July 2018
Volume 59, Issue 9
Open Access
ARVO Annual Meeting Abstract  |   July 2018
Plaque Radiotherapy as Treatment for Medulloepithelioma in 6 Cases at a Single Center
Author Affiliations & Notes
  • Su Mae Ang
    Ocular Oncology, Wills Eye Hospital, Philadelphia, Pennsylvania, United States
  • Lauren A Dalvin
    Ocular Oncology, Wills Eye Hospital, Philadelphia, Pennsylvania, United States
    Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, United States
  • Carol L Shields
    Ocular Oncology, Wills Eye Hospital, Philadelphia, Pennsylvania, United States
  • Footnotes
    Commercial Relationships   Su Mae Ang, None; Lauren Dalvin, Heed Ophthalmic Foundation (F); Carol Shields, Eye Tumor Research Foundation (F)
  • Footnotes
    Support  Eye Tumor Research Foundation
Investigative Ophthalmology & Visual Science July 2018, Vol.59, 5593. doi:
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      Su Mae Ang, Lauren A Dalvin, Carol L Shields; Plaque Radiotherapy as Treatment for Medulloepithelioma in 6 Cases at a Single Center. Invest. Ophthalmol. Vis. Sci. 2018;59(9):5593.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : Medulloepithelioma is a rare congenital ocular tumor, which usually affects the nonpigmented ciliary epithelium and presents within the first decade of life. Limited information has been published describing plaque radiotherapy as treatment for medulloepithelioma. We performed a retrospective, clinical observational study to describe outcomes of patients with medulloepithelioma treated with plaque radiotherapy at a single center.

Methods : All patients with histopathologically confirmed medulloepithlioma treated with plaque radiotherapy at a single institution (Wills Eye Hospital) from 1980 to 2017 were included. Clinical and photographic records were reviewed for patient demographics, presenting symptoms, tumor size and location, treatment, and outcome.

Results : There were 6 patients treated with plaque radiotherapy for medulloepithelioma during the study time period. Patient demographics included mean patient age 145 months (median 41, range 10-624 months), white race (n=6), and female sex (n=5). The main presenting symptom was decreased vision (n=1), strabismus (n=1), corectopia (n=1), hyphema (n=1), ocular pain (n=1), and visible iris lesion (n=1). Tumor features included mean largest basal diameter 11.4 mm (median 10.0, range 7.0-16.0 mm), thickness 7.6 mm (median 8.7, range 3.1-11.0 mm), and ciliary body location (n=6). Pertinent findings on examination included cataract (n=2), lens notch (n=2), lens subluxation (n=1), synechiae (n=1), ectropion uveae (n=1), and retinal detachment (n=1). Plaque radiotherapy was primary treatment (n=5) or secondary treatment (n=1). Mean radiation dose to the apex of tumor was 4.4 Gy (median 3.8, range 3.5-7.0 Gy). Tumor control was achieved in 5 of 6 (83%) patients, with globe salvage in 4 of 6 (67%) patients. The patient who received plaque radiotherapy as secondary treatment underwent enucleation due to tumor recurrence with eye pain and secondary glaucoma. One patient who received primary plaque radiotherapy had appropriate tumor control but came to enucleation due to phthisis bulbi.

Conclusions : Plaque radiotherapy is effective as primary treatment for medulloepithelioma. When plaque radiotherapy is used as a secondary therapy, results might be less adequate.

This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.

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