{"orpha":231632,"name":"Ectopic aldosterone-producing tumor","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Extra-adrenal aldosterone-producing tumor"],"definition":"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.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-231632/","orphanet_url":"https://www.orpha.net/en/disease/detail/231632","names":{"fr":{"name":"Tumeur ectopique sécrétrice d'aldostérone","synonyms":["Tumeur extra-surrénalienne sécrétrice d'aldostérone"],"definition":"Néoplasme rare produisant de l'aldostérone, composé de tissu corticosurrénalien aberrant situé en dehors des glandes surrénales (par exemple dans le rétropéritoine, le tissu adipeux périnéphrique ou périaortique, le thorax, le canal rachidien, les testicules, les ovaires) caractérisé par des symptômes de l'hyperaldostéronisme (hypertension chronique réfractaire et hypokaliémie) ou associés à une hypertrophie localisée de la tumeur."},"de":{"name":"Tumor, Aldosteron-produzierender ektopischer","synonyms":["Extraadrenaler Aldosteron-produzierender Tumor"]},"es":{"name":"Tumor ectópico productor de aldosterona","synonyms":["Tumor extrasuprarrenal productor de aldosterona"],"definition":"Es una neoplasia productora de aldosterona, muy poco frecuente, compuesta por tejido adrenocortical aberrante ubicado fuera de las glándulas suprarrenales (p. ej. en el retroperitoneo, tejido adiposo perirrenal o periaórtico, tórax, canal espinal, testículos, ovarios). Por lo general, está caracterizada por síntomas relacionados con los elevados niveles de aldosterona (tales como hipertensión sostenida y refractaria al tratamiento e hipopotasemia) o síntomas producidos por el crecimiento local del tumor."},"it":{"name":"Tumore ectopico secernente aldosterone","synonyms":["Tumore extra-surrenalico secernente aldosterone"],"definition":"Il tumore ectopico secernente aldosterone è una neoplasia molto rara composta da tessuto adrenocorticale aberrante localizzato al di fuori delle ghiandole surrenali (ad esempio nel retroperitoneo, nel tessuto grasso perirenale o periaortico, nel torace, nel canale spinale, nei testicoli e nelle ovaie). Di solito i pazienti presentano sintomi secondari ad un aumento dei livelli di aldosterone (ad es. ipertensione marcata resistente al trattamento e ipokaliemia) oppure correlati all'aumento localizzato delle dimensioni del tumore."},"nl":{"name":"Ectopische aldosteron-producerende tumor","synonyms":["Extra-adrenale aldosteron-producerende tumor"],"definition":"Ectopische aldosteron-producerende tumor is een uiterst zeldzaam aldosteron-producerend neoplasma bestaande uit afwijkend adrenocorticaal weefsel dat zich buiten de bijnieren bevindt (e.g. in het retroperitoneum, perirenaal of periaortaal vetweefsel, de thorax, het ruggenmergkanaal, de testes, de ovaria), en wordt doorgaans gekarakteriseerd door symptomen gerelateerd aan verhoogde gehaltes van aldosteron (zoals aanhoudende, refractaire hypertensie en hypokaliëmie) of symptomen veroorzaakt door lokale vergroting van de tumor."},"pt":{"name":"Tumor produtor de aldosterona ectópico","synonyms":["Tumor produtor de aldosterona extra-supra-renal"]},"pl":{"name":"Guz ektopowy wydzielający aldosteron","synonyms":["Extra-adrenal aldosterone-producing tumor"],"definition":"*Guz ektopowy wydzielający aldosteron jest niezwykle rzadkim nowotworem produkującym aldosteron, wywodzącym się z nieprawidłowej tkanki kory nadnerczy, zlokalizowanej poza nadnerczami (np. w przestrzeni zaotrzewnowej, w okołonerkowej lub okołoaortalnej tkance tłuszczowej, klatce piersiowej, kanale kręgowym, jądrach, jajnikach); zwykle występują objawy typowe dla hiperaldosteronizmu (przewlekłe, oporne na leczenie nadciśnienie i hipokaliemia) lub związane z miejscowym powiększaniem się guza."},"cs":{"name":"Ektopický aldosteron produkující karcinom","synonyms":["Extra-adrenální aldosteron produkující karcinom"]},"uk":{"name":"Ектопічна альдостерон-продукуюча пухлина","synonyms":["Позанадниркова пухлина, що продукує альдостерон"],"definition":"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."},"zh":{"name":"异位醛固酮肿瘤","synonyms":["肾上腺外醛固酮肿瘤"],"definition":"醛固酮异位性肿瘤是一种极为罕见的分泌醛固酮的肿瘤，由位于肾上腺外（例如腹膜后、肾周或主动脉周围脂肪组织、胸部、椎管、睾丸，卵巢）的异常肾上腺皮质组织构成，典型特征是与醛固酮水平升高相关的症状（如持续的、难治的高血压和低钾血症）或由局部肿瘤增大引起的症状。"},"tr":{"name":"Ektopik aldosteron üreten tümör","synonyms":["Adrenal dışı aldosteron-üreten tümör"]}},"xrefs":[{"source":"ICD-10","code":"E26.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"5A72.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0016506","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4755311","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97978,"name":"Rare endocrine disease"},"parents":[{"orpha":231641,"name":"Rare non surgically correctable form of primary aldosteronism","classifications":[193]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-08-31","source":"8639013[PMID]_15657580[PMID]_8787960[PMID]_26934393[PMID]_27036860[PMID]_171276[PMID]_3518689[PMID]_2344975[PMID]","terms":[{"hpo":"HP:0003351","label":"Decreased circulating renin concentration","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0011740","label":"Glucocortocoid-insensitive primary hyperaldosteronism","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0000822","label":"Hypertension","frequency":"Obligate (100%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002900","label":"Hypokalemia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100615","label":"Ovarian neoplasm","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0006735","label":"Renal cortical adenoma","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200114","label":"Metabolic alkalosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000421","label":"Epistaxis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000360","label":"Tinnitus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008256","label":"Adrenocortical adenoma","frequency":"Excluded (0%)","criteria":null}]}}