{"orpha":213833,"name":"Glassy cell carcinoma of the cervix uteri","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Glassy cell carcinoma of the cervix uteri is a rare cancer of the uterine cervix, composed of nests of large neoplastic cells with 'ground glass' cytoplasm, surrounded by a stroma with prominent eosinophilic infiltrates. It is a poorly differentiated, aggressive variant of adenosquamous carcinoma that usually affects young women and presents with dysfunctional vaginal bleeding and lower abdominal pain. Distant metastases to the lungs, liver spleen or bones are often present at the time of diagnosis. It is often associated with high-risk HPV-infection (types 18, 16 and 32).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-213833/","orphanet_url":"https://www.orpha.net/en/disease/detail/213833","names":{"fr":{"name":"Carcinome à cellules vitreuses du col de l'utérus","synonyms":["Carcinome cervical à cellules vitreuses"],"definition":"Cancer rare du col de l'utérus, composé de nids de grandes cellules néoplasiques au cytoplasme en « verre dépoli », entourées d'un stroma avec des infiltrats majoritairement éosinophiles. Il s'agit d'une forme mal différenciée et agressive du carcinome adénosquameux qui touche généralement les jeunes femmes et se manifeste par des saignements vaginaux anormaux et des douleurs dans le bas-ventre . Des métastases à distance dans les poumons, le foie, la rate ou les os sont souvent présentes au moment du diagnostic. Il est souvent associé à une infection à HPV à haut risque (types 18, 16 et 32)."},"de":{"name":"Glaszellkarzinom der Cervix uteri","definition":"Ein seltenes Karzinom des Gebärmutterhalses, bestehend aus Nestern großer neoplastischer Zellen mit \"geschliffenem\" Zytoplasma, umgeben von einem Stroma mit prominenten eosinophilen Infiltraten. Es handelt sich um eine schlecht differenzierte, aggressive Variante des adenosquamösen Karzinoms, die in der Regel junge Frauen betrifft und sich mit dysfunktionalen vaginalen Blutungen und Unterleibsschmerzen präsentiert. Fernmetastasen in Lunge, Leber, Milz oder Knochen sind zum Zeitpunkt der Diagnose häufig vorhanden. Es ist häufig mit einer Hochrisiko-HPV-Infektion (Typen 18, 16 und 32) assoziiert."},"es":{"name":"Carcinoma de células en vidrio esmerilado de cuello de útero","definition":"El carcinoma de células en vidrio esmerilado de cuello de útero, es un cáncer poco frecuente del cuello uterino compuesto por nidos de células neoplásicas grandes con citoplasma de aspecto en \"vidrio esmerilado\", rodeado por un estroma con prominentes infiltrados eosinofílicos. Es una variante agresiva y pobremente diferenciada del carcinoma adenoescamoso que generalmente afecta a mujeres jóvenes y que se presenta con sangrado vaginal disfuncional y dolor abdominal inferior. Con frecuencia, las metástasis distales a los pulmones, hígado, bazo o huesos están presentes en el momento del diagnóstico. A menudo, se asocia con infección por tipos de VPH de alto riesgo (tipos 18, 16 y 32)."},"it":{"name":"Carcinoma a cellule vitree della cervice uterina","definition":"Il carcinoma a cellule vitree della cervice uterina è una neoplasia rara, composta da nidi di grandi cellule neoplastiche con citoplasma a ''vetro smerigliato'', circondato da uno stroma con evidenti infiltrati eosinofili. Si tratta di una variante scarsamente differenziata e aggressiva del carcinoma adenosquamoso, che di solito colpisce le donne giovani ed esordisce con emorragie vaginali disfunzionali e dolore nella parte inferiore dell'addome. Spesso, al momento della diagnosi, si riscontrano metastasi nei polmoni, nel fegato, nella milza o nelle ossa. Di solito si associa all'infezione da HPV ad alto rischio (tipi 18, 16 e 32)."},"nl":{"name":"Glasachtige cel-carcinoom van cervix uteri","synonyms":["Glasachtige cel-carcinoom van baarmoederhals"],"definition":"Glasachtig celcarcinoom van de cervix uteri is een zeldzame kanker van de baarmoederhals, die bestaat uit nesten van grote neoplastische cellen met matglasachtig cytoplasma, omgeven door een stroma met opvallende eosinofiele infiltraten. Het is een weinig gedifferentieerde, agressieve variant van adenosquameus carcinoom die doorgaans jonge vrouwen treft en zich presenteert met disfunctioneel vaginaal bloedverlies en pijn in de onderbuik. Metastasen op afstand naar de longen, lever, milt of botten zijn vaak aanwezig op het moment van de diagnose. Het is vaak geassocieerd met risicovolle HPV-infectie (types 18, 16 en 32)."},"pt":{"name":"Carcinoma das células vítreas do colo do útero"},"pl":{"name":"Rak szklistkomórkowy szyjki macicy"},"cs":{"name":"Glassy cell karcinom"},"uk":{"name":"Склоподібно-клітинний рак шийки матки","definition":"Glassy cell carcinoma of the cervix uteri is a rare cancer of the uterine cervix, composed of nests of large neoplastic cells with 'ground glass' cytoplasm, surrounded by a stroma with prominent eosinophilic infiltrates. It is a poorly differentiated, aggressive variant of adenosquamous carcinoma that usually affects young women and presents with dysfunctional vaginal bleeding and lower abdominal pain. Distant metastases to the lungs, liver spleen or bones are often present at the time of diagnosis. It is often associated with high-risk HPV-infection (types 18, 16 and 32)."},"zh":{"name":"子宫颈玻璃状细胞癌","definition":"子宫颈玻璃细胞癌是一种罕见的子宫颈癌，由磨玻璃样细胞质的大肿瘤细胞巢组成，周围有明显嗜酸性基质浸润。它是一种分化不良、侵袭性强的腺鳞癌，通常发生在年轻女性身上，表现为功能失调性阴道出血和下腹部疼痛。常在诊断时发现肺、肝、脾或骨的远处转移。它通常与高危型HPV感染（18型、16型和32型）有关。"},"tr":{"name":"Serviks uterinin camsı hücreli 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.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2084412621"},{"source":"MONDO","code":"4542","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4707881","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":213761,"name":"Rare cancer of cervix uteri","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}