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. Interestingly, most cases of PIOL eventuate to central nervous system involvement (PCNSL) while only 20% of PCNSL lead to intraocular (PIOL) involvement. PIOL and PCNSL remain enigmas because both structures are immunologically privileged sites (the brain sits behind the blood-brain barrier and the retina sits behind the blood-retina barrier) and so do not normally have immune cells traficking through these structures. What is more, while the vast majority of PCNSL in patients with acquired immune deficiency syndrome (AIDS) is related to the Epstein-Barr virus (EBV), the development of PCNSL and PIOL in immunocompetent patients is unknown and shows no general relation to infectious DNAs
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:
Causes
- Common causes of intraocular lymphoma, include:
Differentiating Intraocular Lymphoma from Other Diseases
- Intraocular lymphoma must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], 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
- Intraocular lymphoma is usually asymptomatic.
- Symptoms of intraocular lymphoma may include the following:
- [symptom 1]
- [symptom 2]
- [symptom 3]
- [symptom 4]
- [symptom 5]
- [symptom 6]
Physical Examination
- Patients with intraocular lymphoma usually appear [general appearance].
- Physical examination may be remarkable for:
- [finding 1]
- [finding 2]
- [finding 3]
- [finding 4]
- [finding 5]
- [finding 6]
Laboratory Findings
- There are no specific laboratory findings associated with intraocular lymphoma.
Imaging Findings
- On CT, 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].