Astrocytoma grade 2
Synonyms: Diffuse astrocytoma
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
A rare low-grade astrocytoma characterized by a high degree of cellular differentiation, slow growth, and diffuse infiltration of adjacent brain structures, and corresponding to WHO grade II. The tumor typically affects young adults and has an intrinsic tendency for progression to high-grade glioma. Histological variants are fibrillary, gemistocytic, and protoplasmic astrocytoma. Patients most commonly present with seizures, but also with other neurological or neuropsychological abnormalities, depending on the location.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Astrocytoma grade 2 | Diffuse astrocytoma |
| fr | Astrocytome de grade 2 | Astrocytome diffus |
| de | Astrozytom Grad 2 | Astrozytom, diffuses |
| es | Astrocitoma de grado 2 | Astrocitoma difuso |
| it | Astrocitoma di grado 2 | Astrocitoma diffuso |
| nl | Astrocytoom graad 2 | Diffuus astrocytoom |
| pt | Astrocitoma difuso | |
| pl | Gwiaździak rozlany | = en |
| cs | Difuzní astrocytom | = en |
| uk | Дифузна астроцитома | |
| zh | 弥漫性星形细胞瘤 | |
| tr | Yaygın astrositom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D33.2 | NTBT | attributed | validated |
| ICD-11 | 2A00.0Y | NTBT | index | validated |
| MONDO | 0016686 | E | validated | |
| UMLS | C0280785 | E | validated | |
| GARD | 5907 | 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:251592Low-grade astrocytoma
rare neurological diseases; rare neoplastic diseases
Genes
0 direct · 3 via subtypesOrphanet records no gene directly on this code.
Recorded by Orphanet on subtypes of this disorder:
| Gene | Subtype | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|---|
| IDH2 | ORPHA:251598Protoplasmic astrocytoma | Biomarker tested in | Assessed | HGNC:5383 | ENSG00000182054 | 147650 | 15q26.1 | isocitrate dehydrogenase (NADP(+)) 2 | 19228619[PMID]_19554337[PMID]_23109653[PMID]_24149775[PMID]_20714900[PMID]_21075857[PMID] |
| IDH2 | ORPHA:251601Fibrillary astrocytoma | Biomarker tested in | Assessed | HGNC:5383 | ENSG00000182054 | 147650 | 15q26.1 | isocitrate dehydrogenase (NADP(+)) 2 | 19228619[PMID]_19554337[PMID]_23109653[PMID]_24149775[PMID]_20714900[PMID]_21075857[PMID] |
| IDH2 | ORPHA:251604Gemistocytic astrocytoma | Biomarker tested in | Assessed | HGNC:5383 | ENSG00000182054 | 147650 | 15q26.1 | isocitrate dehydrogenase (NADP(+)) 2 | 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 | 0.48 / 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/251595
- This record as JSON: /data/d/251595.json