ORPHA:300385
Pituitary carcinoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- All ages
- Status
- active
A rare pituitary tumor characterized by the presence of a pituitary adenoma that has metastasized either within the central nervous system, or to distant sites. The vast majority of pituitary carcinomas are hormonally active, most frequently with ACTH or prolactin production. The most common clinical symptoms are diabetes insipidus, optic nerve dysfunction, anterior pituitary dysfunction, palsy of cranial nerves III, IV, or VI, and headaches, although patients may also be asymptomatic. The tumors behave aggressively, and prognosis is poor.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pituitary carcinoma | |
| fr | Carcinome hypophysaire | Carcinome pituitaire |
| de | Hypophysenkarzinom | Karzinom der Glandula pituitaria |
| es | Carcinoma hipofisario | |
| it | Carcinoma ipofisario | |
| nl | Hypofysecarcinoom | Carcinoom van hypofyse |
| pt | Carcinoma hipofisário | |
| pl | Rak przysadki | |
| cs | Pituitární karcinom | |
| uk | Карцинома гіпофіза | |
| zh | 垂体癌 | |
| tr | Hipofiz karsinomu |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C75.1 | NTBT | attributed | validated |
| MONDO | 0017582 | E | validated | |
| MONDO | 17582 | E | validated | |
| UMLS | C0346300 | E | validated | |
| GARD | 9371 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:304055Pituitary tumor
rare neoplastic diseases
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
26 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.04 / 100 000 | Europe | Value and class | Validated | 22361014[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER] |
| Lifetime Prevalence | 1-9 / 1 000 000 | 0.87 / 100 000 | Europe | Value and class | Validated | 22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER] |
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
| Annual incidence | <1 / 1 000 000 | 0.025 / 100 000 | Norway | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.008 / 100 000 | Portugal | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.021 / 100 000 | Slovakia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.006 / 100 000 | Slovenia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.021 / 100 000 | Spain | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.026 / 100 000 | Austria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.041 / 100 000 | Belgium | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.014 / 100 000 | Bulgaria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.037 / 100 000 | Czech Republic | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.009 / 100 000 | Estonia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.019 / 100 000 | Finland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.033 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.099 / 100 000 | Ireland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.052 / 100 000 | Italy | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.016 / 100 000 | Latvia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.025 / 100 000 | Lithuania | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.063 / 100 000 | Malta | 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 | 0.015 / 100 000 | Netherlands | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | <1 / 1 000 000 | 0.036 / 100 000 | United Kingdom | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.135 / 100 000 | Croatia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.213 / 100 000 | Iceland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.171 / 100 000 | Poland | 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.
6
HPO phenotypes
22 annotations · validated 2016-06-03| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0011763 | Pituitary carcinoma | Pathognomonic sign |
| Very frequent (99-80%) | ||
| HP:0002315 | Headache | |
| HP:0003154 | Increased circulating ACTH level | |
| HP:0000870 | Increased circulating prolactin concentration | |
| HP:0100836 | Malignant neoplasm of the central nervous system | |
| HP:0008291 | Pituitary corticotropic cell adenoma | |
| HP:0006767 | Pituitary prolactin cell adenoma | |
| Frequent (79-30%) | ||
| HP:0012505 | Enlarged pituitary gland | |
| HP:0012377 | Hemianopia | |
| HP:0040075 | Hypopituitarism | |
| HP:0007987 | Progressive visual field defects | |
| HP:0007663 | Reduced visual acuity | |
| Occasional (29-5%) | ||
| HP:0011442 | Abnormality of central motor function | |
| HP:0001251 | Ataxia | |
| HP:0000845 | Elevated circulating growth hormone concentration | |
| HP:0000365 | Hearing impairment | |
| HP:0010514 | Hyperpituitarism | |
| HP:0011760 | Pituitary growth hormone cell adenoma | |
| HP:0100561 | Spinal cord lesion | |
| Very rare (<4-1%) | ||
| HP:0000873 | Diabetes insipidus | |
| HP:0011759 | Pituitary gonadotropic cell adenoma | |
| HP:0011762 | Pituitary thyrotropic cell adenoma | |
Run the differential on the 7 obligate / very frequent terms
Annotation source: 15741248[PMID]_23400299[PMID]_17287410[PMID]_27242211[PMID]_22404748[PMID]_20660056[PMID]_25633744[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/300385
- This record as JSON: /data/d/300385.json