What is the prognosis of ocular lymphoma?

Updated: Mar 15, 2019
  • Author: Manolette R Roque, MD, MBA, FPAO; Chief Editor: Hampton Roy, Sr, MD  more...
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Death can occur as a result of the systemic spread of lymphoma. [4] Prior or concurrent systemic disease has been noted as the most significant predictive factor for lymphoma-related death, but tumor-related death was also found to be more common and earlier with bilateral disease. Tumor-related death is slightly less where symptoms have been present for more than a year and slightly greater in the elderly.

Patients with primary central nervous system lymphoma with ocular involvement (PCNSLO) have a poor prognosis even with chemoradiation, and many succumb to central nervous system (CNS) disease within 2 years. Yet, median survival of primary central nervous system lymphoma (PCNSL) has increased from 1-1.5 years to over 3 years with newer therapies. Features affecting the prognosis of PCNSLO are not well understood. [57] Death ensues by CNS dissemination. Ocular lymphoma may be the initial manifestation of PCNSL.

In a study of patients with ocular adnexal lymphomas, age, sex, and anatomic location of the lymphomas did not have prognostic significance during a follow-up period of 6 months to 16.5 years. [43] The major prognostic criteria for ocular adnexal lymphomas include anatomic location of the tumor, stage of disease at first presentation, subtype of lymphoma, immunohistochemical markers determining factors such as tumor growth rate, and the serum lactate dehydrogenase (LDH) level. [41]

The extent of disease at the time of presentation was the most important clinical prognostic factor. Advanced disease correlated with increased risk ratios of having persistent disease at the final follow-up and lymphoma-related death. [43]

The longest survival has been seen in patients with low-grade lymphomas (ie, marginal zone lymphoma, follicular lymphoma). [58] However, T-cell lymphomas are associated with high mortality with conventional treatment, as there is a high incidence of systemic involvement. [59]

The overall prognosis for ocular adnexal lymphoid tumors is excellent; when lumped together, 67% are not found to be associated with systemic disease with a mean follow-up of over 4 years. Over the course of follow-up, it could be anticipated that 20-25% of patients not known to have systemic lymphoma develop evidence of disseminated disease within 5 years. [60]

Conjunctival lymphoma is known to have the lowest rate of extraorbital spread and lymphoma-related death, the rate of these 2 events being sequentially greater for patients with predominantly deep orbital lymphoma, lacrimal gland lymphoma, or eyelid lymphoma. [61, 60]

With radiotherapy for orbital disease, the 5-year disease-free survival and overall survival rate has been between 65-73.6% and 65.5-78%. [47, 53] Most relapses occur in the first 2 years after therapy. The risk of late relapse is higher in patients with a divergent histology of both indolent disease and aggressive disease.

Intraocular lymphoma can lead to blindness due to damage of the intraocular structures involved. Orbital lymphoma can cause blindness in untreated or aggressive cases with severe proptosis leading to corneal complications or optic neuropathy. Local irradiation can also cause reversible blindness due to cataract and irreversible blindness in cases of radiation-induced optic neuropathy and retinopathy.

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