Primary peritoneal carcinoma
Synonyms: EOPPC · Extra-ovarian primary peritoneal carcinoma · PPC · Primary peritoneal serous carcinoma · Serous surface papillary carcinoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Adult
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary peritoneal carcinoma | EOPPC · Extra-ovarian primary peritoneal carcinoma · PPC · Primary peritoneal serous carcinoma · Serous surface papillary carcinoma |
| fr | Carcinome péritonéal primaire | CPP · Carcinome papillaire séreux du péritoine · Carcinome péritonéal primitif extra-ovarien · Carcinome séreux primitif du péritoine · Carcinome séreux péritonéal primitif |
| de | Peritonealkarzinom, primäres | Oberflächenkarzinom, seröses papilliäres |
| es | Carcinoma primario peritoneal | CPP · CPPEO · Carcinoma papilar de la superficie serosa · Carcinoma peritoneal primario seroso · Carcinoma primario peritoneal extraovárico · EOPPC · PPC |
| it | Carcinoma peritoneale primitivo | Carcinoma sieroso peritoneale primitivo · EOPPC · PPC · Carcinoma papillare superficiale sieroso · Carcinoma peritoneale primitivo extra-ovarico |
| nl | Primair peritoneaal carcinoom | EOPPC · Extra-ovarieel primair peritoneaal carcinoom · PPC · Primair peritoneaal sereus carcinoom · Sereus oppervlak papilcarcinoom |
| pt | Carcinoma peritoneal primário | |
| pl | Pierwotny rak otrzewnej | EOPPC · Pierwotny rak surowiczy otrzewnej · Powierzchniowy surowiczy brodawkowaty rak · Pozajajnikowy pierwotny rak otrzewnej · PPC |
| cs | Primární peritoneální karcinom | EOPPC · Extraovariální primární peritoneální karcinom · PPC · Primární peritoneální serózní karcinom · Serózní papilární karcinom peritonea |
| uk | Первинна перитоніальна карцинома | EOPPC · Позаяєчникова первинна карцинома очеревини · PPC · Первинна серозна карцинома очеревини · Серозна поверхнева папілярна карцинома |
| zh | 原发性腹膜癌 | 卵巢外原发性腹膜癌 |
| tr | Primer periton karsinomu | TBÖ · EOPPC · Seröz yüzey papiller karsinom · Primer peritoneal seröz karsinom · Ekstra yumurtalık primer peritoneal karsinom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C48.2 | NTBT | attributed | validated |
| MONDO | 0015686 | E | validated | |
| UMLS | C0334361 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:168807Primary malignant peritoneal tumor
rare abdominal surgical diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Codes that Orphanet moved or referred to this one:
- ORPHA:398980OBSOLETE: Primary peritoneal serous/papillary carcinoma referred to · inactive
Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| BRCA1 | Major susceptibility factor in | Assessed | HGNC:1100 | ENSG00000012048 | 113705 | 17q21.31 | BRCA1 DNA repair associated | 22006311[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
6 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0003270 | Abdominal distention | |
| HP:0002027 | Abdominal pain | |
| HP:0002019 | Constipation | |
| HP:0002017 | Nausea and vomiting | |
| HP:0002664 | Neoplasm | |
| HP:0002586 | Peritonitis | |
Run the differential on the 6 obligate / very frequent terms
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/168829
- This record as JSON: /data/d/168829.json