Ang, Su Mae; Dalvin, Lauren A.; Emrich, Jacqueline; Komarnicky, Lydia; Shields, Jerry A.; Shields, Carol L.
Purpose: To describe outcomes of 6 patients treated with plaque radiotherapy for medulloepithelioma.
Design: Retrospective review.
Methods: Computerized medical records were reviewed for cytopathologically confirmed medulloepithelioma treated with plaque radiotherapy from 1970 to 2017. Clinical and radiotherapy parameters along with outcomes of tumor regression, globe salvage, and metastatic disease were recorded.
Results: Of 6 patients with medulloepithelioma, plaque radiotherapy was primary (n = 5) or secondary (n = 1) treatment. The median patient age at diagnosis was 41 months (mean, 145; range, 10–624 months). Patient demographics included white race (n = 6) and female sex (n = 5). Patients presented with strabismus (n = 1), decreased vision (n = 1), ocular pain (n = 1), hyphema (n = 1), corectopia (n = 1), and visible iris lesion (n = 1). Mean tumor basal diameter was 11.2 mm (median, 10.0; range, 7.0–16.0 mm), and mean tumor thickness was 6.8 mm (median, 6.4; range, 3.1–11.0 mm). Related findings included cataract (n = 4), iris neovascularization (n = 5), secondary glaucoma (n = 2), and ectropion uveae (n = 2). Mean radiation dose to tumor apex was 44 Gy (median, 38; range, 35–70 Gy). At mean follow-up of 59 months, tumor control was achieved in 5 (83%) eyes with globe salvage in 4 (67%) eyes. No patient had evidence of metastases or death at last follow-up visit (mean, 59 months; range, 12–210 months).
Conclusions: Plaque radiotherapy is a reasonable alternative treatment for localized, small to medium-size medulloepithelioma. Tumors larger than 11.0 mm, with retrolental cyclitic membrane or extrascelral extension, might still require enucleation.