Retinal haemangioblastoma: an adversary to fight

Size and location decisive in treatment outcome

Retinal haemangioblastoma is a rare disease that is difficult to manage, involving a careful assessment of the balance between potential visual loss caused by the tumour vs visual loss induced by treatment. The identification of the best treatment and its correlation with tumour size and location is much debated.
In current clinical practice, asymptomatic non-exudating tumours are generally observed, particularly if peripapillary or perimacular, and treatment is undertaken if progressive subretinal fluid loss, exudation, or intra-retinal oedema occurs. Tumours with peripheral localisation generally receive prompt treatment at the time of diagnosis.
A retrospective study published in Retina The research team of Dr. Hyesun Kim (South Korea) proposes an aggressive treatment strategy with direct photocoagulation for small retinal haemangioblastomas, which generally respond well to treatment. As the tumour size increases, staged therapy is recommended to reduce side complications.
For medium to large tumours, TTT (Transpupillary Thermo-Therapy) can be effective with regression rates of 70%.
Therefore, the best treatment protocol involves the earliest possible diagnosis (when the tumour mass is small) and timely therapeutic treatment, also because advanced tumours tend to present vitreoretinal exudation with tractional retinal detachment, peripheral ischaemia and in some cases neovascular glaucoma.

For more details see Retina. 2014;34(12):2479-2486.

Dr. Carmelo Chines
Direttore responsabile

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