Pituicytoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare glial tumor originating from pituicytes, the specialized glial cells of the neurohypophysis, characterized by a sellar or suprasellar mass manifesting with clinical signs secondary to mass effect. Typical manifestations are visual disturbances, headaches, and hypopituitarism. Pituicytomas are low-grade tumors, and prognosis is good after total resection.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Pituicytoma | |
| fr | Pituicytome | |
| de | Pituizytom | |
| es | Pituicitoma | |
| it | Pituicitoma | |
| nl | Pituïcytoom | |
| pt | Pituicitoma | |
| pl | Pituicytoma | |
| cs | Pituicytom | |
| uk | Пітуїцитома | |
| zh | 垂体细胞瘤 | |
| tr | Pituisitom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D44.3 | NTBT | attributed | validated |
| ICD-11 | 2A00.0Y | NTBT | index | validated |
| MONDO | 0006372 | E | validated | |
| UMLS | C2986550 | E | validated | |
| MedDRA | 10089140 | 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:251592Low-grade astrocytoma
rare neurological diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 171 cases | Worldwide | Case(s) | Validated | 33971477[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 33971477[PMID]_ORPHANET |
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
20 annotations · validated 2016-06-27| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0011752 | Neoplasm of the posterior pituitary | |
| HP:0011754 | Pituicytoma | |
| Very frequent (99-80%) | ||
| HP:0012503 | Abnormality of the pituitary gland | |
| Frequent (79-30%) | ||
| HP:0002315 | Headache | |
| HP:0001123 | Visual field defect | |
| Occasional (29-5%) | ||
| HP:0011043 | Abnormality of circulating adrenocorticotropin level | |
| HP:0000141 | Amenorrhea | |
| HP:0030018 | Decreased female libido | |
| HP:0000824 | Decreased response to growth hormone stimulation test | |
| HP:0008214 | Decreased serum estradiol | |
| HP:0040171 | Decreased serum testosterone concentration | |
| HP:0012378 | Fatigue | |
| HP:0000044 | Hypogonadotropic hypogonadism | |
| HP:0040075 | Hypopituitarism | |
| HP:0000802 | Impotence | |
| HP:0000870 | Increased circulating prolactin concentration | |
| HP:0002354 | Memory impairment | |
| HP:0008245 | Pituitary hypothyroidism | |
| Very rare (<4-1%) | ||
| HP:0000863 | Central diabetes insipidus | |
| HP:0100829 | Galactorrhea | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 21960498[PMID]_25947224[PMID]_23887161[PMID]_23701182[PMID]_23237862[PMID]_20586639[PMID]_19525896[PMID]_10716149[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/251623
- This record as JSON: /data/d/251623.json