ORPHA:231625
Adrenocortical carcinoma with pure aldosterone hypersecretion
Synonyms: Pure APAC · Pure aldosterone-producing adrenocortical carcinoma · Pure aldosterone-secreting adrenocortical carcinoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A very rare surgically-correctable form of primary aldosteronism (PA) due to an aldosterone-secreting adrenal malignancy.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Adrenocortical carcinoma with pure aldosterone hypersecretion | Pure APAC · Pure aldosterone-producing adrenocortical carcinoma · Pure aldosterone-secreting adrenocortical carcinoma |
| fr | Carcinome corticosurrénalien à hypersécrétion pure d'aldostérone | Corticosurrénalome malin avec excès d'aldostérone |
| de | Adrenokortikales Karzinom mit isolierter Aldosteron-Hypersekretion | |
| es | Carcinoma corticosuprarrenal con hipersecreción pura de aldosterona | APAC pura · Carcinoma corticosuprarrenal productor puro de aldosterona · Carcinoma corticosuprarrenal secretor puro de aldosterona |
| it | Carcinoma cortico-surrenalico con ipersecrezione di aldosterone puro | APAC puro · Carcinoma cortico-surrenalico producente aldosterone puro · Carcinoma cortico-surrenalico secernente aldosterone puro |
| nl | Bijnierschorscarcinoom met zuivere aldosteronhypersecretie | Adrenocorticaal carcinoom met zuivere aldosteronhypersecretie · Zuiver APAC · Zuiver aldosteronproducerend bijnierschorscarcinoom · Zuiver aldosteronsecreterend bijnierschorscarcinoom |
| pt | Carcinoma adrenocortical produtor de aldosterona, puro | |
| pl | Rak kory nadnerczy z hipersekrecją czystego aldosteronu | Czysty APAC · Czysty rak kory nadnerczy produkujący aldosteron · Czysty rak kory nadnerczy wydzielający aldosteron |
| cs | Adrenokortikální karcinom s čistou aldosteronovou hypersekrecí | Čistý APAC · Čistý aldosteron-produkující adrenokortikální karcinom · Čistý aldosteron-vyměšující adrenokortikální karcinom |
| uk | Адренокортикальна карцинома з гіперсекрецією чистого альдостерону | Чиста адренокортикальна карцинома, що продукує альдостерон · Чиста адренокортикальна карцинома, що секретує альдостерон · Чистий APAC |
| zh | 肾上腺皮质癌伴单纯醛固酮分泌过多 | 单纯性醛固酮分泌过多性肾上腺皮质癌 |
| tr | Saf aldosteron hipersekresyonlu adrenokortikal karsinom | Saf APAC · Saf aldosteron-üreten adrenokortikal karsinom · Saf aldosteron-salgılayan adrenokortikal karsinom |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C74.0 | NTBT | attributed | validated |
| ICD-11 | 5A72.0 | NTBT | index | validated |
| MONDO | 0016505 | E | validated | |
| MONDO | 16505 | E | validated | |
| UMLS | C3839852 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:231637Rare surgically correctable form of primary aldosteronism
rare endocrine diseases
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
6
HPO phenotypes
18 annotations · validated 2016-08-29| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0000822 | Hypertension | |
| HP:0100631 | Neoplasm of the adrenal gland | |
| Very frequent (99-80%) | ||
| HP:0003351 | Decreased circulating renin concentration | |
| HP:0011740 | Glucocortocoid-insensitive primary hyperaldosteronism | |
| HP:0002900 | Hypokalemia | |
| HP:0003118 | Increased circulating cortisol level | |
| HP:0200114 | Metabolic alkalosis | |
| HP:0001324 | Muscle weakness | |
| Frequent (79-30%) | ||
| HP:0005135 | Abnormal T-wave | |
| HP:0003081 | Increased urinary potassium | |
| HP:0003394 | Muscle spasm | |
| HP:0002018 | Nausea | |
| HP:0001962 | Palpitations | |
| HP:0003401 | Paresthesia | |
| Occasional (29-5%) | ||
| HP:0000421 | Epistaxis | |
| HP:0002315 | Headache | |
| HP:0002170 | Intracranial hemorrhage | |
| HP:0000360 | Tinnitus | |
Run the differential on the 8 obligate / very frequent terms
Annotation source: 20482833[PMID]_26273475[PMID]_8639013[PMID]_21270113[PMID]_24682757[PMID]_24103295[PMID]_11559548[PMID]
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/231625
- This record as JSON: /data/d/231625.json