{"orpha":168829,"name":"Primary peritoneal carcinoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["EOPPC","Extra-ovarian primary peritoneal carcinoma","PPC","Primary peritoneal serous carcinoma","Serous surface papillary carcinoma"],"definition":"Primary peritoneal carcinoma (PPC) is a rare malignant tumor of the peritoneal cavity of extra-ovarian origin, clinically and histologically similar to advanced-stage serous ovarian carcinoma.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-168829/","orphanet_url":"https://www.orpha.net/en/disease/detail/168829","names":{"fr":{"name":"Carcinome péritonéal primaire","synonyms":["CPP","Carcinome papillaire séreux du péritoine","Carcinome péritonéal primitif extra-ovarien","Carcinome séreux primitif du péritoine","Carcinome séreux péritonéal primitif"],"definition":"Le carcinome péritonéal primitif (CPP) est une tumeur maligne rare de la cavité péritonéale d'origine extra-ovarienne, cliniquement et histologiquement semblable à un carcinome séreux ovarien de degré élevé."},"de":{"name":"Peritonealkarzinom, primäres","synonyms":["Oberflächenkarzinom, seröses papilliäres"],"definition":"Das primäre peritoneale Karzinom (PPC) ist ein seltener maligner Tumor der Peritonealhöhle. Sein Ursprung liegt außerhalb der Ovarien, aber es ähnelt klinisch und histologisch fortgeschrittenen Stadien des serösen Ovarial-Karzinoms (s. dort)."},"es":{"name":"Carcinoma primario peritoneal","synonyms":["CPP","CPPEO","Carcinoma papilar de la superficie serosa","Carcinoma peritoneal primario seroso","Carcinoma primario peritoneal extraovárico","EOPPC","PPC"],"definition":"El carcinoma peritoneal primario (CPP) es un tumor maligno raro de la cavidad peritoneal de origen extra-ovárico, clínica e histológicamente similar a los estadios avanzados del carcinoma seroso de ovario (ver este término)."},"it":{"name":"Carcinoma peritoneale primitivo","synonyms":["Carcinoma sieroso peritoneale primitivo","EOPPC","PPC","Carcinoma papillare superficiale sieroso","Carcinoma peritoneale primitivo extra-ovarico"]},"nl":{"name":"Primair peritoneaal carcinoom","synonyms":["EOPPC","Extra-ovarieel primair peritoneaal carcinoom","PPC","Primair peritoneaal sereus carcinoom","Sereus oppervlak papilcarcinoom"],"definition":"Primair peritoneaal carcinoom (PPC) is een zeldzame kwaadaardige tumor van de peritoneale holte van extra-ovariële oorsprong, die klinisch en histologisch gelijkt op sereus ovariumcarcinoom in een gevorderd stadium (zie deze term)."},"pt":{"name":"Carcinoma peritoneal primário","definition":"O carcinoma peritoneal primário (PPC) é um tumor maligno raro da cavidade peritoneal de origem extra-ovarica, clínica e histologicamente semelhante ao carcinoma seroso do ovário de estadio avançado (ver este termo)."},"pl":{"name":"Pierwotny rak otrzewnej","synonyms":["EOPPC","Pierwotny rak surowiczy otrzewnej","Powierzchniowy surowiczy brodawkowaty rak","Pozajajnikowy pierwotny rak otrzewnej","PPC"]},"cs":{"name":"Primární peritoneální karcinom","synonyms":["EOPPC","Extraovariální primární peritoneální karcinom","PPC","Primární peritoneální serózní karcinom","Serózní papilární karcinom peritonea"]},"uk":{"name":"Первинна перитоніальна карцинома","synonyms":["EOPPC","Позаяєчникова первинна карцинома очеревини","PPC","Первинна серозна карцинома очеревини","Серозна поверхнева папілярна карцинома"],"definition":"Primary peritoneal carcinoma (PPC) is a rare malignant tumor of the peritoneal cavity of extra-ovarian origin, clinically and histologically similar to advanced-stage serous ovarian carcinoma (see this term)."},"zh":{"name":"原发性腹膜癌","synonyms":["卵巢外原发性腹膜癌"],"definition":"原发性腹膜癌（PPC）是一种罕见的腹腔内卵巢外恶性肿瘤，临床表现和组织形态与晚期浆液性卵巢癌相似（见该术语）。"},"tr":{"name":"Primer periton karsinomu","synonyms":["TBÖ","EOPPC","Seröz yüzey papiller karsinom","Primer peritoneal seröz karsinom","Ekstra yumurtalık primer peritoneal karsinom"]}},"xrefs":[{"source":"ICD-10","code":"C48.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0015686","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0334361","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":398980,"name":"OBSOLETE: Primary peritoneal serous/papillary carcinoma","status":"inactive"}],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":168807,"name":"Primary malignant peritoneal tumor","classifications":[182,202,233]}],"children":[],"genes":[{"symbol":"BRCA1","name":"BRCA1 DNA repair associated","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Assessed","source":"22006311[PMID]","hgnc":"1100","ensembl":"ENSG00000012048","omim":"113705","loci":["17q21.31"]}],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"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:0002019","label":"Constipation","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","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}]}}