{"orpha":213736,"name":"Low-grade neuroendocrine tumor of the corpus uteri","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Low-grade neuroendocrine tumor of the uterine corpus","Well-differentiated neuroendocrine neoplasm of the endometrium","Well-differentiated neuroendocrine tumor of the corpus uteri","Well-differentiated neuroendocrine tumor of the endometrium"],"definition":"Low-grade neuroendocrine tumor of the corpus uteri is an extremely rare uterine cancer typically characterized by a well demarcated, solid, frequently pedunculated tumor originating from neuroendocrine cells scattered within the endometrium, often associated with ectopic hormone production. Patients usually present with vaginal bleeding or discharge and a pelvic mass with a polypoid tumor sometimes protruding through the cervical canal. Symptoms related to ectopic hormone production (flushing, sweating, diarrhea, bronchospasm) may also develop.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-213736/","orphanet_url":"https://www.orpha.net/en/disease/detail/213736","names":{"fr":{"name":"Tumeur neuroendocrine de faible grade du corps de l'utérus","synonyms":["Néoplasie endocrine bien différenciée de l'endomètre","Néoplasme neuroendocrine de bas grade du corps de l'utérus","Tumeur endocrine bien différenciée du corps de l'utérus","Tumeur neuroendocrine bien différenciée de l'endomètre"],"definition":"Cancer rare de l'utérus caractérisé par une tumeur solide, bien délimitée, souvent pédiculée, provenant de cellules neuroendocrines disséminées dans l'endomètre, souvent associée à une production hormonale ectopique. Les patientes présentent généralement des saignements ou des pertes vaginales et une masse pelvienne avec une tumeur polypoïde faisant parfois saillie dans le col de l'utérus. Des symptômes liés à la production ectopique d'hormones (bouffées vasomotrices, transpiration, diarrhée, bronchospasme) peuvent également s'observer."},"de":{"name":"Low-grade-NET des Corpus uteri","synonyms":["Gut differenzierte neuroendokrine Neoplasie des Corpus uteri","Gut differenzierte neuroendokrine Neoplasie des Endometriums","Gut differenzierter neuroendokriner Tumor des Corpus uteri","Low-grade neuroendokriner Tumor des Corpus uteri"]},"es":{"name":"Carcinoma neuroendocrino de bajo grado del cuerpo uterino","synonyms":["Neoplasia neuroendocrina bien diferenciada del endometrio","Tumor neuroendocrino bien diferenciado del cuerpo uterino","Tumor neuroendocrino bien diferenciado del endometrio"],"definition":"Es un cáncer uterino muy poco frecuente caracterizado típicamente por un tumor bien demarcado, sólido y frecuentemente pedunculado, que se origina en células neuroendocrinas diseminadas dentro del endometrio, asociado con frecuencia a la producción ectópica de hormonas. Las afectadas suelen presentar hemorragia o secreción vaginal y una masa pélvica con un tumor polipoide que en ocasiones protruye a través del canal cervical. También se pueden desarrollar síntomas relacionados con la producción ectópica de hormonas (enrojecimiento, sudoración, diarrea, broncoespasmo)."},"it":{"name":"Tumore neuroendocrino del corpo dell'utero a basso grado di malignità","synonyms":["Neoplasia endocrina ben differenziata dell'endometrio","Tumore endocrino ben differenziato del corpo uterino","Tumore endocrino ben differenziato dell'endometrio"],"definition":"Il tumore neuroendocrino del corpo dell'utero a basso grado di malignità è una neoplasia molto rara, di solito caratterizzata da una neoplasia solida ben delimitata, spesso peduncolata, che origina dalle cellule neuroendocrine sparse nell'endometrio, in genere associato alla produzione ectopica di ormoni. Di solito le pazienti presentano emorragie o perdite vaginali e una massa pelvica consistente in un tumore polipoide, che occasionalmente protrude nel canale cervicale. Possono coesistere sintomi secondari alla produzione ectopica di ormoni (arrossamento, sudorazione, diarrea, broncospasmo)."},"nl":{"name":"Laaggradige neuro-endocriene tumor van corpus uteri","synonyms":["Goed gedifferentieerd neuro-endocrien neoplasma van endometrium","Goed gedifferentieerde neuro-endocriene tumor van baarmoederlichaam","Goed gedifferentieerde neuro-endocriene tumor van corpus uteri","Goed gedifferentieerde neuro-endocriene tumor van endometrium","Laaggradige neuro-endocriene tumor van baarmoederlichaam"],"definition":"Laaggradige neuro-endocriene tumor van het baarmoederlichaam is een uiterst zeldzame uteriene kanker, en wordt doorgaans gekarakteriseerd door een begrensde, solide, vaak gesteelde tumor die ontstaat uit neuro-endocriene cellen verspreid in het baarmoederslijmvlies, vaak geassocieerd met ectopische productie van hormonen. Patiënten vertonen meestal vaginale bloeding of afscheiding en een pelviene massa met een polypoïde tumor die soms uitpuilt door het cervixkanaal. Ook kunnen symptomen ontwikkelen die zijn gerelateerd aan ectopische productie van hormonen (blozen, zweten, diarree, bronchospasme)."},"pt":{"name":"Neoplasia endócrino bem diferenciada do corpo uterino"},"pl":{"name":"Neuroendokrynny rakowiak trzonu macicy o niskim stopniu złośliwości","synonyms":["Dobrze zróżnicowany neuroendokrynny rak endometrium","Dobrze zróżnicowany neuroendokrynny rak trzonu macicy","Dobrze zróżnicowany neuroendokrynny nowotwór endometrium"],"definition":"*Neuroendokrynny nowotwór trzonu macicy o niskim stopniu złośliwości jest niezwykle rzadkim nowotworem macicy, który typowo przyjmuje postać dobrze odgraniczonego, litego, często uszypułowanego guza, wywodzącego się z komórek neuroendokrynnych rozproszonych w obrębie endometrium i jest często związany z ektopową produkcją hormonów. Objawy kliniczne zwykle obejmują krwawienie/wydzielinę z pochwy oraz obecność polipowatego guza w miednicy, który może być widoczny w obrębie szyjki macicy. Mogą również wystąpić objawy związane z ektopową produkcją hormonów (uderzenia gorąca, pocenie się, biegunka, skurcz oskrzeli)."},"cs":{"name":"Dobře diferencovaná endokrinní neoplázie těla děložního","synonyms":["Dobře diferencovaná endokrinní neoplázie endometria","Dobře diferencovaný endokrinní nádor těla děložního","Dobře diferencovaný endokrinní nádor endometria"]},"uk":{"name":"Нейроендокринна пухлина тіла матки низького ступеня злоякісності","synonyms":["Нейроендокринна пухлина тіла матки низького ступеня злоякісності","Добре диференційована нейроендокринна пухлина ендометрію","Добре диференційована нейроендокринна пухлина тіла матки","Добре диференційована нейроендокринна пухлина ендометрію"],"definition":"Low-grade neuroendocrine tumor of the corpus uteri is an extremely rare uterine cancer typically characterized by a well demarcated, solid, frequently pedunculated tumor originating from neuroendocrine cells scattered within the endometrium, often associated with ectopic hormone production. Patients usually present with vaginal bleeding or discharge and a pelvic mass with a polypoid tumor sometimes protruding through the cervical canal. Symptoms related to ectopic hormone production (flushing, sweating, diarrhea, bronchospasm) may also develop."},"zh":{"name":"子宫体低度神经内分泌肿瘤","synonyms":["子宫体高分化神经内分泌肿瘤"],"definition":"子宫体低级别神经内分泌肿瘤是一种极为罕见的子宫癌，其特征是肿瘤边界清楚、坚实、常有蒂，起源于分散在子宫内膜内的神经内分泌细胞，常伴有异位激素分泌。患者通常表现为阴道出血或分泌物，以及盆腔肿块，伴有时突出于宫颈管的息肉样肿瘤。也可能出现与异位激素分泌有关的症状（潮红、出汗、腹泻、支气管痉挛）。"},"tr":{"name":"Korpus uteri'nin düşük seviyede nöroendokrin tümörü","synonyms":["Uterus korpusunun düşük dereceli nöroendokrin tümörü","Endometriumun iyi diferansiye nöroendokrin tümörü","Korpus uteri'nin iyi diferansiye nöroendokrin tümörü","Endometriumun iyi diferansiye nöroendokrin neoplazmı"]}},"xrefs":[{"source":"ICD-10","code":"C54.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C54.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C54.2","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C54.3","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C54.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C76.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0016270","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5190779","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":213569,"name":"Rare cancer of corpus uteri","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}