Conjunctival malignant melanoma
Synonyms: Conjunctival melanoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare eye tumor characterized by a flat to nodular conjunctival mass with variable pigmentation. It can arise in any part of the conjunctiva but most often presents on the bulbar surface, near the limbus. Invasion of corneal tissue, eyelid margin, sclera or orbit may occur in advanced tumors. Metastatic spread most commonly involves regional lymph nodes or the lung, liver, and brain. Essential diagnostic criteria are stromal invasion and severe cytological and architectural atypia exceeding those expected in a naevus. Desirable criteria are demonstration of melanocytic markers by immunohistochemistry, pagetoid growth, mitotic activity, invasion into cornea or skin, ulceration, and tumor necrosis.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Conjunctival malignant melanoma | Conjunctival melanoma |
| fr | Mélanome malin de la conjonctive | Mélanome conjonctival · Mélanome de la conjonctive |
| de | Malignes Melanom der Bindehaut | Konjunktivales Melanom |
| es | Melanoma conjuntival maligno | |
| it | Melanoma maligno della congiuntiva | Melanoma della congiuntiva |
| nl | Maligne melanoom van conjunctiva | Conjunctivaal melanoom · Conjunctivamelanoom |
| pt | Melanoma maligno da conjuntiva | Melanoma da conjuntiva |
| pl | Czerniak złośliwy spojówki | = en |
| cs | Maligní melanom spojivky | = en |
| uk | Злоякісна меланома конюнктиви | Меланома конюнктиви |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C69.0 | NTBT | index | validated |
| ICD-11 | 2D00.0 | E | specific | validated |
| MONDO | 0002096 | E | validated | |
| UMLS | C0346360 | E | validated | |
| GARD | 10744 | E | validated | |
| MedDRA | 10066384 | 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:101950Rare eye tumor
rare ophthalmic disorders; 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 |
|---|---|---|---|---|---|---|
| Cases/families | 32 cases | Worldwide | Case(s) | Validated | 31768361[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 31768361[PMID]_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/617910
- This record as JSON: /data/d/617910.json