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Retinal hemangioblastoma size, location affect tumor regression
The efficacy of treatment for retinal hemangioblastoma hinges on tumor size and location, according to a study.
The retrospective case series included 37 eyes of 32 patients with retinal hemangioblastomas.
Seventy-three tumors were identified in the 37 eyes. Seven (9.6%) were identified in the juxtapapillary area, and 66 (90.4%) were identified in the peripheral area.
Small tumors less than 0.5 mm underwent laser photocoagulation. Moderate-size tumors that were 0.5 mm to 3 mm underwent transpupillary thermotherapy. Large tumors greater than 3 mm underwent combined transpupillary thermotherapy and cryotherapy.
Mean follow-up was 33.6 months.
After treatment, 90% of small tumors and 66.7% of large tumors regressed; the between-group difference was statistically significant (
Peripheral tumors responded significantly better to treatment than juxtapapillary tumors (
Size and location of tumors were the only factors that significantly affected tumor regression.
Five eyes (14%) experienced treatment-related complications; one-step combination therapy was used more frequently in eyes with complications (
Poor baseline visual acuity correlated significantly with poor visual outcome (
Tumors of patients with good baseline best corrected visual acuity of 20/50 or better typically showed greater regression than those of patients with poor baseline BCVA of 20/400 or worse, but the difference was not significant.
Disclosure: The authors have no relevant financial disclosures.
Perspective
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The treatment of retinal hemangioblastoma is challenging. Management of this benign tumor involves decisions regarding the balance of potential tumor-related vision loss versus treatment-related vision loss. There is a debate regarding best treatment for retinal hemangioblastoma, depending on tumor size and location. Our current philosophy is to observe non-leaking, asymptomatic tumors with caution, particularly if peripapillary or perimacular, and if there is progressive tumor leakage of subretinal fluid, exudation, or intraretinal edema, then therapy is provided. Those that are peripheral are generally offered prompt therapy at initial diagnosis. We agree with the treatment philosophy used by Kim et al using direct photocoagulation for small, combined vascular feeder photocoagulation and thermotherapy for medium, and combined vascular feeder photocoagulation and cryotherapy for large tumors.
Other methods that we offer include photodynamic therapy for small to medium tumors, particularly those near the fovea or optic nerve. Additionally, we employ plaque radiotherapy for medium to large tumors, especially those with extensive subretinal fluid. Often we provide adjunctive intravitreal anti-vascular endothelial growth factor (VEGF) or triamcinolone (intravitreal or subtenon’s) to prevent exudative response.
Based on this study, we agree that the ideal management for this tumor is to identify the mass early and intervene at the first necessary point. Keep in mind that advanced cases tend to display vitreoretinal exudation with tractional retinal detachment, peripheral ischemia and eventually neovascular glaucoma.