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Implantation and Evaluation of Melanoma in the Murine Choroid via Optical Coherence Tomography
Published on: December 2, 2022
The Pediatric Choroidal and Ciliary Body Melanoma Study: A Survey by the European Ophthalmic Oncology Group
Rana'a T Al-Jamal1, Nathalie Cassoux2, Laurence Desjardins2
1Ocular Oncology Service, Department of Ophthalmology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Insights
Children with choroidal and ciliary body melanoma (CCBM) have better survival rates than young adults. Factors like age, gender, and tumor stage significantly impact prognosis for CCBM patients.
Area of Science:
- Ophthalmology
- Oncology
- Pediatrics
Background:
- Choroidal and ciliary body melanoma (CCBM) is a rare malignancy.
- Understanding prognostic factors in pediatric and young adult CCBM is crucial for treatment and survival.
- Previous hypotheses suggested better survival for younger patients, males, and those without ciliary body involvement.
Purpose of the Study:
- To gather comprehensive data on pediatric and young adult CCBM.
- To validate hypotheses regarding survival prognosis in different age groups and genders.
- To identify key factors influencing survival in CCBM.
Main Methods:
- Retrospective, multicenter observational study involving 299 patients (114 children, 185 young adults).
- Data collected via a secure website and centrally reviewed.
- Survival analyzed using Kaplan-Meier and Cox proportional hazards regression.
Main Results:
- Children (median age 15.1 years) showed significantly better 5- and 10-year melanoma-related survival (97% and 92%) compared to young adults (90% and 80%).
- Higher tumor-node-metastasis (TNM) stage and female gender were independently associated with poorer survival.
- Males tended to have better survival than females among children, though not statistically significant.
Conclusions:
- Children with CCBM demonstrate a more favorable survival prognosis than young adults (18-25 years), even after adjusting for TNM stage and gender.
- The association between gender and survival outcomes in CCBM appears to vary between pediatric and young adult populations.
- TNM stage and age group are significant independent predictors of survival in CCBM.
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.
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