Primary intraocular lymphoma

Primary Intraocular Lymphoma (PIOL) is a rare form of eye cancer that originates within the eye. It is a subtype of primary central nervous system lymphoma (PCNSL), which is a type of non-Hodgkin lymphoma (NHL). PIOL primarily affects the retina and vitreous of the eye, but can also involve the optic nerve and choroid.

Epidemiology

PIOL is a rare disease, accounting for less than 1% of all intraocular tumors and less than 1% of all NHLs. It is most commonly diagnosed in individuals aged 50-80 years, with a slight female predominance.

Pathophysiology

The exact cause of PIOL is unknown. However, it is believed to be associated with immune system dysfunction, particularly in individuals with immunodeficiency conditions such as HIV/AIDS or those receiving immunosuppressive therapy.

Clinical Presentation

Patients with PIOL typically present with decreased vision, floaters, and photopsia. On examination, vitreous haze and retinal detachment may be observed. Intraocular pressure may be normal or slightly elevated.

Diagnosis

Diagnosis of PIOL is challenging due to its nonspecific clinical presentation and the need for invasive procedures for definitive diagnosis. Vitreous biopsy is the most common diagnostic procedure, but retinal biopsy may also be performed. Immunohistochemistry and flow cytometry are used to identify the lymphoma cells.

Treatment

Treatment of PIOL involves a combination of chemotherapy, radiotherapy, and immunotherapy. The choice of treatment depends on the extent of the disease and the patient's overall health status.

Prognosis

The prognosis of PIOL is generally poor, with a median survival of 3-5 years. Factors associated with a worse prognosis include older age, poor performance status, and involvement of the central nervous system.

See Also

• Lymphoma
• Non-Hodgkin lymphoma
• Primary central nervous system lymphoma
• Eye cancer