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

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License: CC BY 4.0
Serval ID
serval:BIB_6D25F790F4D7
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Institution
Title
Successful treatment of ciliary body medulloepithelioma with intraocular melphalan chemotherapy: a case report.
Journal
BMC ophthalmology
Author(s)
Stathopoulos C., Gaillard M.C., Schneider J., Munier F.L.
ISSN
1471-2415 (Electronic)
ISSN-L
1471-2415
Publication state
Published
Issued date
18/06/2020
Peer-reviewed
Oui
Volume
20
Number
1
Pages
239
Language
english
Notes
Publication types: Case Reports ; Journal Article
Publication Status: epublish
Abstract
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.
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.
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.
Keywords
Antineoplastic Agents, Alkylating/administration & dosage, Ciliary Body, Humans, Infant, Injections, Intraocular, Intraocular Pressure, Male, Melphalan/administration & dosage, Microscopy, Acoustic, Neuroectodermal Tumors, Primitive/diagnosis, Neuroectodermal Tumors, Primitive/drug therapy, Retinal Neoplasms/diagnosis, Retinal Neoplasms/drug therapy, Visual Acuity, Intracameral injection, Intraocular medulloepithelioma, Intravitreal injection, Melphalan
Pubmed
Web of science
Open Access
Yes
Create date
06/07/2020 14:27
Last modification date
05/05/2021 6:36
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