{"orpha":99928,"name":"Placental site trophoblastic tumor","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PSST"],"definition":"A rare gestational trophoblastic neoplasm characterized histologically by invasion of myometrium by intermediate trophoblastic cells without chorionic villi and containing human placental lactogen hormone (hPL). Tissue necrosis is usually absent and hemorrhage is mild. The tumor develops from the placental implantation site and always occurs following pregnancy, voluntary termination of pregnancy (VTP) or miscarriage. Indicative signs are irregular metrorrhagia some time after spontaneous miscarriage or VTP, presence of metastasis or unexplained metrorrhagia in the weeks and months following normal childbirth or ectopic pregnancy.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99928/","orphanet_url":"https://www.orpha.net/en/disease/detail/99928","names":{"fr":{"name":"Tumeur du site d'implantation","synonyms":["Tumeur trophoblastique placentaire"],"definition":"Forme rare de tumeur trophoblastique gestationnelle caractérisée, sur le plan histologique, par l'invasion du myomètre par des cellules intermédiaires du trophoblaste sans villosités choriales, contenant l'hormone lactogène placentaire humaine (hPL). La nécrose des tissus est généralement absente et l'hémorragie est légère. La tumeur se développe à partir du site d'implantation placentaire et fait toujours suite à une grossesse, une interruption volontaire de grossesse (IVG) ou à une fausse couche. Les signes révélateurs sont des métrorragies irrégulières à distance d'un avortement spontané ou d'une IVG, la présence de métastases ou des métrorragies inexpliquées dans les semaines ou mois suivant un accouchement normal ou une grossesse extra-utérine."},"de":{"name":"Plazentabett-Tumor","synonyms":["PSST","Trophoblastentumor am Plazentaansatz"],"definition":"Eine seltene Trophoblastenneoplasie in der Schwangerschaft, die histologisch durch eine Invasion des Myometriums durch intermediäre Trophoblastenzellen ohne Chorionzotten gekennzeichnet ist und humanes Plazenta-Laktogen-Hormon (hPL) enthält. Gewebsnekrosen sind in der Regel nicht vorhanden und die Blutung ist gering. Der Tumor geht von der Nidationsstelle der Plazenta aus und tritt immer nach einer Schwangerschaft, einem Schwangerschaftsabbruch (VTP) oder einer Fehlgeburt auf. Anzeichen sind eine unregelmäßige Metrorrhagie einige Zeit nach einer spontanen Fehlgeburt oder einem VTP, das Vorhandensein von Metastasen oder eine unerklärliche Metrorrhagie in den Wochen und Monaten nach einer normalen Geburt oder einer Eileiterschwangerschaft."},"es":{"name":"Tumor trofoblástico del sitio placentario","definition":"Es una neoplasia trofoblástica gestacional poco frecuente caracterizada histológicamente por la invasión del miometrio por células trofoblásticas intermedias sin vellosidades coriónicas y que contienen el lactógeno placentari humano (hPL). La necrosis tisular suele estar ausente y la hemorragia es leve. El tumor se desarrolla a partir del lugar de implantación de la placenta y siempre se produce tras un embarazo, una interrupción voluntaria del embarazo (IVE) o un aborto espontáneo. Los signos indicativos son la metrorragia irregular algún tiempo después de un aborto espontáneo o IVE, la presencia de metástasis o la metrorragia inexplicable en las semanas y meses siguientes al parto normal o al embarazo ectópico."},"it":{"name":"Tumore trofoblastico della placenta"},"nl":{"name":"Trofoblasttumor aan implantatiezijde van placenta","synonyms":["PSST"],"definition":"Een zeldzaam gestationeel trofoblastisch neoplasma, histologisch gekenmerkt door invasie van myometrium door intermediaire trofoblasten zonder chorionvilli die humaan placentair lactogeen hormoon (hPL) bevatten. Weefselnecrose is meestal afwezig en hemorragie is mild. De tumor ontwikkelt vanuit de implantatieplaats van de placenta en treedt steeds op na zwangerschap, vrijwillige zwangerschapsafbreking of miskraam. Indicatieve verschijnselen zijn onregelmatige intermenstruele bloeding (metrorragie) enige tijd na spontaan miskraam of vrijwillige zwangerschapsafbreking, aanwezigheid van metastase of onverklaarbare metrorragie in de weken en maanden na normale bevalling of buitenbaarmoederlijke zwangerschap."},"pt":{"name":"Tumor trofoblástico do local da placenta (PSST)","definition":"Neoplasia trofoblástica gestacional rara caracterizada histologicamente pela invasão do miométrio por células trofoblásticas intermediárias sem vilosidades coriónicas contendo hormona lactogénica placentária humana (hPL). A necrose tecidual geralmente está ausente e a hemorragia é ligeira. O tumor desenvolve-se a partir do local de implantação da placenta e ocorre após a gravidez, interrupção voluntária da gravidez (PTV) ou aborto espontâneo. Os sinais indicativos são o aparecimento de metrorragia irregular após aborto espontâneo ou PTV, presença de metástase ou metrorragia inexplicável nas semanas e meses após o parto normal ou gravidez ectópica."},"pl":{"name":"Guz trofoblastyczny miejsca łożyskowego","synonyms":["PSST"],"definition":"Rzadki ciążowy nowotwór trofoblastu, histologicznie charakteryzujący się naciekaniem mięśniówki macicy przez pośrednie komórki trofoblastyczne bez kosmków kosmówkowych, zawierające ludzki hormon laktogenowy łożyska (hPL). Martwica tkanek zwykle nie występuje, a krwotok jest łagodny. Guz rozwija się w miejscu implantacji łożyska i pojawia się po ciąży, po przerwaniu ciąży lub poronieniu. Charakterystyczne objawy obejmują nieregularne krwotoki z macicy jakiś czas po poronieniu samoistnym lub po przerwaniu ciąży, obecność przerzutów lub niewyjaśnione krwotoki z macicy w tygodniach i miesiącach po prawidłowym porodzie lub ciąży pozamacicznej."},"cs":{"name":"Cytotrofoblastický choriokarcinom","synonyms":["PSST"]},"uk":{"name":"Трофобластична пухлина плацентарної ділянки","synonyms":["PSST"],"definition":"A rare gestational trophoblastic neoplasm characterized histologically by invasion of myometrium by intermediate trophoblastic cells without chorionic villi and containing human placental lactogen hormone (hPL). Tissue necrosis is usually absent and hemorrhage is mild. The tumor develops from the placental implantation site and always occurs following pregnancy, voluntary termination of pregnancy (VTP) or miscarriage. Indicative signs are irregular metrorrhagia some time after spontaneous miscarriage or VTP, presence of metastasis or unexplained metrorrhagia in the weeks and months following normal childbirth or ectopic pregnancy."},"zh":{"name":"胎盘部位滋养细胞肿瘤","synonyms":["PSST"],"definition":"胎盘部位滋养细胞肿瘤是一种罕见的妊娠滋养细胞肿瘤，起源于胎盘植入部位，常发生在妊娠、自愿终止妊娠(VTP)或流产后。"},"tr":{"name":"Plasental bölge trofoblastik tümör","synonyms":["PSST"]}},"xrefs":[{"source":"ICD-10","code":"D39.2","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"2F31.2","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2089729304"},{"source":"ICD-11","code":"2F76","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"782186074"},{"source":"ICD-11","code":"2F96","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"2030427600"},{"source":"ICD-11","code":"XH1RM5","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"959500275"},{"source":"MONDO","code":"0020552","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D018245","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0206666","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7403","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":59305,"name":"Gestational trophoblastic neoplasm","classifications":[202,205]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.02,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Lifetime Prevalence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.86,"area":"Europe","status":"Validated","source":"22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":"14614893[PMID]","terms":[{"hpo":"HP:0000141","label":"Amenorrhea","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011434","label":"Low 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:0005268","label":"Spontaneous abortion","frequency":"Very frequent (99-80%)","criteria":null}]}}