The spectrum of intraocular lymphoid tumors ranges from benign reactive lymphoid hyperplasia to types of primary or secondary malignant lymphomas.

Primary intraocular lymphoma is commonly a non-Hodgkin’s large B cell lymphoma of the eye and the CNS.

The ocular findings include large clumps of cells in the vitreous, and the fundus examination is significant for multifocal, large, yellow, sub-retinal and sub-RPE infiltrative lesions.

Intraocular lymphomas can be classified into several subgroups: primary intraocular lymphoma (of the retina and vitreous), primary uveal lymphoma and secondary lymphoma.

DIFFERENTIAL DIAGNOSIS of Intraocular-CNS Lymphoma:

Subretinal and Sub-RPE Infiltrates:

• Acute posterior multifocal placoid pigment epitheliopathy
• Amelanotic choroidal melanoma
• Bilateral diffuse uveal melanocytic proliferation
• Birdshot retinochoroidopathy
• Choroidal granuloma (e.g., sarcoidosis, tuberculosis)
• Choroidal metastasis
• Diffuse unilateral subacute neuroretinitis
• Fundus flavimaculatus
• Multifocal choroiditis
• Multiple evanescent white dot syndrome
• Pneumocystis choroiditis
• Sympathetic ophthalmia
• Vogt-Koyanagi-Harada syndrome

Retinal and Retinal Vascular Infiltrates:

• Fungal retinochoroiditis
• Retinal periphlebitis (e.g., sarcoidosis, toxoplasmosis)
• Retinal vascular occlusion
• Retinal vasculitis (e.g., frosted branch angiitis, Behçet’s disease)
• Toxoplasmosis
• Viral retinitis (e.g., acute retinal necrosis, cytomegalovirus retinitis)

Vitreous:

• • Amyloidosis
  • Intermediate or posterior uveitis
  • Old vitreous hemorrhage
  • Senile vitritis

Optic nerve

• • Infiltrative disease (e.g., leukemia, metastasis, granuloma)
  • Papilledema