Intraocular lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: PIOL

Overview

Intraocular lymphoma (also known as "PIOL") is a rare subtype of primary central nervous system lymphoma. Intraocular lymphoma may affect the eye secondarily from a metastasis from a non-ocular tumor or may arise within the eye primarily. According to the World Health Organization (WHO) classification, intraocular lymphomas are most commonly a diffuse large B-cell immunohistologic subtype of non-Hodgkin's lymphoma. The most common symptoms of intraocular lymphoma is blurred or decreased vision. In the majority of patients, central nervous system involvement, only accounts for 20% of primary central nervous system lymphomas. The median age at diagnosis among patients with intraocular lymphoma is between 50-60 years. The pathogenesis of intraocular lymphoma is characterized by the affection of the sub-retinal pigment epithelium, which is normally involved in the light absorption. On fluorescein angiography, findings may reveal "leopard spot" patterns due to sub-retinal pigment epithelium infiltrates that stain early and progressively or mottling of the sub-retinal pigment epithelium due to hyper- and hypo-fluorescent window defects. The initial therapy for patients with intraocular lymphoma is corticosteroids.

Historical Perspective

• Intraocular lymphoma was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].

Classification

• Intraocular lymphoma may be classified according to [classification method] into [number] subtypes/groups:

• Primary cerebral lymphoma
• Primary leptomeningeal lymphoma
• Primary intraocular lymphoma
• Primary spinal lymphoma
• Neurolymphomatosis

Pathophysiology

• The pathogenesis of intraocular lymphoma is characterized by
• Genes associated with the development of intraocular lymphoma, include:
• On gross pathology, characteristic findings of intraocular lymphoma, include:
• On microscopic histopathological analysis, characteristic findings of intraocular lymphoma, include:

• Atypical lymphocytes (gold standard)

Causes

• Common causes of intraocular lymphoma, include:

Differentiating Intraocular Lymphoma from Other Diseases

• Intraocular lymphoma must be differentiated from other diseases that cause chronic loss of vision, such as:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics

• The prevalence of intraocular lymphoma is approximately [number or range] per 100,000 individuals worldwide.
• In [year], the incidence of intraocular lymphoma was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

• Patients of all age groups may develop intraocular lymphoma.

• Intraocular lymphoma is more commonly observed among patients aged [age range] years old.
• Intraocular lymphoma is more commonly observed among [elderly patients/young patients/children].

Gender

• Intraocular lymphoma affects men and women equally.

• [Gender 1] are more commonly affected with intraocular lymphoma than [gender 2].
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

• There is no racial predilection for intraocular lymphoma.

• Intraocular lymphoma usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop intraocular lymphoma.

Risk Factors

• Common risk factors in the development of intraocular lymphoma, include:

Natural History, Complications and Prognosis

• The majority of patients with intraocular lymphoma are symptomatic at the time of diagnosis.
• Early clinical features include
• If left untreated, patients with intraocular lymphoma may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of intraocular lymphoma include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally good, and the median survival rate of patients with intraocular lymphoma is approximately

Diagnosis

Diagnostic Criteria

• The diagnosis of intraocular lymphoma is made with the following diagnostic criteria:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• Symptoms of intraocular lymphoma may include the following:

• Burning of the eye
• Redness of the eye
• Blurred vision
• Photophobia or sensitivity to light
• Eye pain
• Floaters (which are dark spots that float in the visual field)
• Headache

Physical Examination

• Patients with intraocular lymphoma usually appear pale or malnourished.
• Physical examination may be remarkable for:

• Decreased visual acuity (most common)
• Irregular pupil
• Increased lacrimation
• Eye redness
• Increased intraocular pressure

Laboratory Findings

• There are no specific laboratory findings associated with intraocular lymphoma.

Imaging Findings

• Magnetic resonance image (MRI) is the imaging modality of choice for patients with intraocular lymphoma.
• On MRI, characteristic findings of intraocular lymphoma, include:

Treatment

Medical Therapy

• There is no treatment for intraocular lymphoma; the mainstay of therapy is supportive care.

• The mainstay of therapy for intraocular lymphoma is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for intraocular lymphoma.
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of intraocular lymphoma.
• [Surgical procedure] can only be performed for patients with [disease stage] intraocular lymphoma.

Prevention

• There are no primary preventive measures available for intraocular lymphoma.
• Once diagnosed and successfully treated, patients with intraocular lymphoma are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].