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Treatment and Outcome of Childhood Intraocular (Uveal) Melanoma
Source: Childhood Intraocular (Uveal) Melanoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: August 13, 2024 · Captured 2026-09-09.
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Treatment options for childhood intraocular (uveal) melanoma include the following:
Surgery.
Radiation therapy.
Bispecific fusion proteins (tebentafusp).
A retrospective single-institution review identified 18 patients younger than 21 years with uveal melanoma.[3][Level of evidence C1] Patients were treated with enucleation (n = 9), transscleral en bloc resection (n = 3), plaque radiation therapy (n = 5), or proton-beam radiation therapy (n = 1).
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Eight of these patients (44%) developed metastatic disease, all of whom died of their disease. The median survival time after detection of metastasis was 2.3 months (95% confidence interval [CI], 0.0–5.2 months).
The median overall survival (OS) of the 18 patients after treatment of the primary intraocular tumors was 11.9 years (95% CI, 7.3–16.5 years).
Laser surgery has been used to treat childhood intraocular melanoma.[1,2]
An open-label, randomized, phase III trial of adult patients with previously untreated HLA-A*02:01–positive metastatic uveal melanoma investigated treatment with tebentafusp. Tebentafusp is a bispecific restricted T-cell receptor for the glycoprotein 100 peptide; it is fused to a CD3 single-chain variable fragment. Patients were randomly assigned to receive either tebentafusp or treatment according to the investigator's choice.[4]
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Patients who received tebentafusp had an OS rate of 73%, compared with 50% for patients in the control group (hazard ratio [HR] for death, 0.51; P < .001).
The progression-free survival rate was also significantly higher for the tebentafusp group (31% vs. 16% at 6 months; HR for progression or death, 0.71; P = .01).
The most common toxicities for patients who received tebentafusp were cytokine release syndrome, mostly grades 1 and 2, and rash.
For information about the treatment of uveal melanoma in adults, see Intraocular (Uveal) Melanoma Treatment.
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Preserved source evidence · Not medical advice