Intravitreal Melphalan for Treatment of Primary Vitreoretinal Lymphoma

Shields, Carol L.; Sioufi, Kareem; Mashayekhi, Arman; Shields, Jerry A.

doi:10.1001/jamaophthalmol.2017.1810

Cited by 21 publications

(10 citation statements)

References 8 publications

(20 reference statements)

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“…This drug and delivery have been used extensively in the treatment of retinoblastoma (typically a dose of 20e30 mg) 16 and sparingly in vitreoretinal lymphoma (10 mg/ml). 17 One report describes its use in a single patient (12 injections of 10 mg/0.05 ml) for the treatment of vitreous seeding from uveal melanoma with a satisfactory outcome. 18 The premise behind this technique is to deliver a high dose of local drug while sparing the body the toxicity of systemic delivery.…”

Section: Discussionmentioning

confidence: 99%

Intravitreous Cutaneous Metastatic Melanoma in the Era of Checkpoint Inhibition

et al. 2020

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Purpose: Cutaneous melanoma metastatic to the vitreous is very rare. This study investigated the clinical findings, treatment, and outcome of patients with metastatic cutaneous melanoma to the vitreous. Most patients received checkpoint inhibition for the treatment of systemic disease, and the significance of this was explored.Design: Multicenter, retrospective cohort study.Participants: Fourteen eyes of 11 patients with metastatic cutaneous melanoma to the vitreous. Methods: Clinical records, including fundus photography and ultrasound results, were reviewed retrospectively, and relevant data were recorded for each patient eye.Main Outcome Measures: Clinical features at presentation, ophthalmic and systemic treatments, and outcomes.Results: The median age at presentation of ophthalmic disease was 66 years (range, 23e88 years), and the median follow-up from diagnosis of ophthalmic disease was 23 months. Ten of 11 patients were treated with immune checkpoint inhibition at some point in the treatment course. The median time from starting immunotherapy to ocular symptoms was 17 months (range, 4.5e38 months). Half of eyes demonstrated amelanotic vitreous debris. Five eyes demonstrated elevated intraocular pressure, and 4 eyes demonstrated a retinal detachment. Six patients showed metastatic disease in the central nervous system. Ophthalmic treatment included external beam radiation (30e40 Gy) in 6 eyes, intravitreous melphalan (10e20 mg) in 4 eyes, enucleation

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Section: Discussionmentioning

confidence: 99%

Intravitreous Cutaneous Metastatic Melanoma in the Era of Checkpoint Inhibition

et al. 2020

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“…Melphalan injections directly into the vitreous or the aqueous humor via intravitreal or intracameral injections respectively, allow high intraocular tumoricidal drug concentrations to be reached, while bypassing systemic adverse effects and are therefore currently the treatment of choice for vitreous and aqueous seeding in retinoblastoma [19,20]. Recently intravitreal melphalan has also been used to salvage two cases with primary vitreoretinal lymphoma [21]. In our case, intraocular melphalan alone was sufficient to achieve complete regression, not only of the seeding but also of the primary solid tumor, presumably because of its friable nature and the absence of concomitant ciliary muscle infiltration.…”

Section: Discussionmentioning

confidence: 99%

Successful treatment of ciliary body medulloepithelioma with intraocular melphalan chemotherapy: a case report

et al. 2020

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Background Intraocular medulloepithelioma is commonly treated with primary enucleation. Conservative treatment options include brachytherapy, local resection and/or cryotherapy in selected cases. We report for the first time the use of targeted chemotherapy to treat a ciliary body medulloepithelioma with aqueous and vitreous seeding. Case presentation A 17-month-old boy with a diagnosis of ciliary body medulloepithelioma with concomitant seeding and neovascular glaucoma in the right eye was seen for a second opinion after parental refusal of enucleation. Examination under anesthesia showed multiple free-floating cysts in the pupillary area associated with iris neovascularization and a subluxated and notched lens. Ultrasound biomicroscopy revealed a partially cystic mass adjacent to the ciliary body between the 5 and 9 o’clock meridians as well as multiple nodules in the posterior chamber invading the anterior vitreous inferiorly. Fluorescein angiography demonstrated peripheral retinal ischemia. Left eye was unremarkable. Diagnosis of intraocular medulloepithelioma with no extraocular invasion was confirmed and conservative treatment initiated with combined intracameral and intravitreal melphalan injections given according to the previously described safety-enhanced technique. Ciliary tumor and seeding totally regressed after a total of 3 combined intracameral (total dose 8.1 μg) and intravitreal (total dose 70 μg) melphalan injections given every 7–10 days. Ischemic retina was treated with cryoablation as necessary. Three years later, ab interno trabeculotomy followed by 360° gonioscopy-assisted transluminal trabeculotomy 6 months later was performed for uncontrolled intraocular pressure despite antihypertensive drugs combined to cyclophotocoagulation and 7 intravitreal anti-VEGF injections for recurrent iris neovascularization. Cataract was removed at the same operative time. The child has remained disease- and metastasis-free at a 5-year follow-up since the last melphalan injection (25-month follow-up after the combined lensectomy-trabeculotomy) with a controlled intraocular pressure under topical quadritherapy and a best corrected Snellen visual acuity of 0.08. Conclusions We report for the first time complete regression of a non-infiltrating ciliary body medulloepithelioma with seeding achieved with only a small number of intracameral and intravitreal melphalan injections. Concomitant secondary neovascular glaucoma and cataract needed appropriate management to allow long-term eye and vision preservation.

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“…Disappearance of sub-RPE deposits following intravitreal chemotherapy or ocular radiotherapy provides evidence for such clearance. There is scope for studies comparing therapeutic vitrectomy with ocular radiotherapy as well as intravitreal chemotherapy with methotrexate or melphalan [13]. …”

Section: Discussionmentioning

confidence: 99%

Insights from a Case of Vitreoretinal Lymphoma

et al. 2018

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Purpose/Background: The aim of this study was to report a patient with vitreoretinal lymphoma with clinical features providing hypothesis-generating insights into the pathophysiology of this disease. Methods: Clinical history and imaging studies (i.e., fundus photography, optical coherence tomography, fundus autofluorescence, and fluorescein angiography) were documented. Results: A 71-year-old woman presented with a 2-month history of blurred vision in the right eye and bilateral vitreous infiltrates unresponsive to topical and systemic steroids. Vitreous biopsy of the left eye was diagnostic for lymphoma. Bulky subretinal deposits in the right eye responded to systemic therapy. The left fundus showed diffuse hypoautofluorescence and punctate, hyperfluorescent sub-retinal pigment epithelial tumor deposits, which resolved leaving hypoautofluorescent atrophic retinal pigment epithelium (RPE) scars, except inferotemporally, where retinal vasculopathy had occurred. Conclusions: The clinical features suggest that occlusion of the inferotemporal retinal arteriole prevented sub-RPE lymphomatous deposits and subsequent RPE atrophy in this area of vascular nonperfusion. This suggests that “primary” vitreoretinal lymphoma is secondary to hematogenous spread from systemic loci. This finding, together with the ocular tumor control achieved entirely by systemic therapy, indicates scope for studies investigating systemic treatment protocols, especially those including immune-modulatory agents.

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Intravitreal Melphalan for Treatment of Primary Vitreoretinal Lymphoma

Cited by 21 publications

References 8 publications

Intravitreous Cutaneous Metastatic Melanoma in the Era of Checkpoint Inhibition

Intravitreous Cutaneous Metastatic Melanoma in the Era of Checkpoint Inhibition

Successful treatment of ciliary body medulloepithelioma with intraocular melphalan chemotherapy: a case report

Insights from a Case of Vitreoretinal Lymphoma

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