{"orpha":494454,"name":"Vulvar adenocarcinoma","type":"Histopathological subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Adenocarcinoma of the vulva"],"definition":"A rare vulvar carcinoma characterized by a malignant epithelial neoplasm of glandular origin and/or with glandular characteristics arising in the vulva, including adenocarcinoma of mammary gland type, sweat gland type, and intestinal type, as well as adenocarcinomas of the Bartholin glands and Paget disease of the vulva. Depending on the type of tumor and disease stage, patients may present with a solitary vulvar mass, bleeding, or (in the case of Paget disease) a pruritic, erythematous, eczematous lesion.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-494454/","orphanet_url":"https://www.orpha.net/en/disease/detail/494454","names":{"fr":{"name":"Adénocarcinome de la vulve","synonyms":["Adénocarcinome vulvaire"],"definition":"Carcinome de la vulve rare caractérisé par un néoplasme épithélial malin d'origine glandulaire et/ou présentant des caractéristiques glandulaires affectant la vulve, comprenant les adénocarcinomes prenant naissance dans la glande mammaire, la glande sudoripare et la glande intestinale, ainsi que les adénocarcinomes prenant naissance dans les glandes de Bartholin et la maladie de Paget de la vulve. Selon le type de tumeur et le stade de la maladie, les patientes peuvent présenter une masse vulvaire isolée, des saignements ou (dans le cas de la maladie de Paget) une lésion prurigineuse, érythémateuse et eczémateuse."},"de":{"name":"Adenokarzinom der Vulva","definition":"Ein seltenes Vulvakarzinom, bei dem es sich um eine maligne epitheliale Neoplasie drüsigen Ursprungs und/oder mit drüsigen Merkmalen handelt, die in der Vulva entsteht, einschließlich Adenokarzinome vom Typ der Brustdrüse, der Schweißdrüse und des Darms sowie Adenokarzinome der Bartholin-Drüsen und Morbus Paget der Vulva. Je nach Tumorart und Krankheitsstadium können sich die Patientinnen mit einer solitären vulvären Gewebeverdichtung, Blutungen oder (im Falle der Paget-Krankheit) mit einer juckenden, erythematösen, ekzematösen Läsion vorstellen."},"es":{"name":"Adenocarcinoma vulvar","synonyms":["Adenocarcinoma de la vulva"],"definition":"Es un carcinoma vulvar poco frecuente caracterizado por una neoplasia epitelial maligna de origen glandular y/o con características glandulares que se origina en la vulva. Incluye el adenocarcinoma de tipo glándula mamaria, de tipo glándula sudorípara y de tipo intestinal, así como los adenocarcinomas de la glándula de Bartolino y la enfermedad de Paget de la vulva. Según el tipo de tumor y el estadio de la enfermedad, las pacientes pueden presentar una masa vulvar aislada, sangrado o (en el caso de la enfermedad de Paget) una lesión pruriginosa, eritematosa y eccematosa."},"it":{"name":"Adenocarcinoma vulvare","definition":"Si tratta di un carcinoma raro della vulva, caratterizzato da una neoplasia epiteliale maligna di origine ghiandolare e/o con caratteristiche ghiandolari, che origina dalla vulva, e comprende l'adenocarcinoma di tipo mammario, delle ghiandole sudoripare e intestinale, l'adenocarcinoma delle ghiandole di Bartolini e la malattia di Paget della vulva. A seconda del tipo di tumore e dello stadio della malattia, le pazienti possono presentare una massa vulvare solitaria, emorragie o (nel caso della malattia di Paget) una lesione pruriginosa, eritematosa ed eczematosa."},"nl":{"name":"Adenocarcinoom van vulva","definition":"Een zeldzaam carcinoom van vulva, gekarakteriseerd door een maligne epitheliaal neoplasma van glandulaire oorsprong en/of met glandulaire kenmerken dat ontstaat in vulva, waaronder adenocarcinoom van borstklier-type, zweetklier-type, en intestinaal type, alsook adenocarcinomen van klieren van Bartholin en ziekte van Paget van vulva. Afhankelijk van het type van de tumor en het stadium van de ziekte kunnen patiënten zich presenteren met een solitaire massa van vulva, bloeding, of (in geval van ziekte van Paget) een pruritische, erythemateuze, eczemateuze laesie."},"pt":{"name":"Adenocarcinoma vulvar"},"pl":{"name":"Rak gruczołowy sromu","synonyms":["Gruczolak złośliwy sromu"]},"cs":{"name":"Adenokarcinom vulvy","synonyms":["Adenocarcinoma of the vulva"]},"uk":{"name":"Аденокарцинома вульви","synonyms":["Аденокарцинома вульви"],"definition":"A rare vulvar carcinoma characterized by a malignant epithelial neoplasm of glandular origin and/or with glandular characteristics arising in the vulva, including adenocarcinoma of mammary gland type, sweat gland type, and intestinal type, as well as adenocarcinomas of the Bartholin glands and Paget disease of the vulva. Depending on the type of tumor and disease stage, patients may present with a solitary vulvar mass, bleeding, or (in the case of Paget disease) a pruritic, erythematous, eczematous lesion."},"zh":{"name":"外阴腺癌","synonyms":["Adenocarcinoma of the vulva"]},"tr":{"name":"Vulvar adenokarsinom","synonyms":["Vulvanın adenokarsinomu"]}},"xrefs":[{"source":"ICD-10","code":"C51.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0024336","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1336975","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10063536","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":494418,"name":"Vulvar carcinoma","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[],"phenotypes":null}