Primary oculocerebral lymphoma
Synonyms: Primary oculocerebral non-Hodgkin lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Primary oculocerebral lymphoma is a rare, primary, organ-specific, extranodal non-Hodgkin's lymphoma (typically diffuse large B-cell lymphoma), simultaneously affecting the intraocular compartments (retina, vitreous, optic nerve, uvea and others) and the central nervous system (commonly the cerebellum, spinal cord or pia mater). The presenting symptoms vary depending on the localization of the tumor and may include vitreous floaters or blurred vision, raised intracranial pressure (headache, vomiting, papilledema) and/or focal neurological deficits.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary oculocerebral lymphoma | Primary oculocerebral non-Hodgkin lymphoma |
| fr | Lymphome oculo-cérébral primitif | LOCP · Lymphome oculo-cérébral primitif non hodgkinien |
| de | Lymphom, okulozerebrales primäres | Non-Hodgkin-Lymphom, okulozerebrales primäres · Okulozerebrales NHL |
| es | Linfoma oculocerebral primario | Linfoma no Hodgkin oculocerebral primario |
| it | Linfoma oculocerebrale primitivo | Linfoma non-Hodgkin oculocerebrale primitivo |
| nl | Primair oculocerebraal lymfoom | Primair oculocerebraal non-Hodgkin-lymfoom |
| pt | Linfoma oculocerebral primário | Linfoma não-Hodgkin oculocerebral primário |
| pl | Pierwotny chłoniak oczno-mózgowy | = en |
| cs | Primární okulocerebrální lymfom | Primární okulocerebrální non-Hodgkinský lymfom |
| uk | Первинна окулоцеребральна лімфома | Первинна окулоцеребральна неходжкінська лімфома |
| zh | 原发性眼脑淋巴瘤 | 原发性眼脑非霍奇金淋巴瘤 |
| tr | Primer oküloserebral lenfoma | Primer oküloserebral Hodgkin olmayan lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C85.7 | NTBT | attributed | validated |
| ICD-11 | 2B33.5 | NTBT | index | validated |
| MONDO | 0017205 | E | validated | |
| UMLS | C5679779 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (3):
- ORPHA:279911Primary organ-specific lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:101950Rare eye tumor
rare ophthalmic disorders; rare neoplastic diseases - ORPHA:98062Rare nervous system tumor
rare neurological diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/279897
- This record as JSON: /data/d/279897.json