Astrocytoma grade 3
Synonyms: Anaplastic astrocytoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare, high-grade, malignant glial tumor, histologically characterized by abundance of pleomorphic astrocytes and multiple mitotic figures, often associated with diffuse infiltration of the surrounding tissue, considerable edema and mass effect and involvement of the contralateral brain. Depending on the primary localization of the tumor, patients can present with signs of raised intracranial pressure (headache, vomiting, papilledema), seizures, progressive neurological deficits, and/or behavioral changes. The tumor is most commonly localized in the frontal and temporal lobes, brain stem and spinal cord.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Astrocytoma grade 3 | Anaplastic astrocytoma |
| fr | Astrocytome de grade 3 | Astrocytome anaplasique |
| de | Astrozytom Grad 3 | Astrozytom, anaplastisches |
| es | Astrocitoma de grado 3 | Astrocitoma anaplásico |
| it | Astrocitoma di grado 3 | Astrocitoma anaplastico |
| nl | Astrocytoom graad 3 | Anaplastisch astrocytoom |
| pt | Astrocitoma anaplásico | |
| pl | Gwiaździak anaplastyczny | = en |
| cs | Anaplastický astrocytom | = en |
| uk | Анапластична астроцитома | |
| zh | 间变性星形细胞瘤 | |
| tr | Anaplastik astrositom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C71.9 | NTBT | index | validated |
| ICD-11 | 2A00.0Y | NTBT | index | validated |
| ICD-11 | XH96C7 | E | specific | validated |
| MONDO | 0016684 | E | validated | |
| UMLS | C0334579 | E | validated | |
| GARD | 5860 | E | validated | |
| MedDRA | 10002224 | 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:251561High-grade astrocytoma
rare neurological diseases; rare neoplastic diseases
No children.
Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| IDH2 | Biomarker tested in | Assessed | HGNC:5383 | ENSG00000182054 | 147650 | 15q26.1 | isocitrate dehydrogenase (NADP(+)) 2 | 24993250[PMID]_19228619[PMID]_19554337[PMID]_23109653[PMID]_24149775[PMID]_20714900[PMID]_21075857[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.4 / 100 000 | United States | Value and class | Validated | Central Brain Tumor OTHERistry of the United States 2010-2014[OTHER]_29117289[PMID] |
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/251589
- This record as JSON: /data/d/251589.json