{"orpha":874,"name":"Primary adult heart tumor","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Adult cardiac tumor","Adult heart tumor"],"definition":"A rare disorder that manifest in adults and generally present with a variety of non-specific manifestations (depending on tumor site and infiltration) such as weight loss, exhaustion, hemorrhagic pericardial effusion, heart failure, arrhythmias, and embolisms, or that can also be asymptomatic. In adults 75% of heart tumors are benign, with myxoma being the most common benign tumor (accounting for 50-70% of all primary heart tumors) and rhabdomyosarcoma comprising 75% of malignant heart tumors. Other malignant tumors of the heart include fibrosarcoma and leiomyosarcoma.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-874/","orphanet_url":"https://www.orpha.net/en/disease/detail/874","names":{"fr":{"name":"Tumeur cardiaque primaire de l'adulte","definition":"Une maladie cardiaque rare qui se manifeste chez l'adulte et qui présente généralement une variété de manifestations non spécifiques (en fonction du site tumoral et de l'infiltration) telles que la perte de poids, l'épuisement, l'épanchement péricardique hémorragique, l'insuffisance cardiaque, les arythmies et les embolies. Les patients peuvent également être asymptomatique. Chez les adultes, 75% des tumeurs cardiaques sont bénignes, le myxome étant la tumeur bénigne la plus courante (représentant 50 à 70% de toutes les tumeurs cardiaques primaires) et le rhabdomyosarcome comprenant 75% des tumeurs cardiaques malignes. D'autres tumeurs malignes du coeur comprennent le fibrosarcome et le léiomyosarcome."},"de":{"name":"Herztumor, primärer, des Erwachsenen","synonyms":["Primärer kardialer Tumor des Erwachsenen"]},"es":{"name":"Tumor cardíaco primario del adulto","definition":"Este término hace referencia a neoplasias del corazón que se manifiestan en adultos y que, por lo general, presentan una variedad de manifestaciones inespecíficas (dependiendo de la localización del tumor y de la infiltración), tales como pérdida de peso, agotamiento, derrame pericárdico hemorrágico, insuficiencia cardíaca, arritmias y embolias, aunque también pueden estar asintomáticos. El 75% de los tumores cardíacos en adultos son benignos, siendo el mixoma el tumor benigno más común (representa el 50-70% de todos los tumores cardíacos primarios) y el rabdomiosarcoma el que comprende el 75% de los tumores cardíacos malignos. Otros tumores cardiacos malignos incluyen el fibrosarcoma y el leiomiosarcoma."},"it":{"name":"Tumore cardiaco primitivo dell'adulto","definition":"Il tumore del cuore dell'adulto fa riferimento alle neoplasie cardiache che insorgono negli adulti e in genere esordiscono con segni clinici aspecifici (che dipendono dalla localizzazione del tumore e dalla sua infiltrazione) come la perdita di peso, la stanchezza estrema, i versamenti emorragici pericardici, l'insufficienza cardiaca, le aritmie, le embolie; i pazienti possono anche essere asintomatici. Negli adulti, il 75% dei tumori cardiaci è benigno; il mixoma è il tumore benigno più comune (50-70% di tutti i tumori cardiaci primitivi), mentre il rabdomiosarcoma (si veda questo termine) rappresenta il 75% dei tumori cardiaci maligni. Altri tumori maligni del cuore sono il fibrosarcoma e il leiomiosarcoma (si vedano questi termini)."},"nl":{"name":"Primaire harttumor bij volwassenen","synonyms":["Cardiale tumor bij volwassenen","Harttumor bij volwassenen"],"definition":"Een zeldzame aandoening die zich manifesteert bij volwassenen en doorgaans tot uiting komt met een variëteit aan niet-specifieke manifestaties (afhankelijk van de locatie en infiltratie van de tumor) zoals gewichtsverlies, uitputting, hemorragische pericardiale effusie, hartfalen, hartritmestoornissen, en embolieën, of die ook asymptomatisch kan zijn. Bij volwassenen is 75% van de harttumoren goedaardig, waarbij myxoom de meest gangbare goedaardige tumor is (vertegenwoordigt 50-70% van alle primaire harttumoren) en rhabdomyosarcoom (zie deze term) 75% van de maligne harttumoren uitmaakt. Andere maligne harttumoren zijn onder meer fibrosarcoom en leiomyosarcoom (zie deze termen)."},"pt":{"name":"Tumor cardíaco do adulto"},"pl":{"name":"Pierwotny guz serca dorosłych","synonyms":["Guz serca dorosłych"]},"cs":{"name":"Nádor srdce v dospělém věku","synonyms":["Adultní kardiální nádor"]},"uk":{"name":"Первинна пухлина серця у дорослих","synonyms":["Пухлина серця у дорослих","Пухлина серця у дорослих"],"definition":"A rare disorder that manifest in adults and generally present with a variety of non-specific manifestations (depending on tumor site and infiltration) such as weight loss, exhaustion, hemorrhagic pericardial effusion, heart failure, arrhythmias, and embolisms, or that can also be asymptomatic. In adults 75% of heart tumors are benign, with myxoma being the most common benign tumor (accounting for 50-70% of all primary heart tumors) and rhabdomyosarcoma comprising 75% of malignant heart tumors. Other malignant tumors of the heart include fibrosarcoma and leiomyosarcoma."},"zh":{"name":"原发性成人心脏肿瘤","synonyms":["成人心脏肿瘤"]},"tr":{"name":"Primer yetişkin kalp tümörü","synonyms":["Erişkin kalp tümörü","Erişkin kalp tümörü"]}},"xrefs":[{"source":"ICD-10","code":"C38.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"D15.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2F01","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1784866291"},{"source":"MONDO","code":"0019493","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4275152","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":168194,"name":"Rare cardiac tumor","classifications":[146,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}