Therapeutic outcomes of retinal hemangioblastomas

Retina. 2014 Dec;34(12):2479-86. doi: 10.1097/IAE.0000000000000254.

Abstract

Purpose: To report the results of treatments and therapeutic complications of retinal hemangioblastomas (RH).

Methods: Retrospective consecutive case series. Data from 32 patients (37 eyes) with RH were reviewed for characteristics of RH and treatment outcomes.

Results: Among 32 patients, we identified 73 RHs in 37 eyes. At baseline, 24 of 37 eyes (65%) had 20/50 visual acuity or better, 8 eyes (22%) had intermediate vision (20/400-20/50), and 5 eyes (13%) had poor vision (≤20/400). Seven RHs (9.6%) were located in the juxtapapillary area, and 66 RHs (90.4%) were located in peripheral area. Small RHs (54.8%; <0.5 mm in size) were treated with laser photocoagulation, moderate-sized RHs (24.7%; 0.5-3.0 mm in size) were treated with transpupillary thermotherapy, and large RHs (20.5%; >3.0 mm in size) were treated with a combination of transpupillary thermotherapy and cryotherapy. After treatment, 90% of small RHs regressed, whereas only 67% of large RHs regressed (P = 0.044). Peripheral RHs showed better response to treatment than juxtapapillary RHs (P = 0.010). Treatment-related complications occurred in 5 eyes (14%), and 1-step combination therapy was applied more frequently in the complication group (P = 0.048).

Conclusion: Small RHs in peripheral areas may require aggressive treatment because they respond well to treatment. In larger RHs, staged treatment could reduce treatment-related complications. Transpupillary thermotherapy could be an effective method in tumor regression for moderate-to-large-sized RHs showing tumor regression rate of 70%.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Female
  • Hemangioblastoma / diagnosis
  • Hemangioblastoma / physiopathology
  • Hemangioblastoma / therapy*
  • Humans
  • Hyperthermia, Induced*
  • Laser Coagulation*
  • Male
  • Middle Aged
  • Retinal Neoplasms / diagnosis
  • Retinal Neoplasms / physiopathology
  • Retinal Neoplasms / therapy*
  • Retrospective Studies
  • Treatment Outcome
  • Visual Acuity / physiology
  • von Hippel-Lindau Disease / complications