Desmoplastic infantile astrocytoma/ganglioglioma
Synonyms: DIA/DIG
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Infancy, Neonatal
- Status
- active
Desmoplastic infantile astrocytoma/ganglioglioma are mixed neuronal-glial tumors representing a histological spectrum of the same tumor. They are usually supratentorially located, large, cystic masses with a peripheral solid component, characterized by prominent desmoplastic stroma and pleomorphic populations of neoplastic cells with either astrocytic or ganglionic differentiation and poorly differentiated cells in variable proportions. They usually present in the first 18 months of age with rapid head growth, bulging anterior fontanel and bone structures over the tumor, signs of raised intracranial pressure (headache, vomiting, papilledema), focal neurological signs and sometimes seizures.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Desmoplastic infantile astrocytoma/ganglioglioma | DIA/DIG |
| fr | Astrocytome/gangliogliome desmoplasique infantile | = en |
| de | Astrozytom/Gangliogliom, desmoplastisches, infantiles | = en |
| es | Ganglioglioma/astrocitoma desmoplásico infantil | = en |
| it | Astrocitoma/ganglioglioma desmoplastico infantile | = en |
| nl | Desmoplastisch infantiel astrocytoom/ganglioglioom | = en |
| pt | Astrocitoma/ganglioglioma desmoplásico da infância | = en |
| pl | Desmoplastyczny gwiaździak/zwojakoglejak wieku dziecięcego | = en |
| cs | Desmoplastický astrocytom/gangliogliom dětského věku | = en |
| uk | Десмопластична інфантильна астроцитома/гангліогліома | = en |
| zh | 促结缔组织增生性婴儿星形细胞瘤/神经节神经胶质瘤 | = en |
| tr | Desmoplastik infantil astrositom / gangliogliom | DIA / DIG |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D33.0 | NTBT | attributed | not yet validated |
| ICD-11 | 2A00.21 | NTBT | index | validated |
| MONDO | 0016731 | E | validated | |
| UMLS | C4757951 | 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:251934Mixed neuronal-glial tumor
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/251940
- This record as JSON: /data/d/251940.json