ORPHA:91349
Non-functioning pituitary adenoma
Synonyms: NFPA
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood
- Status
- active
A rare pituitary tumor originating from normally hormone-producing cells of the adenohypophysis, characterized by a sellar or extrasellar mass manifesting with clinical signs secondary to mass effect, but without evidence for hormonal hypersecretion. Typical manifestations are visual disturbances, headaches, cranial nerve dysfunction, and hypopituitarism but the mass may also be discovered incidentally.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Non-functioning pituitary adenoma | NFPA |
| fr | Adénome hypophysaire non fonctionnel | = en |
| de | Hypophysenadenom, nicht-funktionelles | = en |
| es | Adenoma hipofisario no secretor | = en |
| it | Adenoma ipofisario non funzionante | = en |
| nl | Niet-functioneel hypofyseadenoom | = en |
| pt | Adenoma hipofisário não-secretor | = en |
| pl | Nieczynny gruczolak przysadki | = en |
| cs | Hormonálně neaktivní adenom hypofýzy | = en |
| uk | Нефункціонуюча аденома гіпофіза | = en |
| zh | 非功能性垂体腺瘤 | = en |
| tr | İşlevsiz hipofiz adenomu | = en |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D35.2 | NTBT | attributed | validated |
| ICD-11 | 2F37.0 | E | specific | validated |
| MONDO | 0019613 | E | validated | |
| UMLS | C0338078 | E | validated | |
| MedDRA | 10029556 | 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:99408Pituitary adenoma
rare endocrine diseases; rare neoplastic diseases
4
Genes
0 direct · 4 via subtypesOrphanet records no gene directly on this code.
Recorded by Orphanet on subtypes of this disorder:
| Gene | Subtype | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|---|
| AIP | ORPHA:314786Silent pituitary adenoma | Major susceptibility factor in | Assessed | HGNC:358 | ENSG00000110711 | 605555 | 11q13.2 | AHR interacting HSP90 co-chaperone | 23321498[PMID] |
| AIP | ORPHA:314790Null pituitary adenoma | Major susceptibility factor in | Assessed | HGNC:358 | ENSG00000110711 | 605555 | 11q13.2 | AHR interacting HSP90 co-chaperone | 23321498[PMID] |
| MEN1 | ORPHA:314786Silent pituitary adenoma | Major susceptibility factor in | Assessed | HGNC:7010 | ENSG00000133895 | 613733 | 11q13.1 | menin 1 | 23321498[PMID] |
| MEN1 | ORPHA:314790Null pituitary adenoma | Major susceptibility factor in | Assessed | HGNC:7010 | ENSG00000133895 | 613733 | 11q13.1 | menin 1 | 23321498[PMID] |
5
Prevalence and incidence
9 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | — | Europe | Class only | Validated | ORPHANET_26072284[PMID] |
| Annual incidence | 1-9 / 100 000 | 4 / 100 000 | Finland | Value and class | Validated | 20534753[PMID] |
| Annual incidence | 1-9 / 100 000 | 1.1 / 100 000 | Sweden | Value and class | Validated | 10770176[PMID] |
| Point prevalence | 6-9 / 10 000 | 94 / 100 000 | Belgium | Value and class | Validated | 16968795[PMID] |
| Point prevalence | 6-9 / 10 000 | 80.5 / 100 000 | Switzerland | Value and class | Validated | 19968031[PMID] |
| Point prevalence | 6-9 / 10 000 | 77.6 / 100 000 | United Kingdom | Value and class | Validated | 19650784[PMID] |
| Point prevalence | 6-9 / 10 000 | 68 / 100 000 | Finland | Value and class | Validated | 20534753[PMID] |
| Annual incidence | 1-9 / 100 000 | 2.55 / 100 000 | Europe | Value and class | Not yet validated | ORPHANET_25946030[PMID]_20534753[PMID]_10770176[PMID] |
| Annual incidence | 1-9 / 100 000 | 1.05 / 100 000 | Worldwide | Value and class | Validated | 25946030[PMID]_20534753[PMID]_10770176[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.
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HPO phenotypes
58 annotations · validated 2024-03-13| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002893 | Pituitary adenoma | |
| Frequent (79-30%) | ||
| HP:0011362 | Abnormal hair quantity | |
| HP:0011804 | Abnormal muscle physiology | |
| HP:0000140 | Abnormality of the menstrual cycle | |
| HP:0012503 | Abnormality of the pituitary gland | |
| HP:0000846 | Adrenal insufficiency | |
| HP:0011748 | Adrenocorticotropic hormone deficiency | |
| HP:0011735 | Adrenocorticotropin deficient adrenal insufficiency | |
| HP:0010972 | Anemia of inadequate production | |
| HP:0000830 | Anterior hypopituitarism | |
| HP:0030521 | Bitemporal hemianopia | |
| HP:0011734 | Central adrenal insufficiency | |
| HP:0002920 | Decreased circulating ACTH level | |
| HP:0030018 | Decreased female libido | |
| HP:0000868 | Decreased fertility in females | |
| HP:0012041 | Decreased fertility in males | |
| HP:0000824 | Decreased response to growth hormone stimulation test | |
| HP:0003388 | Easy fatigability | |
| HP:0100639 | Erectile dysfunction | |
| HP:0012378 | Fatigue | |
| HP:0000134 | Female hypogonadism | |
| HP:0002315 | Headache | |
| HP:0000135 | Hypogonadism | |
| HP:0000044 | Hypogonadotropic hypogonadism | |
| HP:0040075 | Hypopituitarism | |
| HP:0002615 | Hypotension | |
| HP:0000802 | Impotence | |
| HP:0000837 | Increased circulating gonadotropin level | |
| HP:0008993 | Increased intraabdominal fat | |
| HP:0000858 | Irregular menstruation | |
| HP:0000026 | Male hypogonadism | |
| HP:0002017 | Nausea and vomiting | |
| HP:0000980 | Pallor | |
| HP:0008245 | Pituitary hypothyroidism | |
| HP:0000529 | Progressive visual loss | |
| HP:0008240 | Secondary growth hormone deficiency | |
| HP:0002013 | Vomiting | |
| Occasional (29-5%) | ||
| HP:0006897 | Abducens palsy | |
| HP:0000618 | Blindness | |
| HP:0000863 | Central diabetes insipidus | |
| HP:0030998 | Cerebrospinal fluid rhinorrhoea | |
| HP:0006824 | Cranial nerve paralysis | |
| HP:0000873 | Diabetes insipidus | |
| HP:0000651 | Diplopia | |
| HP:0007011 | Fourth cranial nerve palsy | |
| HP:0012377 | Hemianopia | |
| HP:0030517 | Heteronymous hemianopia | |
| HP:0000870 | Increased circulating prolactin concentration | |
| HP:0030088 | Increased serum testosterone level | |
| HP:0007942 | Internal ophthalmoplegia | |
| HP:0000053 | Macroorchidism | |
| HP:0002050 | Macroorchidism, postpubertal | |
| HP:0012246 | Oculomotor nerve palsy | |
| HP:0000871 | Panhypopituitarism | |
| HP:0000508 | Ptosis | |
| HP:0001250 | Seizure | |
| HP:0001117 | Sudden loss of visual acuity | |
| HP:0002321 | Vertigo | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 30521182[PMID]_8496297[PMID]_26072284[PMID]_26070464[PMID]_25208537[PMID]_21474687[PMID]_19945027[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/91349
- This record as JSON: /data/d/91349.json