Pulmonary blastoma
Synonyms: Pneumoblastoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial
- Age of onset
- Adult
- Status
- active
A biphasic primary lung neoplasm, belonging to the group of sarcomatoid lung carcinomas (SLCs). The tumor contains both an epithelial well-differentiated component, showing tubular architecture resembling the normal fetal lung, and a mesenchymal undifferentiated stroma with a so-called ''blastema-like'' configuration that resembles an embryonic lung.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pulmonary blastoma | Pneumoblastoma |
| fr | Blastome pulmonaire | Pneumoblastome |
| de | Pulmonales Blastom | Pneumoblastom |
| es | Blastoma pulmonar | = en |
| it | Blastoma polmonare | = en |
| nl | Pulmonaal blastoom | Pneumoblastoom |
| pt | Pneumoblastoma | Blastoma pulmonar |
| pl | Blastoma płuc | = en |
| cs | Plicní blastom | Pneumoblastom |
| uk | Бластома легень | Пневмобластома |
| zh | 肺胚细胞瘤 | 肺母细胞瘤 |
| tr | Pulmoner blastom | Pnömoblastom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C34.1 | BTNT | attributed | validated |
| ICD-10 | C34.2 | BTNT | attributed | validated |
| ICD-10 | C34.3 | BTNT | attributed | validated |
| ICD-10 | C34.8 | BTNT | attributed | validated |
| ICD-10 | C34.9 | BTNT | attributed | validated |
| ICD-11 | 2C25.Y | NTBT | index | validated |
| ICD-11 | XH5VH1 | E | specific | validated |
| MONDO | 0005933 | E | validated | |
| MeSH | D018202 | E | validated | |
| UMLS | C0206629 | 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:101945Rare bronchopulmonary and pleural cavity tumors
rare respiratory diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
8 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 350 cases | Worldwide | Case(s) | Validated | [EXPERT] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET |
| Annual incidence | <1 / 1 000 000 | — | Europe | Class only | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.002 / 100 000 | Belgium | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.005 / 100 000 | Finland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.001 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.002 / 100 000 | Italy | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.002 / 100 000 | Netherlands | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
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
9 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0100749 | Chest pain | |
| HP:0012735 | Cough | |
| HP:0002105 | Hemoptysis | |
| HP:0100528 | Pleuropulmonary blastoma | |
| HP:0002113 | Pulmonary infiltrates | |
| Frequent (79-30%) | ||
| HP:0002094 | Dyspnea | |
| HP:0001945 | Fever | |
| HP:0006532 | Recurrent pneumonia | |
| HP:0001824 | Weight loss | |
Run the differential on the 5 obligate / very frequent terms
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/64741
- This record as JSON: /data/d/64741.json