Primary uveal melanoma in a 4-year-old black child

Gray, Michael E.; Shaikh, Adeel H.; Corrêa, Zélia M.; Augsburger, James J.

doi:10.1016/j.jaapos.2013.06.010

Cited by 6 publications

(11 citation statements)

References 8 publications

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“…Interestingly, 1 child in this group (case 1 in Table 1) had very advanced disease with extraocular extension and oculodermal melanosis. 14 Despite the aggressive presentation and the acute development of metastatic disease, the gene expression profile in this patient was class 1B. 15,16 Most patients in this study (n = 16 of 18 [89%]) were in their teenage years (13.1 to ≤20 years) when diagnosed with PUM.…”

Section: Discussionmentioning

confidence: 86%

“…The observed actuarial probabilities of metastasis and metastatic death in any given population of patients with PUM depend on a number of clinical and pathologic determinable factors. [10][11][12][13][14][15] The number of patients and deaths in this small series of cases was insufficient for multivariate analysis and identification of any prognostic factors in this subgroup. Interestingly, 1 child in this group (case 1 in Table 1) had very advanced disease with extraocular extension and oculodermal melanosis.…”

Section: Discussionmentioning

confidence: 99%

“…This unusual case has been reported separately. 14 Patients were treated with enucleation of the affected eye in 9 cases (50%), plaque radiotherapy in 5 cases (28%), transscleral en bloc resection in 3 cases (17%), and proton beam irradiation in 1 case (6%). The tumor was evaluated histopathologically in the 12 cases that were managed by enucleation or transscleral tumor extension.…”

Section: Key Pointsmentioning

confidence: 99%

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Clinical Features, Metastasis, and Survival in Patients Younger Than 21 Years With Posterior Uveal Melanoma

2019

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IMPORTANCE Given the rarity of posterior uveal melanoma in patients younger than 21 years, reporting clinical experience in this area has relevance. OBJECTIVE To describe the baseline clinical features, treatment, and clinical course of a group of patients younger than 21 years who have primary posterior uveal melanoma. DESIGN, SETTING, AND PARTICIPANTS This retrospective descriptive case series of patients younger than 21 years who have a primary choroidal or ciliochoroidal melanoma was conducted at a single-center subspecialty referral practice. Patients in the relevant age group who were treated in a single practice between July 1980 and December 2013 were included; clinical data collected through December 2017 were captured to permit adequate follow-up time in all cases. MAIN OUTCOMES AND MEASURES Conventional descriptive statistics of relevant clinical variables (eg, demographic, tumor, treatment, and outcome variables) of each patient were recorded. Actuarial metastasis-free and overall survival curves were computed and plotted, as was a postdetection survival curve of patients who developed metastasis during available follow-up. RESULTS Of 2265 patients with posterior uveal melanoma encountered by the authors during the study interval, 18 (0.8%) were younger than 21 years when diagnosed and treated. Ten were female and 8 male, and the mean (SD) age was 16.6 (4.2) years. Through available follow-up, 8 of these patients had developed metastatic uveal melanoma (44%). All 8 died of metastasis. Actuarial survival analysis showed that the cumulative probability of metastatic death in this group exceeded 50%. The median overall survival time after treatment of the primary intraocular tumor was 11.9 (95% CI, 7.3-16.5) years. The median survival time after detection of metastasis in the 8 patients who developed metastasis was 2.3 months (95% CI, 0.0-5.2) months. CONCLUSIONS AND RELEVANCE Posterior uveal melanoma in patients younger than 21 years appears to have a similar if not worse prognosis than patients with PUM in the population overall. Owing to the later onset of metastasis observed, patients younger than 21 years should continue to have surveillance tests for more than 10 years after treatment.

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Section: Discussionmentioning

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Section: Key Pointsmentioning

confidence: 99%

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Clinical Features, Metastasis, and Survival in Patients Younger Than 21 Years With Posterior Uveal Melanoma

2019

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“…A total of 166 patients met these inclusion criteria. Two patients who were gross outliers compared with all other patients in the group were eliminated subsequently: one was a previously reported 4-year-old child who presented with an eye filled completely by tumor and massive orbital tumor extension [11], and the other was an adult patient who had two separate tumors in his affected eye, one of which was categorized by GEP testing as class 2. After these exclusions, our residual study group consisted of 164 patients.…”

Section: Methodsmentioning

confidence: 99%

Comparative Metastatic Rates in GEP Class 1A versus 1B Posterior Uveal Melanoma: Results Contrary to Expectations

2022

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Purpose: The purpose of this study was to determine whether the metastatic rates in patients with gene expression profile (GEP) class 1A versus 1B posterior uveal malignant melanoma supported or contradicted predictions of very low metastatic rate in GEP 1A cases and moderate rate in GEP 1B cases. Patients/Methods: 164 patients with a cytopathologically-confirmed primary posterior uveal malignant melanoma classified by GEP testing as class 1 (100 GEP 1A, 64 GEP 1B) were evaluated. Kaplan-Meier rates of metastasis were computed and plotted for the GEP class 1 subgroups. Median follow-up of patients who were still alive without metastasis on the date of data analysis was 100.5 months for the GEP 1A patients and 97.2 months for the GEP 1B patients. Results: The actuarial 5-year rate of uveal melanoma metastasis was 10.8% (std. error = 3.2%) in the GEP 1A patients versus 0% in the GEP 1B patients, and the actuarial 10-year rate of metastasis was 12.2% (std. error = 3.5%) in the GEP 1A patients versus 2.1% (std. error 2.1%) in the GEP 1B patients. Conclusion: The results of this retrospective single-center study cast doubt on the validity of the prognostic stratification of GEP class 1 posterior uveal malignant melanomas into very low risk (GEP 1A) versus intermediate risk (GEP 1B) of metastasis subgroups provided by the commercially available GEP test.

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“…Uveal melanoma (UM) is a rare neoplasm which, despite its rarity, represents the most common primary ocular malignancy in adults; it develops more frequently from melanocytes of the choroid but can also arise in other sites, such as ciliary bodies and iris [1]. UM has rarely been reported in pediatric ages, especially in advanced stages, with extraocular extension [2,3].…”

Section: Introductionmentioning

confidence: 99%

Immunohistochemical Expression of ABCB5 as a Potential Prognostic Factor in Uveal Melanoma

et al. 2019

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Uveal melanoma represents the most common primary intraocular malignancy in adults; it may arise in any part of the uveal tract, with choroid and ciliary bodies being the most frequent sites of disease. In the present paper we studied ABCB5 expression levels in patients affected by uveal melanoma, both with and without metastasis, in order to evaluate if ABCB5 is associated with a higher risk of metastatic disease and can be used as a poor prognostic factor in uveal melanoma. The target population consisted of 23 patients affected by uveal melanoma with metastasis and 32 without metastatic disease. A high expression of ABCB5 was seen in patients with metastasis (14/23, 60.9%), compared to that observed in patients without metastasis (13/32, 40.6%). In conclusion, we found that ABCB5 expression levels were correlated with faster metastatic progression and poorer prognosis, indicating their role as a prognostic factor in uveal melanoma.

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Primary uveal melanoma in a 4-year-old black child

Cited by 6 publications

References 8 publications

Clinical Features, Metastasis, and Survival in Patients Younger Than 21 Years With Posterior Uveal Melanoma

Clinical Features, Metastasis, and Survival in Patients Younger Than 21 Years With Posterior Uveal Melanoma

Comparative Metastatic Rates in GEP Class 1A versus 1B Posterior Uveal Melanoma: Results Contrary to Expectations

Immunohistochemical Expression of ABCB5 as a Potential Prognostic Factor in Uveal Melanoma

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