Diffuse leptomeningeal melanocytosis
Synonyms: DLM · Leptomeningeal melanomatosis
- Type
- Disease
- Group
- Disorder
- Status
- active
Diffuse leptomeningeal melanocytosis is a rare tumor of meninges arising from leptomeningeal melanocytes, characterized by diffuse infiltration of the leptomeninges (pia mater and arachnoidea) anywhere in the central nervous system. Clinical features may include stillbirth, intracranial hypertension and hydrocephalus, seizure, ataxia, syringomyelia, cranial nerve palsy, intracranial haemorrhage, sphincter dysfunction and neuropsychiatric symptoms. Transformation into malignant melanoma of the central nervous system was reported. It may be associated with congenital nevi, as a part of neurocutaneous melanosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Diffuse leptomeningeal melanocytosis | DLM · Leptomeningeal melanomatosis |
| fr | Mélanocytose leptoméningée diffuse | DLM · Mélanomatose leptoméningée |
| de | Melanozytose, diffuse leptomeningeale | DLM · Melanomatose, leptomeningeale |
| es | Melanocitosis leptomeníngea difusa | DLM · Melanomatosis leptomeníngea |
| it | Melanocitosi diffusa delle leptomeningi | Melanomatosi leptomeningea · DLM |
| nl | Diffuse leptomeningeale melanocytose | DLM · Leptomeningeale melanomatose |
| pt | Melanocitose leptomeníngea difusa | DLM · Melanomatose leptomeníngea |
| pl | Rozlana czerniakowatość opon mózgowych | DLM |
| cs | Difuzní leptomeningeální melanocytóza | DLM · Leptomeningeální melanomatóza |
| uk | Дифузний лептоменінгеальний меланоцитоз | DLM · Лептоменінгеальний меланоматоз |
| zh | 弥漫性软脑膜黑素细胞增多症 | 软脑膜黑色素瘤病 |
| tr | Yaygın leptomeninjiyal melanositoz | DLM · Leptomeningeal melanomatoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C70.9 | NTBT | attributed | validated |
| ICD-11 | 2A01.0Y | NTBT | attributed | validated |
| MONDO | 0016745 | E | validated | |
| UMLS | C4749348 | 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
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/252031
- This record as JSON: /data/d/252031.json