Choriocarcinoma of the central nervous system
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare primary germ cell tumor of central nervous system characterized by a lesion typically in the region of the pineal gland and the suprasellar compartment, composed of cytotrophoblastic elements and multinucleated syncytiotrophoblastic giant cells. Ectatic stromal vascular channels, blood lakes, and extensive hemorrhagic necrosis are the rule. The tumor usually arises in the second decade of life and predominantly in males. Clinical presentation depends on location and size and includes signs of increased intracranial pressure, visual disturbances, and endocrine abnormalities. Prognosis is generally poor.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Choriocarcinoma of the central nervous system | |
| fr | Choriocarcinome du système nerveux central | |
| de | Chorionkarzinom des Zentralnervensystems | |
| es | Coriocarcinoma del sistema nervioso central | |
| it | Coriocarcinoma del sistema nervoso centrale | |
| nl | Choriocarcinoom van centraal zenuwstelsel | |
| pt | Coriocarcinoma do sistema nervoso central | |
| pl | Nabłoniak kosmówkowy centralnego układu nerwowego | |
| cs | Choriokarcinom centrálního nervového systému | |
| uk | Хоріокарцинома центральної нервової системи | |
| zh | 中枢神经系统绒毛膜癌 | |
| tr | Merkezi sinir sisteminin koryokarsinomu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C71.2 | NTBT | attributed | not yet validated |
| ICD-11 | 2A00.1Y | NTBT | index | validated |
| MONDO | 0016740 | E | validated | |
| UMLS | C1332876 | 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:251995Primary germ cell tumor of central nervous system
rare neurological diseases; rare neoplastic diseases; rare transplant-related disorders
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/252015
- This record as JSON: /data/d/252015.json