Intraductal papillary mucinous carcinoma of pancreas
Synonyms: IPMN · Pancreatic intraductal papillary mucinous carcinoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Intraductal papillary mucinous carcinoma of pancreas is a rare epithelial tumor of pancreas characterized by malignant, mucin-producing cystic mass, originating from the pancreatic ductal system, associated with local invasion and metastatic spread, composed of mucin-producing, columnar epithelial cells covering the dilated pancreatic ducts with a papillary structure. The presenting symptoms are non-specific and include abdominal pain, pancreatitis, steatorrhea, jaundice and diabetes. Many patients are asymptomatic at the time of diagnosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Intraductal papillary mucinous carcinoma of pancreas | IPMN · Pancreatic intraductal papillary mucinous carcinoma |
| fr | Carcinome intracanalaire papillaire mucineux du pancréas | Carcinome intracanalaire papillaire mucineux pancréatique · TIPMP · Tumeur intracanalaire papillaire mucineuse du pancréas |
| de | Intraduktale papilläre muzinöse Neoplasie des Pankreas | IPMN |
| es | Carcinoma intraductal papilar mucinoso de páncreas | Carcinoma pancreático mucinoso intraductal papilar |
| it | Carcinoma mucinoso papillare intraduttale del pancreas | Carcinoma pancreatico mucinoso papillare intraduttale |
| nl | Intraductaal papillair mucineus carcinoom van pancreas | Intraductaal papillair mucineus carcinoom van alvleesklier · Pancreatisch intraductaal papillair mucineus carcinoom |
| pt | Carcinoma mucinoso papilar intraductal do pâncreas | |
| pl | Wewnątrzprzewodowy brodawkowaty rak śluzowy trzustki | = en |
| cs | Intraduktální papilární mucinózní karcinom pankreatu | Pankreatický intraduktální papilární mucinózní karcinom |
| uk | Внутрішньопротокова папілярна муцинозна карцинома підшлункової залози | IPMN · Внутрішньопротокова папілярна муцинозна карцинома підшлункової залози |
| zh | 胰管内乳头状黏液性癌 | 胰管内乳头状黏液性癌 |
| tr | Pankreasın intraduktal papiller müsinöz karsinomu | IPMN · Pankreatik intraduktal papiller müsinöz karsinom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C25.3 | NTBT | attributed | validated |
| ICD-11 | 2C10.0 | NTBT | index | validated |
| MONDO | 0004285 | E | validated | |
| UMLS | C1335304 | E | validated | |
| MedDRA | 10073365 | 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
13 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.018 / 100 000 | Spain | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.028 / 100 000 | Switzerland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.016 / 100 000 | Netherlands | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.001 / 100 000 | United Kingdom | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.011 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.017 / 100 000 | Belgium | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.028 / 100 000 | Czech Republic | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.031 / 100 000 | France | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.048 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.006 / 100 000 | Ireland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.006 / 100 000 | Italy | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.041 / 100 000 | Norway | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.006 / 100 000 | Portugal | Value and class | 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/424058
- This record as JSON: /data/d/424058.json