Proton Therapy for Uveal Melanoma in 43 Juvenile Patients

Petrović, Aleksandra; Bergin, Ciara; Schalenbourg, Ann; Goitein, Gudrun; Zografos, Léonidas

doi:10.1016/j.ophtha.2013.10.032

Cited by 19 publications

(25 citation statements)

References 33 publications

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“…5,8,17 Those may be the reasons why this group of patients had a higher number of events compared with other studies' reports of lower rates of metastasis. 8,17,18 However, 1 study that found a lower frequency of young patients with posterior uveal melanoma (only 17 of 3207 patients ≤20 years of age [0.5%]) treated with proton beam irradiation showed no deaths or development of metastasis during a long follow-up period (median, 16 [range, 4.7-25.2] years) that was comparable with ours. 18 In our group of patients younger than 21 years, a substantial proportion developed metastasis ( Figure 1) and died of that metastasis ( Figure 2) during available follow-up.…”

Section: Discussionmentioning

confidence: 58%

“…5,8,17,18 Because this was shown by other researchers, the expectation in this analysis was to find a substantially better metastasis and survival outcome in this subgroup compared with older patients, despite the management method used for their PUM. 5,8,17,18 Because we did not find evidence of BAP1 tumor predisposition syndrome and only 1 patient that had oculodermal melanosis, there are only few explanations as to the findings of this analysis compared with other reports. First, because young people who develop a PUM have fewer competing risks for death than elderly persons with the same type of tumor, young people appear to have a higher long-term cumulative probability of death from metastasis than older persons do.…”

Section: Discussionmentioning

confidence: 80%

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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: 58%

Section: Discussionmentioning

confidence: 80%

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

2019

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“…Studies of tumours outside the cns-including skull base chordoma and chondrosarcoma 22,23 , uveal melanoma 23,24 , germ-cell tumours 25 , high-risk neuroblastoma 26,27 , parameningeal rhabdomyosarcoma 28 , bladder and prostate rhabdomyosarcoma 29 , other soft tissue sarcomas 30 , Ewing sarcoma 31 , and mediastinal Hodgkin lymphoma 32 -uniformly showed that pbt is well tolerated, with local control rates similar to or higher than those achieved with photon rt. Although planning studies and early clinical results are promising, long-term data supporting a clear advantage of pbt over photon rt are not yet available.…”

Section: Pediatricsmentioning

confidence: 99%

Recommendations for the Referral of Patients for Proton-Beam Therapy, an Alberta Health Services Report: A Model for Canada?

et al. 2014

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able and willing to travel abroad to a proton facility. Commonly accepted indications for referral include chordoma and chondrosarcoma, intraocular melanoma, and solid tumours in children and adolescents who have the greatest risk for long-term sequelae. Current data do not provide sufficient evidence to recommend routine referral of patients with most head-and-neck, breast, lung, gastrointestinal tract, and pelvic cancers, including prostate cancer. It is recommended that all referrals be considered by a multidisciplinary team to select appropriate cases.

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“…Juvenile uveal melanoma occurring in patients younger than 21 years old is rare, but associated with a significantly favourable survival (Petrovic et al. ). Male gender has been proposed to be associated with increased mortality in patients with uveal melanoma (Virgili et al.…”

Section: Introductionmentioning

confidence: 99%

Intraocular biopsy of uveal melanoma Risk assessment and identification of genetic prognostic markers

Bagger

2018

Acta Ophthalmologica

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Proton Therapy for Uveal Melanoma in 43 Juvenile Patients

Cited by 19 publications

References 33 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

Recommendations for the Referral of Patients for Proton-Beam Therapy, an Alberta Health Services Report: A Model for Canada?

Intraocular biopsy of uveal melanoma Risk assessment and identification of genetic prognostic markers

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