Intraocular lymphoma: Difference between revisions
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==Classification== | ==Classification== | ||
*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 | |||
*Primary central nervous system lymphoma may be classified into 5 subtypes: | *Primary central nervous system lymphoma may be classified into 5 subtypes: | ||
:*Primary cerebral lymphoma | :*Primary cerebral lymphoma | ||
Revision as of 21:40, 9 May 2016
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
- 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
- Primary central nervous system lymphoma may be classified into 5 subtypes:
- 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:
- No remarkable findings
- On microscopic histopathological analysis, characteristic findings of intraocular lymphoma, include:
- Atypical lymphocytes (gold standard)
Causes
- There are no established causes of intraocular lymphoma.
Differentiating Intraocular Lymphoma from Other Diseases
- Intraocular lymphoma must be differentiated from other diseases that cause chronic loss of vision, or headaches, such as:
Epidemiology and Demographics
- The prevalence of intraocular lymphoma remains unknown.
Age
- Intraocular lymphoma is more commonly observed among patients aged 50 to 60 years old.
- Intraocular lymphoma is more commonly observed among middle aged adults.
Gender
- Males are more commonly affected with intraocular lymphoma than females.
Race
- There is no racial predilection for 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
- Severe symptoms of intraocular lymphoma, may include:
- Aphasia
- Seizures
- Ataxia
- Visual field defect
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].