How is intraocular lymphoma treated?

Updated: Mar 15, 2019
  • Author: Manolette R Roque, MD, MBA, FPAO; Chief Editor: Hampton Roy, Sr, MD  more...
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Answer

Answer

Because of the high recurrence rate and refractory nature of primary central nervous system lymphoma with ocular involvement (PCNSLO), treatment is difficult. [17] However, treatment of intraocular lymphoma underwent a significant advance with the introduction of chemoradiation to the central nervous system (CNS) and ocular radiation.

Radiation (3500-4000 cGy) alone to the eyes and CNS gave high rates of initial response, but patients usually succumbed to recurrent disease. With multimodality therapy, including a boosted radiation dose (5000-10,000 cGy) to the spinal cord and intrathecal methotrexate, vision can be improved and life can be prolonged, with some patients alive at 9 years after treatment. In selected cases, patients with isolated ocular disease have been treated with ocular radiation alone, with some longer-term survivors. [44, 45, 46]

Innovations in treatment include multiagent primary chemotherapy. This approach was designed to reduce the radiation-associated cognitive defects that can occur in up to 40% of patients older than 50 years. The regimen includes methotrexate and procarbazine, vincristine, thiotepa, or both vincristine and cytarabine. Complete remission has been seen for as long as 30 months. [44, 45, 46]

Radiotherapy for PCNSLO is highly effective, and complications are generally acceptable. Complications of radiotherapy include cataract, dry eye, corneal ulcer, neovascular glaucoma, radiation retinopathy, and optic neuropathy. [47, 48] In the absence of a clear advantage to intravitreal chemotherapy, which involves repetitive injections and associated risks, radiotherapy may still be the most appropriate first-line treatment in most cases. [49]

Research is ongoing to determine if lower doses of local radiation are effective and still reduce associated complications.


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