{"orpha":168811,"name":"Malignant peritoneal mesothelioma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Diffuse malignant peritoneal mesothelioma","Primary malignant peritoneal mesothelioma"],"definition":"Malignant peritoneal mesothelioma is a primary peritoneal malignancy occurring in the lining cells (mesothelium) of the peritoneal cavity.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-168811/","orphanet_url":"https://www.orpha.net/en/disease/detail/168811","names":{"fr":{"name":"Mésothéliome péritonéal malin","synonyms":["Mésothéliome malin primitif","Mésothéliome péritonéal malin diffus"],"definition":"Le mésothéliome péritonéal malin (MPM) est une tumeur maligne péritonéale primitive des cellules pariétales (mésothélium) de la cavité péritonéale. Le mésothéliome péritonéal représente 10 à 30% des mésothéliomes malins."},"de":{"name":"Peritonealmesotheliom, malignes","synonyms":["Mesotheliom, malignes primäres"],"definition":"Das Maligne peritoneale Mesotheliom ist ein primäres Malignom des Peritoneums, das in den Deckzellen (Mesothel) des Peritoneums entsteht. Es macht 10-30% aller malignen Mesotheliome aus."},"es":{"name":"Mesotelioma peritoneal maligno","synonyms":["Mesotelioma peritoneal maligno difuso","Mesotelioma peritoneal maligno primario"],"definition":"El mesotelioma peritoneal maligno es un tumor primario peritoneal que se desarrolla a partir de las células epiteliales (mesotelio) de la cavidad peritoneal."},"it":{"name":"Mesotelioma peritoneale maligno","synonyms":["Mesotelioma peritoneale maligno diffuso","Mesotelioma peritoneale maligno primitivo"],"definition":"Il mesotelioma peritoneale maligno è un tumore primitivo del peritoneo che origina dalle cellule parietali (mesotelio) della cavità peritoneale. Il mesotelioma peritoneale rappresenta il 10-30% dei mesoteliomi maligni."},"nl":{"name":"Maligne peritoneaal mesothelioom","synonyms":["Diffuus maligne peritoneaal mesothelioom","Kwaadaardig peritoneaal mesothelioom","Primair maligne peritoneaal mesothelioom"],"definition":"Kwaadaardig peritoneaal mesothelioom is een primaire peritoneale maligniteit die in de dekcellen (mesotheel) van de peritoneale holte optreedt."},"pt":{"name":"Mesotelioma peritoneal maligno","definition":"O mesotelioma peritoneal maligno é uma neoplasia maligna peritoneal primária com origem nas células que revestem (mesotélio) a cavidade peritoneal."},"pl":{"name":"Złośliwy międzybłoniak otrzewnej","synonyms":["Pierwotny złośliwy międzybłoniak otrzewnej","Rozlany złośliwy międzybłoniak otrzewnej"]},"cs":{"name":"Peritoneální maligní mezoteliom","synonyms":["Difuzní peritoneální maligní mezoteliom","Primární peritoneální maligní mezoteliom"]},"uk":{"name":"Злоякісна перитоніальна мезотеліома","synonyms":["Дифузна злоякісна мезотеліома очеревини","Первинна злоякісна мезотеліома очеревини"],"definition":"Malignant peritoneal mesothelioma is a primary peritoneal malignancy occurring in the lining cells (mesothelium) of the peritoneal cavity."},"zh":{"name":"恶性腹膜间皮瘤","synonyms":["原发性恶性腹膜间皮瘤"],"definition":"恶性腹膜间皮瘤是一种原发于腹膜内皮细胞（间皮）的腹膜恶性肿瘤。"},"tr":{"name":"Habis peritoneal mezotelyoma","synonyms":["Yaygın habis peritoneal mezotelyoma","Primer habis peritoneal mezotelyoma"]}},"xrefs":[{"source":"ICD-10","code":"C45.1","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2C51.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1934564626"},{"source":"MONDO","code":"0005512","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0346109","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10056558","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":168807,"name":"Primary malignant peritoneal tumor","classifications":[182,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.2,"area":"France","status":"Validated","source":"[EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"1-9 / 100 000","value":0.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2016[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.5,"area":"Italy","status":"Validated","source":"[EXPERT]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0003270","label":"Abdominal distention","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001541","label":"Ascites","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002664","label":"Neoplasm","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002586","label":"Peritonitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001928","label":"Abnormality of coagulation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002595","label":"Ileus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010741","label":"Pedal edema","frequency":"Occasional (29-5%)","criteria":null}]}}