{"orpha":213777,"name":"High-grade neuroendocrine carcinoma of the cervix uteri","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["High-grade neuroendocrine carcinoma of the uterine cervix","Poorly differentiated neuroendocrine carcinoma of the cervix uteri","Poorly differentiated neuroendocrine cervical carcinoma"],"definition":"High-grade neuroendocrine carcinoma of the cervix uteri is a rare, aggressive, primary cervical neoplasm, originating from neuroendocrine cells present in the lining epithelium of the cervix, characterized, macroscopically, by usually large lesions, sometimes with a barrel-shaped appearance. Patients often present with abnormal vaginal bleeding or discharge, pelvic/abdominal pain, post-coital spotting and/or dysuria, while symptoms related to carcinoid syndrome are not frequent.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-213777/","orphanet_url":"https://www.orpha.net/en/disease/detail/213777","names":{"fr":{"name":"Carcinome neuroendocrine de haut grade du col de l'utérus","synonyms":["Carcinome neuroendocrine cervical peu différencié","Carcinome neuroendocrine peu différencié du col de l'utérus"],"definition":"Cancer primitif rare et agressif du col de l'utérus, prenant naissance dans les cellules neuroendocrines présentes dans l'épithélium bordant le col de l'utérus, caractérisé sur le plan macroscopique par des lésions généralement étendues, prenant parfois un aspect en tonneau. Les patientes présentent souvent des saignements vaginaux anormaux ou des sécrétions vaginales anormales, des douleurs pelviennes/abdominales, des saignements après un rapport et/ou une dysurie, mais les symptômes liés au syndrome carcinoïde ne sont pas fréquents."},"de":{"name":"High-grade-NET der Cervix uteri","synonyms":["Karzinom der Cervix uteri, neuroendokrines, wenig differenziertes","Zervixkarzinom, neuroendokrines, high-grade","Zervixkarzinom, neuroendokrines, wenig differenziertes"]},"es":{"name":"Carcinoma neuroendocrino de cuello uterino de alto grado","synonyms":["Carcinoma cervical neuroendocrino pobremente diferenciado","Carcinoma neuroendocrino de alto grado del cuello uterino","Carcinoma neuroendocrino pobremente diferenciado del cuello uterino"],"definition":"Es una neoplasia primaria cervical agresiva y poco frecuente que se origina a partir de células neuroendocrinas presentes en el epitelio de revestimiento del cuello uterino. Está caracterizado macroscópicamente por lesiones generalmente grandes, en ocasiones con apariencia de barril. Las afectadas presentan, a menudo, sangrado o flujo vaginal anómalo, dolor pélvico / abdominal, manchado postcoital y/o disuria, mientras que los síntomas relacionados con el síndrome carcinoide no son frecuentes."},"it":{"name":"Carcinoma endocrino scarsamente differenziato della cervice uterina","synonyms":["Carcinoma cervicale endocrino scarsamente differenziato"],"definition":"Il carcinoma neuroendocrino della cervice uterina ad alto grado di malignità è una neoplasia primitiva aggressiva rara della cervice dell'utero, che origina dalle cellule neuroendocrine dell'epitelio che riveste la cervice. Dal punto di vista macroscopico, è caratterizzato da lesioni di solito ampie, talvolta dall'aspetto che ricorda una botte. Spesso le pazienti presentano emorragie o perdite vaginali anomale, dolore pelvico/addominale, spotting post-coitale e/o disuria, mentre sono rari i sintomi correlati alla sindrome del carcinoide"},"nl":{"name":"Hooggradig neuro-endocrien carcinoom van cervix uteri","synonyms":["Hooggradig neuro-endocrien carcinoom van baarmoederhals","Slecht gedifferentieerd neuro-endocrien carcinoom van baarmoederhals","Slecht gedifferentieerd neuro-endocrien carcinoom van cervix uteri","Slecht gedifferentieerd neuro-endocrien cervicaal carcinoom"],"definition":"Hooggradig neuro-endocrien carcinoom van de baarmoederhals is een zeldzaam, agressief, primair neoplasma van de baarmoederhals, dat ontstaat uit neuro-endocriene cellen in epitheelbekleding van de cervix, en dat macroscopisch gekarakteriseerd wordt door meestal grote letsels, soms met een tonvormig uiterlijk Patiënten vertonen vaak abnormale vaginale bloeding of afscheiding, pelviene/abdominale pijn, postcoïtale bloeding en/of dysurie, terwijl symptomen gerelateerd aan carcinoïdsyndroom niet frequent voorkomen."},"pt":{"name":"Carcinoma endócrino mal diferenciado do colo do útero"},"pl":{"name":"Neuroendokrynny rakowiak szyjki macicy o wysokim stopniu złośliwości","synonyms":["Słabo zróżnicowany neuroendokrynny rakowiak szyjki macicy"]},"cs":{"name":"Špatně diferencovaný endokrinní karcinom hrdla děložního","synonyms":["Špatně diferencovaný endokrinní cervikální karcinom"]},"uk":{"name":"Нейроендокринна карцинома шийки матки високого ступеня злоякісності","synonyms":["Нейроендокринна карцинома шийки матки високого ступеня злоякісності","Погано диференційована нейроендокринна карцинома шийки матки","Погано диференційована нейроендокринна карцинома шийки матки"],"definition":"High-grade neuroendocrine carcinoma of the cervix uteri is a rare, aggressive, primary cervical neoplasm, originating from neuroendocrine cells present in the lining epithelium of the cervix, characterized, macroscopically, by usually large lesions, sometimes with a barrel-shaped appearance. Patients often present with abnormal vaginal bleeding or discharge, pelvic/abdominal pain, post-coital spotting and/or dysuria, while symptoms related to carcinoid syndrome are not frequent."},"zh":{"name":"子宫颈高度神经内分泌癌","synonyms":["子宫颈低分化神经内分泌癌"],"definition":"子宫颈高级神经内分泌癌是一种罕见的侵袭性原发性子宫颈肿瘤，起源于子宫颈被覆上皮中的神经内分泌细胞，肉眼上表现为大病灶，有时呈桶状外观。患者常表现为阴道异常出血或分泌物、盆腔/腹部疼痛、性交后点状出血和/或排尿困难，而与类癌综合征相关的症状并不常见。"},"tr":{"name":"Serviks uterinin yüksek seviyede nöroendokrin karsinomu","synonyms":["Kötü diferansiye nöroendokrin servikal karsinom","Rahim ağzının yüksek dereceli nöroendokrin karsinomu","Serviks uteri'nin kötü diferansiye nöroendokrin karsinomu"]}},"xrefs":[{"source":"ICD-10","code":"C53.0","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C53.1","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"C53.8","qualifier":"BTNT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C77.3","qualifier":"NTBT","icd_relation":"attributed","validated":true,"icd11_uri":"null"},{"source":"MONDO","code":"0016276","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4751235","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":100101,"name":"Neuroendocrine tumor with other location","classifications":[193,202]},{"orpha":213761,"name":"Rare cancer of cervix uteri","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult","Elderly"],"prevalence":[],"phenotypes":null}