Intraductal tubulopapillary neoplasm of pancreas
Synonyms: ITPN
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare epithelial tumor of pancreas characterized by a solid, nodular mass growing within dilated pancreatic ducts, histologically composed of nodules of back-to-back tubular glands forming large cribriform structures, with high-grade dysplasia and ductal differentiation. There is no overt production of mucin. About half of the tumors occur in the head of the pancreas, one third involve the gland diffusely. Patients present with nonspecific symptoms including abdominal pain, vomiting, weight loss, steatorrhea, and diabetes mellitus, while obstructive jaundice is uncommon. This tumor type accounts for less than 1% of exocrine neoplasms and 3% of intraductal neoplasms of the pancreas.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Intraductal tubulopapillary neoplasm of pancreas | ITPN |
| fr | Carcinome intracanalaire tubulopapillaire du pancréas | = en |
| de | Intraduktale tubulopapilläre Neoplasie des Pankreas | = en |
| es | Neoplasia tubulopapilar intraductal de páncreas | |
| it | Neoplasia tubulopapillare intraduttale del pancreas | = en |
| nl | Intraductaal tubulopapillair neoplasma van pancreas | = en |
| pt | Neoplasia do pâncreas intraductal tubulopapilar | |
| pl | Nowotwór trzustki śródprzewodowy kanalikowo- brodawkowaty | = en |
| cs | Intraduktální tubulopapilární novotvar slinivky břišní | = en |
| uk | Внутрішньопротокова тубулопапілярна пухлина підшлункової залози | = en |
| zh | 胰腺导管内管状乳头状肿瘤 | = en |
| tr | Pankreasın intraduktal tübülopapiller neoplazmı | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C25.3 | NTBT | attributed | validated |
| ICD-11 | 2C10.Y | NTBT | attributed | validated |
| MONDO | 0034987 | E | validated | |
| UMLS | C2987189 | 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:424033Rare epithelial tumor of pancreas
rare gastroenterological diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | Unknown | — | Worldwide | Class only | Validated | 28529742[PMID]_26464736[PMID] |
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/580572
- This record as JSON: /data/d/580572.json