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What are they?

The retina is a sensitive tissue of the eye containing a multitude of blood vessels. These vessels may grow and proliferate anomalously, be it from birth or acquired, leading to different vascular tumours of the retina. 

Types

There are four types:

  1. Capillary hemangioblastoma (which is occasionally associated with Von Hippel-Lindau syndrome)
  2. Cavernous hemangioma
  3. Vasoproliferative proliferal tumours 
  4. Racemose hemangioma (congenital arteriovenous communications of the retina)

Symptoms

The symptoms depend on the type of tumour and its location. Hemangioblastoma of the retina and vasoproliferative tumours can exudate (lose liquid), leading to exudative retinal detachment or it may generate proliferations that exert pressure on the retina. 

In these cases, the patient show signs of gradually worsening vision. Cavernous and racemose hemangioma are congenital and if they are located near the centre of the retina, they can lead to underdeveloped eyesight (amblyopia). 

Treatment

There are multiple treatment alternatives depending on the type of tumour, its location and the associated complications. The most commonly used treatments are intravitreal drug injections that are capable of reducing vascular permeability, laser, cryotherpay and vitreous surgery to treat tractional complications or retina detachment, episcleral brachytherapy and external radiotherapy. In many cases, combined treatments are required. It is a complex pathology that should be treated in a centre of reference.

Professionals who treat this pathology

Frequently asked questions

  • The only vascular tumour of the retina that has a known hereditary component is capillary hemangioblastoma in the context of Von Hippel-Lindau syndrome. Its inheritance pattern is autosomal dominant and the gene causing the disease is known. When it comes to a tumour with these characteristics, it is suitable to carry out a well- designed genetic study.

  • Capillary hemangioblastoma associated with Von Hippel-Lindau syndrome usually develops with tumours in different organs (kidney, cerebullum, spinal cord) throughout a person's lifetime. These cases require a multidisciplinary approach and monitoring. Racemose hemangioma in its most developed form and especially when it affects the optic nerve, can also be associated with vascular deformations in the intracraneal space.  

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