Primary melanoma of the central nervous system
Synonyms: Malignant melanoma of meninges · Primary melanoma of the CNS
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
Primary melanoma of the central nervous system is a rare tumor of meninges arising from leptomeningeal melanocytes, typically in the perimedullary or high cervical region, in the absence of melanoma outside the CNS. The tumor is typically a darkly pigmented, solid mass, often containing hemorrhagic or necrotic areas, composed of sheets of pleomorphic cells with prominent nucleoli, with frequent mitotic figures and parenchymal invasion. Intracranial tumor may present with signs of raised intracranial pressure, focal neurological symptoms related to tumor location, seizures or subarachnoid hemorrhage, spinal tumor may present with back pain, muscle weakness, numbness, plegia or urinary incontinence.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary melanoma of the central nervous system | Malignant melanoma of meninges · Primary melanoma of the CNS |
| fr | Mélanome primitif du système nerveux central | Mélanome primitif du SNC |
| de | Primäres Melanom des Zentralnervensystems | Malignes Melanom der Meningen · Primäres Melanom des ZNS |
| es | Melanoma primario del sistema nervioso central | Melanoma maligno de meníngeo · Melanoma primario del SNC |
| it | Melanoma primitivo del sistema nervoso centrale | |
| nl | Primair melanoom van centraal zenuwstelsel | Maligne melanoom van hersenvliezen · Primair melanoom van CZS |
| pt | Melanoma primário do sistema nervoso central | |
| pl | Pierwotny czerniak ośrodkowego układu nerwowego | = en |
| cs | Primární melanom centrálního nervového systému | = en |
| uk | Первинна меланома центральної нервової системи | Злоякісна меланома мозкових оболонок · Первинна меланома ЦНС |
| zh | 中枢神经系统原发性黑色素瘤 | 中枢神经系统恶性黑色素瘤 |
| tr | Merkezi sinir sisteminin primer melanomu | CNS'nin primer melanomu · Meninkslerin habis melanomu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C70.9 | NTBT | attributed | validated |
| ICD-11 | 2A0Z | NTBT | index | validated |
| MONDO | 0016747 | E | validated | |
| UMLS | C0349626 | E | validated | |
| GARD | 12016 | 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 (1):
- ORPHA:252028Primary melanocytic tumor of central nervous system
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 |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.007 / 100 000 | Worldwide | Value and class | 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/252050
- This record as JSON: /data/d/252050.json