Intraocular lymphoma
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:
- [group1]
- [group2]
- [group3]
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].