Photodynamic Therapy for Retinal Hemangioblastoma: Treatment Outcomes of 17 Consecutive Patients

Abstract

Purpose: To report the efficacy of photodynamic therapy (PDT) for management of retinal hemangioblastoma.

Design: Retrospective case series.

Participants: Seventeen patients with retinal hemangioblastoma treated with PDT.

Methods: The medical records of 17 patients with retinal hemangioblastoma treated with PDT were reviewed, and treatment outcomes were assessed. Photodynamic therapy was performed with 6 mg/m² body surface area of verteporfin infused intravenously over 10 minutes activated by 50 J/cm² laser light at 689 nm for 83 or 166 seconds.

Main outcome measures: Tumor control, subretinal and intraretinal fluid resolution, and visual outcome.

Results: Eighteen retinal hemangioblastomas in 17 eyes were treated with PDT. Median patient age was 31 years (mean, 36 years; range, 7-66 years), and median follow-up was 51 months (mean, 61 months; range, 2-144 months). Genetic testing confirmed von Hippel-Lindau disease in 8 of 17 patients (47%). The tumors were unilateral in all patients and unifocal in most patients (n = 13/17 [76%]). The tumor median basal diameter was 3.5 mm (mean, 3.4 mm; range, 1.5-6.0 mm) and median thickness was 2.1 mm (mean, 2.3 mm; range, 1.0-5.0 mm). The tumor location was juxtapapillary in 9 of 18 cases (50%). Associated findings included subretinal fluid (n = 14/17 [82%]) and macular edema (n = 12/17 [71%]). The median number of PDT sessions was 1.5 (mean, 1.8; range, 1.0-4.0). Standard duration of PDT (83 seconds) was used in all cases except 2, in which double duration (166 seconds) was used. Outcomes revealed tumor control in 13 of 18 tumors (72%), partial or complete resolution of subretinal fluid in 10 of 14 eyes (71%), and partial or complete resolution of macular edema in 7 of 12 eyes (58%), and stable or improved visual acuity in 12 of 17 eyes (71%). Photodynamic therapy-related transient exudative response was noted in 4 of 17 eyes (24%).

Conclusions: Photodynamic therapy is an effective treatment for both juxtapapillary and peripheral retinal hemangioblastomas, providing satisfactory rates of tumor control and visual stabilization and improvement. Patients should be monitored for PDT-related transient exudative response.

Keywords: Hemangioblastoma; Photodynamic therapy; Retina; Tumor; von Hippel Lindau.