Ectopic aldosterone-producing tumor
Synonyms: Extra-adrenal aldosterone-producing tumor
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Ectopic aldosterone-producing tumor is an extremely rare aldosterone-producing neoplasm composed of aberrant adrenocortical tissue located outside the adrenal glands (e.g. in retroperitoneum, perirenal or periaortic fatty tissue, thorax, spinal canal, testes, ovaries) typically characterized by symptoms related to increased aldosterone levels (such as sustained, treatment-resistant hypertension and hypokalemia) or symptoms caused by local tumor enlargement.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Ectopic aldosterone-producing tumor | Extra-adrenal aldosterone-producing tumor |
| fr | Tumeur ectopique sécrétrice d'aldostérone | Tumeur extra-surrénalienne sécrétrice d'aldostérone |
| de | Tumor, Aldosteron-produzierender ektopischer | Extraadrenaler Aldosteron-produzierender Tumor |
| es | Tumor ectópico productor de aldosterona | Tumor extrasuprarrenal productor de aldosterona |
| it | Tumore ectopico secernente aldosterone | Tumore extra-surrenalico secernente aldosterone |
| nl | Ectopische aldosteron-producerende tumor | Extra-adrenale aldosteron-producerende tumor |
| pt | Tumor produtor de aldosterona ectópico | Tumor produtor de aldosterona extra-supra-renal |
| pl | Guz ektopowy wydzielający aldosteron | = en |
| cs | Ektopický aldosteron produkující karcinom | Extra-adrenální aldosteron produkující karcinom |
| uk | Ектопічна альдостерон-продукуюча пухлина | Позанадниркова пухлина, що продукує альдостерон |
| zh | 异位醛固酮肿瘤 | 肾上腺外醛固酮肿瘤 |
| tr | Ektopik aldosteron üreten tümör | Adrenal dışı aldosteron-üreten tümör |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E26.8 | NTBT | attributed | validated |
| ICD-11 | 5A72.0 | NTBT | attributed | validated |
| MONDO | 0016506 | E | validated | |
| UMLS | C4755311 | 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:231641Rare non surgically correctable form of primary aldosteronism
rare endocrine 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
13 annotations · validated 2016-08-31| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0003351 | Decreased circulating renin concentration | |
| HP:0011740 | Glucocortocoid-insensitive primary hyperaldosteronism | |
| HP:0000822 | Hypertension | |
| Very frequent (99-80%) | ||
| HP:0002315 | Headache | |
| HP:0002900 | Hypokalemia | |
| HP:0100615 | Ovarian neoplasm | |
| HP:0006735 | Renal cortical adenoma | |
| Frequent (79-30%) | ||
| HP:0200114 | Metabolic alkalosis | |
| Occasional (29-5%) | ||
| HP:0000421 | Epistaxis | |
| HP:0001324 | Muscle weakness | |
| HP:0002018 | Nausea | |
| HP:0000360 | Tinnitus | |
| Excluded (0%) | ||
| HP:0008256 | Adrenocortical adenoma | |
Run the differential on the 7 obligate / very frequent terms
Annotation source: 8639013[PMID]_15657580[PMID]_8787960[PMID]_26934393[PMID]_27036860[PMID]_171276[PMID]_3518689[PMID]_2344975[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/231632
- This record as JSON: /data/d/231632.json