ORPHA:424065
Pancreatic solid pseudopapillary neoplasm
Synonyms: Frantz tumor · SPN · Solid pseudopapillary carcinoma of pancreas · Solid pseudopapillary neoplasm of the pancreas
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare carcinoma of the pancreas characterized by a variable combination of nonspecific signs and symptoms, such as abdominal pain, jaundice, abdominal fullness, anorexia, nausea, vomiting, and weight loss. One-third of the patients are asymptomatic. The tumor has low malignant potential, but can invade locally.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pancreatic solid pseudopapillary neoplasm | Frantz tumor · SPN · Solid pseudopapillary carcinoma of pancreas · Solid pseudopapillary neoplasm of the pancreas |
| fr | Tumeur pseudopapillaire et solide du pancréas | Carcinome pseudo-papillaire et solide du pancréas · Carcinome pseudo-papillaire et solide pancréatique · TPPSP · Tumeur de Frantz · Tumeur pseudo-papillaire et solide pancréatique |
| de | Solides pseudopapilläres Karzinom des Pankreas | Frantz-Tumor · Pankreatisches solides pseudopapilläres Karzinom · SPN · Solid-pseudopapilläre Neoplasie des Pankreas · Solide pseudopapilläre Neoplasie des Pankreas |
| es | Neoplasia sólida pseudopapilar pancreática | Carcinoma pancreático sólido pseudopapilar · Neoplasia sólida pseudopapilar de páncreas |
| it | Carcinoma pancreatico pseudopapillare solido | |
| nl | Solide pseudopapillair neoplasma van pancreas | Frantz-tumor · SPN · Solide pseudopapillair carcinoom van pancreas · Solide pseudopapillair neoplasma van alvleesklier |
| pt | Carcinoma sólido pseudopapilar do pâncreas | SPN · Tumor Frantz · Neoplasia sólida pseudopapilar do pâncreas |
| pl | Lity rzekomobrodawkowaty rak trzustki | = en |
| cs | Solidní pseudopapilární karcinom pankreatu | Pankreatický solidní pseudopapilární karcinom |
| uk | Солідна псевдопапілярна карцинома підшлункової залози | Солідна псевдопапілярна карцинома підшлункової залози · Солідна псевдопапілярна пухлина підшлункової залози |
| zh | 胰腺实性假乳头状癌 | 胰腺实性假乳头状癌 |
| tr | Pankreasın katı psödopapiller karsinomu | Pankreatik solid psödopapiller karsinom · Pankreasın katı psödopapiller neoplazmı |
Definition in:
2
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 | 0018525 | E | validated | |
| UMLS | C1336029 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:217074Rare carcinoma of pancreas
rare gastroenterological diseases; rare neoplastic diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
13 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
| Annual incidence | <1 / 1 000 000 | 0.003 / 100 000 | Austria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.008 / 100 000 | Belgium | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.003 / 100 000 | Croatia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.011 / 100 000 | Czech Republic | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.005 / 100 000 | France | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.004 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.005 / 100 000 | Italy | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.003 / 100 000 | Norway | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.009 / 100 000 | Spain | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.002 / 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 | 0.003 / 100 000 | Europe | 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.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/424065
- This record as JSON: /data/d/424065.json