{"orpha":99926,"name":"Gestational choriocarcinoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A form of gestational trophoblastic neoplasia characterized histologically by trophoblast proliferation, absence of chorionic villi (except in cases of intraplacental choriocarcinoma) and tissue necrosis with bleeding. The tumor occurs secondary to pregnancy (ectopic or normal), miscarriage, voluntary termination of pregnancy (VTP) or a hydatidiform mole. Indicative signs are persistent unexplained metrorrhagia or secondary increase, stagnation, or non-normalization at 6 months of total serum chorionic gonadotropin (hCG) levels after removal of a hydatidiform mole; persistent unexplained metrorrhagia following spontaneous abortion or VTP; occasionally unexplained metrorrhagia in the weeks or months following normal childbirth or an ectopic pregnancy. Occasionally, metastases (lung, liver, brain, kidneys, vagina) are indicative signs in women of childbearing age.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99926/","orphanet_url":"https://www.orpha.net/en/disease/detail/99926","names":{"fr":{"name":"Choriocarcinome gestationnel","definition":"Forme de tumeur trophoblastique gestationnelle caractérisée, sur le plan histologique, par la prolifération de tissus trophoblastiques, l'absence de villosités choriales (sauf en cas de choriocarcinome gestationnel intraplacentaire) et la nécrose des tissus avec hémorragie. La tumeur est secondaire à une grossesse (extra-utérine ou normale), à une fausse couche, à une interruption volontaire de grossesse (IVG) ou à une môle hydatiforme. Les signes révélateurs sont des métrorragies persistantes et inexpliquées ou une ré-ascension, une stagnation ou une absence de normalisation à six mois des taux de choriogonadotrophine (hCG) totale sérique après évacuation d'une môle hydatiforme ; des métrorragies persistantes inexpliquées après un avortement spontané ou une IVG ; occasionnellement des métrorragies inexpliquées dans les semaines ou mois suivant un accouchement normal ou une grossesse extra-utérine. Occasionnellement, des métastases (pulmonaires, hépatiques, cérébrales, rénales ou vaginales) sont révélatrices chez les femmes en âge de procréer."},"de":{"name":"Chorionkarzinom, gestationales","synonyms":["Schwangerschafts-Chorionkarzinom"],"definition":"Eine Form der gestationalen trophoblastischen Neoplasie, die histologisch durch eine Trophoblastenproliferation, das Fehlen von Chorionzotten (außer bei intraplazentarem Choriokarzinom) und Gewebsnekrose mit Blutungen gekennzeichnet ist. Der Tumor tritt sekundär nach einer (ektopischen oder normalen) Schwangerschaft, einer Fehlgeburt, einem freiwilligen Schwangerschaftsabbruch (VTP) oder einer hydatidiformen Mole auf. Anzeichen dafür sind eine anhaltende ungeklärte Metrorrhagie oder ein sekundärer Anstieg, eine Stagnation oder eine Nichtnormalisierung des Gesamtserumspiegels an Choriongonadotropin (hCG) 6 Monate nach der Entfernung einer hydatidiformen Mole; eine anhaltende ungeklärte Metrorrhagie nach einem Spontanabort oder einer VTP; gelegentlich eine ungeklärte Metrorrhagie in den Wochen oder Monaten nach einer normalen Entbindung oder einer ektopen Schwangerschaft. Gelegentlich sind Metastasen (Lunge, Leber, Gehirn, Nieren, Vagina) bei Frauen im gebärfähigen Alter ein Indiz."},"es":{"name":"Coriocarcinoma gestacional","definition":"Es una forma de neoplasia trofoblástica gestacional caracterizada histológicamente por la proliferación de trofoblastos, ausencia de vellosidades coriónicas (excepto en los casos de coriocarcinoma intraplacentario) y necrosis tisular con hemorragia. El tumor es secundario a un embarazo (ectópico o normal), a un aborto espontáneo, a una interrupción voluntaria del embarazo (IVE) o a una mola hidatiforme. Los signos indicativos son la metrorragia persistente e inexplicable o el aumento secundario, el estancamiento o la no normalización a los 6 meses de los niveles de gonadotropina coriónica total (hCG) tras la extirpación de una mola hidatiforme; la metrorragia persistente e inexplicable tras un aborto espontáneo o una IVE; ocasionalmente, la metrorragia inexplicable en las semanas o meses posteriores a un parto normal o a un embarazo ectópico. Ocasionalmente, las metástasis (pulmón, hígado, cerebro, riñones, vagina) son signos indicativos en mujeres en edad fértil."},"it":{"name":"Coriocarcinoma gestazionale"},"nl":{"name":"Gestationeel choriocarcinoom","synonyms":["Zwangerschapsgerelateerd choriocarcinoom"],"definition":"Een vorm van gestationele trofoblastische neoplasie, histologisch gekenmerkt door proliferatie van trofoblasten, afwezigheid van chorionvilli (behalve in geval van intraplacentair choriocarcinoom) en weefselnecrose met bloeding. De tumor treedt secundair op aan zwangerschap (ectopisch of normaal), miskraam, vrijwillige zwangerschapsafbreking of mola hydatidosa (mola-zwangerschap). Indicatieve verschijnselen zijn aanhoudende onverklaarbare metrorragie (onregelmatige intermenstruele bloeding) of secundaire toename, stagnatie of niet-normalisatie van totale gehaltes van choriongonadotrofine (hCG) in serum zes maanden na verwijdering van mola hydatidosa; aanhoudende onverklaarbare metrorragie na spontane abortus of vrijwillige zwangerschapsafbreking; occasionele onverklaarbare metrorragie in de weken of maanden volgend op normale bevalling of buitenbaarmoederlijke zwangerschap. Soms zijn uitzaaiingen (long, lever, hersenen, nier, vagina) indicatieve verschijnselen bij vrouwen op vruchtbare leeftijd."},"pt":{"name":"Coriocarcinoma gestacional","definition":"Forma de neoplasia trofoblástica gestacional caracterizada histologicamente por proliferação trofoblástica, ausência de vilosidades coriónicas (exceto em casos de coriocarcinoma intraplacentário) e necrose tecidual com sangramento. O tumor ocorre secundário à gravidez (ectópica ou normal), aborto espontâneo, interrupção voluntária da gravidez (PTV) ou mola hidatiforme. Os sinais são a metrorragia persistente e inexplicável com agravamento secundário, estabilização ou não normalização dentro de 6 meses dos níveis séricos totais de gonadotrofina coriónica (hCG) após a remoção de uma mola hidatiforme; metrorragia persistente e inexplicável após aborto espontâneo ou TVP; metrorragia ocasionalmente inexplicável nas semanas ou meses após o parto normal ou uma gravidez ectópica. Ocasionalmente, metástases (pulmão, fígado, cérebro, rins, vagina) são sinais indicativos em indivíduos em idade fértil."},"pl":{"name":"Kosmówczak ciążowy","definition":"Postać ciążowej choroby trofoblastycznej, która histologicznie charakteryzuje się proliferacją trofoblastów, brakiem kosmków kosmówki (z wyjątkiem przypadków kosmówczaka wewnątrzłożyskowego) oraz martwicą tkanek z krwawieniem. Guz występuje wtórnie do ciąży (ektopowej lub prawidłowej), poronienia, przerwania ciąży lub zaśniadu groniastego. Objawami choroby są utrzymujące się niewyjaśnione krwotoki maciczny lub wtórny wzrost, stagnacja lub brak normalizacji po 6 miesiącach stężenia gonadotropiny kosmówkowej (hCG) w surowicy po usunięciu zaśniadu groniastego; uporczywe niewyjaśnione krwotoki z macicy po spontanicznym poronieniu lub po przerwaniu ciąży; rzadkiej niewyjaśniony krwotok maciczny w tygodniach lub miesiącach po normalnym porodzie lub ciąży pozamacicznej. Czasami objawem wskaźnikowym u kobiet w wieku rozrodczym są przerzuty (do płuc, wątroby, mózgu, nerek, pochwy)."},"cs":{"name":"Gestační choriokarcinom"},"uk":{"name":"Гестаційна хоріокарцинома","definition":"A form of gestational trophoblastic neoplasia characterized histologically by trophoblast proliferation, absence of chorionic villi (except in cases of intraplacental choriocarcinoma) and tissue necrosis with bleeding. The tumor occurs secondary to pregnancy (ectopic or normal), miscarriage, voluntary termination of pregnancy (VTP) or a hydatidiform mole. Indicative signs are persistent unexplained metrorrhagia or secondary increase, stagnation, or non-normalization at 6 months of total serum chorionic gonadotropin (hCG) levels after removal of a hydatidiform mole; persistent unexplained metrorrhagia following spontaneous abortion or VTP; occasionally unexplained metrorrhagia in the weeks or months following normal childbirth or an ectopic pregnancy. Occasionally, metastases (lung, liver, brain, kidneys, vagina) are indicative signs in women of childbearing age."},"zh":{"name":"妊娠性绒毛膜癌","definition":"妊娠绒毛膜癌是一种妊娠滋养细胞肿瘤(GTT，见该词条)，继发于妊娠(异位或正常)、流产、自愿终止妊娠(VTP)或葡萄胎(见该词条)。"},"tr":{"name":"Gestasyonel koryokarsinom"}},"xrefs":[{"source":"ICD-10","code":"C58","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C75.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2136066651"},{"source":"MONDO","code":"0020550","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0349557","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":99913,"name":"Extragonadal non-dysgerminomatous germ cell tumor","classifications":[202,233]},{"orpha":59305,"name":"Gestational trophoblastic neoplasm","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"},{"type":"Lifetime Prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-06-03","source":"23999759[PMID]","terms":[{"hpo":"HP:0100768","label":"Choriocarcinoma","frequency":"Obligate (100%)","criteria":"Diagnostic criterion"},{"hpo":"HP:0011433","label":"High maternal serum chorionic gonadotropin","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100608","label":"Metrorrhagia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002664","label":"Neoplasm","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0005268","label":"Spontaneous abortion","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0031502","label":"Trophoblastic tumor","frequency":"Very frequent (99-80%)","criteria":null}]}}