahar Frenkel, Jacob Pe'er, Sachin M Salvi, Ian G Rennie, Iwona Rospond-Kubiak, Jaroslaw Kociecki, Jens Folke Kiilgaard, Steffen Heegaard, Victoria M L Cohen, Mandeep S Sagoo, Anush Amiryan, Svetlana Saakyan, Nils Eide, Jørgen Krohn, Edoardo Midena, Raffaele Parrozzani, Jean-Daniel Grange, Emine Kilic, Maria Antonietta Blasi, Maria Antonia Saornil, Tero T Kivelä

Department of Clinical Medicine

25 Citations (Scopus)

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.

Original language	English
Journal	Ophthalmology
Volume	123
Issue number	4
Pages (from-to)	898-907
Number of pages	10
ISSN	0161-6420
DOIs	https://doi.org/10.1016/j.ophtha.2015.12.024
Publication status	Published - 1 Apr 2016

Keywords

Adolescent
Child
Child, Preschool
Choroid Neoplasms
Ciliary Body
Europe
Eye Enucleation
Female
Health Surveys
Humans
Male
Medical Oncology
Melanoma
Neoplasm Recurrence, Local
Ophthalmologic Surgical Procedures
Ophthalmology
Photochemotherapy
Radiotherapy
Retrospective Studies
Survival Rate
Uveal Neoplasms
Young Adult
Journal Article
Multicenter Study
Observational Study
Research Support, Non-U.S. Gov't

Access to Document

10.1016/j.ophtha.2015.12.024

Cite this

Al-Jamal, R. T., Cassoux, N., Desjardins, L., Damato, B., Konstantinidis, L., Coupland, S. E., Heimann, H., Petrovic, A., Zografos, L., Schalenbourg, A., Velazquez-Martin, J. P., Krema, H., Bogdali, A., Markiewicz, A., Romanowska-Dixon, B., Metz, C. H. D., Biewald, E., Bornfeld, N., Kiratli, H., ... Kivelä, T. T. (2016). The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group. Ophthalmology, 123(4), 898-907. https://doi.org/10.1016/j.ophtha.2015.12.024

Al-Jamal, RT, Cassoux, N, Desjardins, L, Damato, B, Konstantinidis, L, Coupland, SE, Heimann, H, Petrovic, A, Zografos, L, Schalenbourg, A, Velazquez-Martin, JP, Krema, H, Bogdali, A, Markiewicz, A, Romanowska-Dixon, B, Metz, CHD, Biewald, E, Bornfeld, N, Kiratli, H, Bronkhorst, IHG, Jager, MJ, Marinkovic, M, Fili, M, Seregard, S, Frenkel, S, Pe'er, J, Salvi, SM, Rennie, IG, Rospond-Kubiak, I, Kociecki, J, Kiilgaard, JF , Heegaard, S, Cohen, VML, Sagoo, MS, Amiryan, A, Saakyan, S, Eide, N, Krohn, J, Midena, E, Parrozzani, R, Grange, J-D, Kilic, E, Blasi, MA, Saornil, MA & Kivelä, TT 2016, 'The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group', Ophthalmology, vol. 123, no. 4, pp. 898-907. https://doi.org/10.1016/j.ophtha.2015.12.024

@article{2d0050a1cc3a4adf973bdde1810b270a,

title = "The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group",

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.",

keywords = "Adolescent, Child, Child, Preschool, Choroid Neoplasms, Ciliary Body, Europe, Eye Enucleation, Female, Health Surveys, Humans, Male, Medical Oncology, Melanoma, Neoplasm Recurrence, Local, Ophthalmologic Surgical Procedures, Ophthalmology, Photochemotherapy, Radiotherapy, Retrospective Studies, Survival Rate, Uveal Neoplasms, Young Adult, Journal Article, Multicenter Study, Observational Study, Research Support, Non-U.S. Gov't",

author = "Al-Jamal, {Rana'a T} and Nathalie Cassoux and Laurence Desjardins and Bertil Damato and Lazaros Konstantinidis and Coupland, {Sarah E} and Heinrich Heimann and Aleksandra Petrovic and Leonidas Zografos and Ann Schalenbourg and Velazquez-Martin, {Juan P} and Hatem Krema and Anna Bogdali and Anna Markiewicz and Bozena Romanowska-Dixon and Metz, {Claudia H D} and Eva Biewald and Norbert Bornfeld and Hayyam Kiratli and Bronkhorst, {Inge H G} and Jager, {Martine J} and Marina Marinkovic and Maria Fili and Stefan Seregard and Shahar Frenkel and Jacob Pe'er and Salvi, {Sachin M} and Rennie, {Ian G} and Iwona Rospond-Kubiak and Jaroslaw Kociecki and Kiilgaard, {Jens Folke} and Steffen Heegaard and Cohen, {Victoria M L} and Sagoo, {Mandeep S} and Anush Amiryan and Svetlana Saakyan and Nils Eide and J{\o}rgen Krohn and Edoardo Midena and Raffaele Parrozzani and Jean-Daniel Grange and Emine Kilic and Blasi, {Maria Antonietta} and Saornil, {Maria Antonia} and Kivel{\"a}, {Tero T}",

year = "2016",

month = apr,

day = "1",

doi = "10.1016/j.ophtha.2015.12.024",

language = "English",

volume = "123",

pages = "898--907",

journal = "Ophthalmology",

issn = "0161-6420",

publisher = "Elsevier",

number = "4",

}

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

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 -

The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group

Abstract

Keywords

Access to Document

Fingerprint

Cite this