{"orpha":231625,"name":"Adrenocortical carcinoma with pure aldosterone hypersecretion","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Pure APAC","Pure aldosterone-producing adrenocortical carcinoma","Pure aldosterone-secreting adrenocortical carcinoma"],"definition":"A very rare surgically-correctable form of primary aldosteronism (PA) due to an aldosterone-secreting adrenal malignancy.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-231625/","orphanet_url":"https://www.orpha.net/en/disease/detail/231625","names":{"fr":{"name":"Carcinome corticosurrénalien à hypersécrétion pure d'aldostérone","synonyms":["Corticosurrénalome malin avec excès d'aldostérone"],"definition":"Le carcinome corticosurrénalien à hypersécrétion pure d'aldostérone est une forme très rare, curable chirurgicalement, d'hyperaldostéronisme primitif (HAP) dû à une tumeur surrénalienne maligne (ou corticosurrénalome) sécrétant de l'aldostérone."},"de":{"name":"Adrenokortikales Karzinom mit isolierter Aldosteron-Hypersekretion","definition":"Das Adrenokortikale Karzinom mit isolierter Aldosteron-Hypersekretion (pure APAC), eine sehr seltene Form von chirurgisch korigierbarem primärem Hyper-Aldosteronismus (PA; s. dort) ist ein Aldosteron sezernierendes Malignom der Nebenniere."},"es":{"name":"Carcinoma corticosuprarrenal con hipersecreción pura de aldosterona","synonyms":["APAC pura","Carcinoma corticosuprarrenal productor puro de aldosterona","Carcinoma corticosuprarrenal secretor puro de aldosterona"],"definition":"Es una forma muy poco frecuente de aldosteronismo primario (AP) que puede corregirse quirúrgicamente, y que es debida a una neoplasia suprarrenal secretora de aldosterona."},"it":{"name":"Carcinoma cortico-surrenalico con ipersecrezione di aldosterone puro","synonyms":["APAC puro","Carcinoma cortico-surrenalico producente aldosterone puro","Carcinoma cortico-surrenalico secernente aldosterone puro"],"definition":"Il carcinoma cortico-surrenalico con ipersecrezione di aldosterone puro (APAC puro) è una forma molto rara di (iper) aldosteronismo primitivo (PA; si veda questo termine), correggibile chirurgicamente; questa malattia è causata da un tumore maligno dei surreni secernente aldosterone."},"nl":{"name":"Bijnierschorscarcinoom met zuivere aldosteronhypersecretie","synonyms":["Adrenocorticaal carcinoom met zuivere aldosteronhypersecretie","Zuiver APAC","Zuiver aldosteronproducerend bijnierschorscarcinoom","Zuiver aldosteronsecreterend bijnierschorscarcinoom"],"definition":"Een zeer zeldzame, chirurgisch corrigeerbare vorm van primair aldosteronisme (PA) als gevolg van een maligne neoplasma van bijnier dat aldosteron afscheidt."},"pt":{"name":"Carcinoma adrenocortical produtor de aldosterona, puro","definition":"O carcinoma adrenocortical com hipersecreção pura de aldosterona (APAC puro) é uma forma muito rara corrigível cirurgicamente de (hiper)aldosteronismo primário (PA; ver este termo), causada por doença maligna da supra-renal secretora de aldosterona."},"pl":{"name":"Rak kory nadnerczy z hipersekrecją czystego aldosteronu","synonyms":["Czysty APAC","Czysty rak kory nadnerczy produkujący aldosteron","Czysty rak kory nadnerczy wydzielający aldosteron"]},"cs":{"name":"Adrenokortikální karcinom s čistou aldosteronovou hypersekrecí","synonyms":["Čistý APAC","Čistý aldosteron-produkující adrenokortikální karcinom","Čistý aldosteron-vyměšující adrenokortikální karcinom"]},"uk":{"name":"Адренокортикальна карцинома з гіперсекрецією чистого альдостерону","synonyms":["Чиста адренокортикальна карцинома, що продукує альдостерон","Чиста адренокортикальна карцинома, що секретує альдостерон","Чистий APAC"],"definition":"A very rare surgically-correctable form of primary aldosteronism (PA) due to an aldosterone-secreting adrenal malignancy."},"zh":{"name":"肾上腺皮质癌伴单纯醛固酮分泌过多","synonyms":["单纯性醛固酮分泌过多性肾上腺皮质癌"],"definition":"原发性醛固酮增多症（PA）的一种非常罕见的需外科治疗纠正的类型，由分泌醛固酮的肾上腺恶性肿瘤引起。"},"tr":{"name":"Saf aldosteron hipersekresyonlu adrenokortikal karsinom","synonyms":["Saf APAC","Saf aldosteron-üreten adrenokortikal karsinom","Saf aldosteron-salgılayan adrenokortikal karsinom"]}},"xrefs":[{"source":"ICD-10","code":"C74.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"5A72.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1857108449"},{"source":"MONDO","code":"0016505","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"16505","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3839852","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":231637,"name":"Rare surgically correctable form of primary aldosteronism","classifications":[193]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-08-29","source":"20482833[PMID]_26273475[PMID]_8639013[PMID]_21270113[PMID]_24682757[PMID]_24103295[PMID]_11559548[PMID]","terms":[{"hpo":"HP:0000822","label":"Hypertension","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0100631","label":"Neoplasm of the adrenal gland","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0003351","label":"Decreased circulating renin concentration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011740","label":"Glucocortocoid-insensitive primary hyperaldosteronism","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002900","label":"Hypokalemia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003118","label":"Increased circulating cortisol level","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200114","label":"Metabolic alkalosis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0005135","label":"Abnormal T-wave","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003081","label":"Increased urinary potassium","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003394","label":"Muscle spasm","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001962","label":"Palpitations","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003401","label":"Paresthesia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000421","label":"Epistaxis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002170","label":"Intracranial hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000360","label":"Tinnitus","frequency":"Occasional (29-5%)","criteria":null}]}}