Which clinical history findings are characteristic of primary CNS lymphoma with ocular involvement (PCNSLO)?

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

Intraocular lymphoma has been divided anatomically into vitreoretinal and uveal forms. The vitreoretinal form is associated with PCNSL and is typically a large B-cell tumor (intermediate-grade lymphoma). In contrast, the uveal form is associated with systemic NHL and also with involvement of orbital structures. It is typically small B-cell proliferation (low-grade lymphoma) and usually occurs with advanced systemic disease. Rare cases of T-cell lymphoma with ocular involvement have been reported.

On initial presentation, PCNSLO may be either unilateral or bilateral, but ultimately, 80-90% of patients have bilateral involvement. Intracranial disease occurs in 56-85% of patients with ocular disease, and estimates suggest that 15-25% of patients who present with CNS disease have ocular disease—hence the distinction between PCNSL and PCNSLO. [20, 21, 22, 23, 24]

The typical clinical profile is an elderly patient with uveitis that is refractory to treatment. The most common subjective symptoms are painless decreased vision, photophobia, red eye, and floaters. In some patients with known PCNSL, ocular disease may be discovered on routine screening. Because of its insidious onset and ability to simulate other conditions, delay in diagnosis is common.

Vision loss is frequent in PCNSLO and may range from mild to severe. With extensive disease, circulating tumor cells can appear in the anterior chamber in as many as 75% of patients. The cells simulate iridocyclitis and can even form a pseudohypopyon. Secondary anterior segment changes include neovascularization of the iris and iridocorneal angle with possible glaucoma. In rare circumstances, PCNSLO can form a mass in the iris or angle.

In the posterior segment, vitreous cells are a typical finding and are present in most cases. The characteristic fundus lesion is a low-lying, yellow-to-white mass deep to the sensory retina. Lesions may be single or multiple, confluent or discrete. They may even appear as multiple punctate lesions.

Lesions may be infiltrative and involve all layers of the retina. Retinal hemorrhage is rarely seen. The deep location of the infiltrates can give rise to exudative retinal detachment. If chorioretinal lesions regress, scarring and atrophy of the retinal pigment epithelium may be the only remaining fundus findings. Optic neuropathy may also be a feature. [20, 21, 22, 23, 24]

PCNSLO appears to occur with increased frequency in persons who are severely immunosuppressed.


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