Mucinous cystadenocarcinoma of the pancreas
Synonyms: Pancreatic mucinous cystadenocarcinoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare, epithelial tumor of the pancreas characterized, histologically, by columnar, mucin-producing epithelium associated with ovarian-type subepithelial stroma, which does not communicate with the pancreatic ductal system, most frequently localized to the body or tail of the pancreas. Clinically, small tumors (<3 cm) are usually asymptomatic, while large tumors typically present obstructive jaundice, a palpable abdominal mass, and may associate portal hypertension, hemobilia and diabetes mellitus.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Mucinous cystadenocarcinoma of the pancreas | Pancreatic mucinous cystadenocarcinoma |
| fr | Cystadénocarcinome mucineux du pancréas | Cystadénocarcinome mucineux pancréatique |
| de | Muzinöses Zystadenokarzinom des Pankreas | MCN · Muzinös-zystische Neoplasie des Pankreas |
| es | Cistoadenocarcinoma mucinoso de páncreas | Cistoadenocarcinoma pancreático mucinoso |
| it | Cistadenocarcinoma mucinoso del pancreas | Cistadenocarcinoma mucinoso pancreatico |
| nl | Mucineus cystadenocarcinoom van pancreas | Mucineus cystadenocarcinoom van alvleesklier · Pancreatisch mucineus cystadenocarcinoom |
| pt | Cistoadenocarcinoma mucinoso do pâncreas | |
| pl | Torbielogruczolakorak śluzowy trzustki | = en |
| cs | Mucinózní cystadenokarcinom pankreatu | Pankreatický mucinózní cystadenokarcinom |
| uk | Муцинозна цистаденокарцинома підшлункової залози | Муцинозна цистаденокарцинома підшлункової залози |
| zh | 胰腺粘液性囊腺癌 | 胰腺粘液性囊腺癌 |
| tr | Pankreasın müsinöz kistadenokarsinomu | Pankreatik müsinöz kistadenokarsinomu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C25.0 | BTNT | attributed | validated |
| ICD-10 | C25.1 | BTNT | attributed | validated |
| ICD-10 | C25.2 | BTNT | attributed | validated |
| ICD-10 | C25.7 | BTNT | attributed | validated |
| ICD-10 | C25.8 | BTNT | attributed | validated |
| ICD-11 | 2C10.0 | NTBT | index | validated |
| MONDO | 0018523 | E | validated | |
| MONDO | 18523 | E | validated | |
| UMLS | C2063873 | 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:217074Rare carcinoma of pancreas
rare gastroenterological diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
14 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.01 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.006 / 100 000 | Belgium | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.002 / 100 000 | Bulgaria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.002 / 100 000 | Czech Republic | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.012 / 100 000 | Finland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.003 / 100 000 | France | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.012 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.019 / 100 000 | Italy | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.004 / 100 000 | Portugal | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.024 / 100 000 | Spain | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.006 / 100 000 | Switzerland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.019 / 100 000 | Netherlands | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.004 / 100 000 | United Kingdom | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Point prevalence | Unknown | — | Europe | Class only | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/424053
- This record as JSON: /data/d/424053.json