Primary central nervous system lymphoma
Synonyms: PCNSL · Primary CNS lymphoma · Primary brain lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
Primary central nervous system lymphoma (PCNSL) is a rare nervous system tumor, predominantly due to diffuse large B-cell lymphoma, that involves brain, leptomeninges, eyes, or rarely spinal cord, in the absence of systemic diffusion at the time of diagnosis. It is characterized by a solitary tumor that, depending on its location, can lead to a variety of symptoms such as headache, nausea, vomiting (and other signs of raised intracranial pressure), focal neurologic deficits, neuropsychiatric and ocular symptoms, seizures and personality changes.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary central nervous system lymphoma | PCNSL · Primary CNS lymphoma · Primary brain lymphoma |
| fr | Lymphome primitif du système nerveux central | Lymphome cérébral primitif · Lymphome primitif du SNC · PCNSL |
| de | Primäres Lymphom des Zentralnervensystems | PZNSL · ZNS-Lymphom, primäres |
| es | Linfoma primario del sistema nervioso central | Linfoma cerebral primario · Linfoma del SNC primario · PCNSL |
| it | Linfoma primitivo del sistema nervoso centrale | Linfoma cerebrale primitivo · Linfoma primitivo del CNS · PCNSL |
| nl | Primair lymfoom van centraal zenuwstelsel | PCZSL · Primair CZS-lymfoom · Primair hersenlymfoom |
| pt | Linfoma primário do sistema nervoso central | Linfoma primário CNS · Linfoma primário cerebral · PCNSL |
| pl | Pierwotny chłoniak ośrodkowego układu nerwowego | PCNSL · Pierwotny chłoniak CUN · Pierwotny chłoniak mózgu |
| cs | Primární lymfom centrálního nervového systému | PCNSL · Primární lymfom CNS · Primární mozkový lymfom |
| uk | Первинна лімфома центральної нервової системи | PCNSL · Первинна лімфома головного мозку · Первинна лімфома ЦНС |
| zh | 原发性中枢神经系统淋巴瘤 | 原发性脑淋巴瘤 |
| tr | Primer santral sinir sistemi lenfoması | PCNSL · Primer CNS lenfoma · Primer beyin lenfoması |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.3 | NTBT | attributed | validated |
| ICD-11 | 2A8Z | NTBT | index | validated |
| MONDO | 0002571 | E | validated | |
| UMLS | C0280803 | E | validated | |
| GARD | 9318 | E | validated | |
| MedDRA | 10036685 | 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:289644Epstein-Barr virus-associated malignant lymphoproliferative disorder
rare neoplastic diseases - ORPHA:279911Primary organ-specific lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders - 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
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.44 / 100 000 | United States | Value and class | Validated | Central Brain Tumor OTHERistry of the United States 2010-2014[OTHER]_29117289[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.26 / 100 000 | Sweden | Value and class | Validated | 28983970[PMID] |
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/46135
- This record as JSON: /data/d/46135.json