{"orpha":284395,"name":"Well-differentiated fetal adenocarcinoma of the lung","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["WDFA"],"definition":"Well-differentiated fetal adenocarcinoma of the lung is a rare, primary, low-grade, bronchopulmonary neoplasm characterized by a well-circumscribed, usually large, pulmonary mass that is histologically composed of glycogen-rich neoplastic glands and tubules that resemble fetal lungs at 10 to 16 weeks of gestation and benign adjacent stroma. It typically presents with chest pain, cough, dyspnea, hemoptysis and/or generalized, non-specific symptoms, such as night sweats, lethargy, poor appetite and weight loss.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-284395/","orphanet_url":"https://www.orpha.net/en/disease/detail/284395","names":{"fr":{"name":"Adénocarcinome pulmonaire bien différencié de type foetal","synonyms":["WDFA"],"definition":"L'adénocarcinome pulmonaire bien différencié de type foetal est un néoplasme broncho-pulmonaire primaire rare, de faible grade. Il se caractérise par une masse pulmonaire habituellement large, bien circonscrite, composée sur le plan histologique par des glandes et des tubules néoplasiques riches en glycogène, ressemblant à des poumons foetaux à 10 à 16 semaines de grossesse, et par un stroma adjacent bénin. L'adénocarcinome pulmonaire se manifeste typiquement par une douleur thoracique, une toux, une dyspnée, une hémoptysie et/ou des signes généralisés non spécifiques tels que des sueurs nocturnes, une léthargie, un faible appétit et une perte de poids."},"de":{"name":"Fetales Adenokarzinom der Lunge, gut-differenziert","synonyms":["WDFA"],"definition":"Eine seltene, primäre, niedriggradige, bronchopulmonale Neoplasie, die durch eine gut umschriebene, zumeist umfangreiche pulmonale Masse gekennzeichnet ist, die histologisch betrachtet aus glykogenreichen neoplastischen Drüsen und Tubuli besteht und Ähnlichkeiten zur fetalen Lunge in der 10. bis 16. Schwangerschaftswoche und gutartigem angrenzendem Stroma aufweist. Sie präsentiert sich typischerweise mit Brustschmerzen, Husten, Dyspnoe, Hämoptyse und/oder generalisierten, unspezifischen Symptomen, wie Nachtschweiß, Lethargie, Appetitlosigkeit und Gewichtsverlust."},"es":{"name":"Adenocarcinoma pulmonar fetal bien diferenciado","synonyms":["WDFA"],"definition":"El adenocarcinoma pulmonar fetal bien diferenciado es una neoplasia broncopulmonar primaria poco frecuente de bajo grado caracterizada por una masa pulmonar bien circunscrita, por lo general grande, que se compone histológicamente de glándulas neoplásicas ricas en glucógeno, túbulos que se asemejan a pulmones fetales de 10 a 16 semanas de gestación y estroma adyacente benigno. Por lo general, se presenta con dolor torácico, tos, disnea, hemoptisis y/o síntomas inespecíficos generalizados, tales como sudoración nocturna, letargia, falta de apetito y pérdida de peso."},"it":{"name":"Adenocarcinoma polmonare fetale differenziato","synonyms":["WDFA"],"definition":"L'adenocarcinoma polmonare fetale differenziato è una neoplasia broncopolmonare primitiva rara a basso grado di malignità. È caratterizzato da una massa polmonare di solito di grandi dimensioni e ben circoscritta, composta, dal punto di vista istologico, da ghiandole e tubuli neoplastici ricchi di glicogeno, che assomigliano ai polmoni di un feto tra la decima e la sedicesima settimana di gestazione, e da stroma benigno circostante. Di solito esordisce con dolore toracico, tosse, dispnea, emottisi e/o sintomi aspecifici generalizzati, come la sudorazione notturna, la letargia, la mancanza di appetito e la perdita di peso."},"nl":{"name":"Goed gedifferentieerd foetaal adenocarcinoom van long","synonyms":["WDFA"],"definition":"Goed gedifferentieerd foetaal adenocarcinoom van de long is een zeldzaam, primair, laaggradig, bronchopulmonaal neoplasma en wordt gekarakteriseerd door een goed begrensde, meestal grote, pulmonale massa die histologisch bestaat uit glycogeenrijke neoplastische klieren en buisjes, die lijken of foetale longen na gestatie van 10 tot 16 weken, en goedaardig omliggend stroma. Het uit zich doorgaans met pijn in de borst, hoesten, dyspneu, hemoptoë en/of gegeneraliseerde, niet-specifieke symptomen zoals nachtzweten, lethargie, weinig eetlust en gewichtsverlies."},"pt":{"name":"Adenocarcinoma bem diferenciado fetal do pulmão"},"pl":{"name":"Dobrze zróżnicowany gruczolakorak płuc płodu","synonyms":["WDFA"]},"cs":{"name":"Dobře diferencovaný fetální adenokarcinom plic","synonyms":["WDFA"]},"uk":{"name":"Добре диференційована фетальна аденокарцинома легень","synonyms":["WDFA"],"definition":"Well-differentiated fetal adenocarcinoma of the lung is a rare, primary, low-grade, bronchopulmonary neoplasm characterized by a well-circumscribed, usually large, pulmonary mass that is histologically composed of glycogen-rich neoplastic glands and tubules that resemble fetal lungs at 10 to 16 weeks of gestation and benign adjacent stroma. It typically presents with chest pain, cough, dyspnea, hemoptysis and/or generalized, non-specific symptoms, such as night sweats, lethargy, poor appetite and weight loss."},"zh":{"name":"高分化胎儿肺腺癌","synonyms":["WDFA"],"definition":"分化良好的胎儿肺腺癌是一种罕见的原发性低级别支气管肺部肿瘤，其特征是边界清晰，通常有较大的肺部肿块，组织学上上由富含糖原的肿瘤腺体和小管组成，类似于妊娠10至16周的胎肺和良性相邻良性基质。常表现为胸痛、咳嗽、呼吸困难、咯血和/或全身性、非特异性症状，如盗汗、嗜睡、食欲不振和体重减轻。"},"tr":{"name":"Akciğerin iyi diferansiye fetal adenokarsinomu","synonyms":["WDFA"]}},"xrefs":[{"source":"ICD-10","code":"C34.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C34.2","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C34.3","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C34.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C25.0","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0017292","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3873372","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":101945,"name":"Rare bronchopulmonary and pleural cavity tumors","classifications":[184,202]}],"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":null}