ORPHA:424080
Undifferentiated carcinoma with osteoclast-like giant cells of pancreas
Synonyms: OGCT of pancreas · Osteoclastic giant cell tumor of pancreas · Pancreatic osteoclastic giant cell tumor · Pancreatic undifferentiated carcinoma with osteoclast-like giant cells · Undifferentiated carcinoma of pancreas with osteoclast-like giant cells
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare type of pancreatic ductal adenocarcinoma characterized by composition of non-neoplastic osteoclast-like multinucleated giant cells, a mononuclear histiocytic component, and the neoplastic cells, which vary from spindle-shaped to epithelioid and can be very large and pleomorphic. Clinical features of pancreatic ductal adenocarcinoma include abdominal pain, nausea, weight loss, jaundice, and new-onset diabetes.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Undifferentiated carcinoma with osteoclast-like giant cells of pancreas | OGCT of pancreas · Osteoclastic giant cell tumor of pancreas · Pancreatic osteoclastic giant cell tumor · Pancreatic undifferentiated carcinoma with osteoclast-like giant cells · Undifferentiated carcinoma of pancreas with osteoclast-like giant cells |
| fr | Carcinome indifférencié à cellules géantes de type ostéoclastique du pancréas | Carcinome pancréatique indifférencié à cellules géantes de type ostéoclastique |
| de | Undifferenziertes Karzinom des Pankreas mit osteoklastenähnlichen Riesenzellen | OGCT des Pankreas · Osteoklastischer Riesenzelltumor des Pankreas · Undifferenziertes Pankreaskarzinom mit osteoklastenähnlichen Riesenzellen |
| es | Carcinoma indiferenciado de páncreas con células gigantes tipo osteoclasto | Carcinoma pancreático indiferenciado con células gigantes tipo osteoclasto · TCGO de páncreas · Tumor de células gigantes osteoclásticas de páncreas · Tumor pancreático de células gigantes osteoclásticas |
| it | Carcinoma non differenziato del pancreas con cellule giganti simili ad osteoclasti | Carcinoma pancreatico non differenziato con cellule giganti simili ad osteoclasti · OGCT del pancreas · Tumore osteoclastico a cellule giganti del pancreas · Tumore pancreatico osteoclastico a cellule giganti |
| nl | Ongedifferentieerd carcinoom met osteoclastachtige reuscellen van pancreas | OGCT van pancreas · Ongedifferentieerd carcinoom van pancreas met osteoclastachtige reuscellen · Osteoclastische reusceltumor van pancreas · Pancreatisch ongedifferentieerd carcinoom met osteoclastachtige reuscellen |
| pt | Tumor osteoclástico de células gigantes do pâncreas | Carcinoma pancreático indiferenciado com células gigantes osteoclasto-like · OGCT pancreático |
| pl | Osteoklastyczny olbrzymiokomórkowy guz trzustki | Niezróżnicowany rak trzustki z podobnymi do osteoklastów komórkami olbrzymimi · OGCT trzustki · Rak niezróżnicowany trzustki zbudowany z komórek olbrzymich podobnych do osteoklastów |
| cs | Osteoklastický obrovskobuněčný tumor pankreatu | OGCT pankreatu · Pankreatický osteoklastický obrovskobuněčný tumor · Pankreatický nediferencovaný karcinom s osteoklastoidními obrovskými buňkami · Nediferencovaný karcinom pankreatu s osteoklastoidními obrovskými buňkami |
| uk | Недиференційована карцинома з остеокластоподібними гігантськими клітинами підшлункової залози | OGCT підшлункової залози · Остеокластична гігантоклітинна пухлина підшлункової залози · Остеокластична гігантоклітинна пухлина підшлункової залози · Недиференційована карцинома підшлункової залози з остеокластоподібними гігантськими клітинами · Недиференційована карцинома підшлункової залози з остеокластоподібними гігантськими клітинами |
| zh | 胰腺破骨细胞样巨细胞瘤 | 未分化的胰腺破骨细胞样瘤 |
| tr | Pankreasın osteoklastik dev hücreli tümörü | OGCT pankreası · Pankreatik osteoklastik dev hücreli tümör · Osteoklast ile seyreden dev hücreli pankreatik farklılaşmamış karsinom · Osteoklast ile seyreden dev hücreli farklılaşmamış pankreas karsinomu |
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 | attributed | validated |
| MONDO | 0006479 | E | validated | |
| UMLS | C2007059 | 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
9 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.002 / 100 000 | Belgium | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.006 / 100 000 | Czech Republic | 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 | 0.001 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.043 / 100 000 | Iceland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.001 / 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 | Lithuania | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.004 / 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 | 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/424080
- This record as JSON: /data/d/424080.json