Functioning gonadotropic adenoma
Synonyms: Functioning pituitary gonadotropic adenoma · Gonadotroph adenoma
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
Functioning gonadotropic adenoma is a very rare pituitary tumor, macroscopically characterized by a soft, well vascularized, variable sized adenoma, with occasional areas of hemorrage or necrosis, that secretes biologically active gonadotropins. In addition to common neurological signs due to mass effect (headache and/or visual field deterioration), additional clinical manifestations include menstrual irregularities (secondary amenorrhea, oligomenorhea or severe menorrhagia), galactorrhea, infertility or ovarian hyperstimulation syndrome (in premenopausal women), testicular enlargement and, occasionally, hypogonadism (in men) and isosexual precocious puberty (in children).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Functioning gonadotropic adenoma | Functioning pituitary gonadotropic adenoma · Gonadotroph adenoma |
| fr | Adénome gonadotrope fonctionnel | Adénome gonadotrope hypophysaire fonctionnel |
| de | Adenom, funktionelles gonadotropes | Adenom, hypophysäres, funktionelles gonadotropes · Gonadotropes Adenom · Hypophysenadenom, gonadotropes |
| es | Adenoma gonadotrópico funcionante | Adenoma gonadotropo · Adenoma hipofisario gonadotrópico funcionante · Adenoma hipofisario gonadotrópico secretor |
| it | Adenoma gonadotropo funzionante | Adenoma gonadotropo ipofisario funzionante |
| nl | Functioneel gonadotroop adenoom | Functioneel hypofysair gonadotroop adenoom · Gonadotroop adenoom |
| pt | Adenoma gonadotrópico funcionante | Adenoma gonadotrópico · Adenoma gonadotrópico hipofisário funcionante |
| pl | Czynny gruczolak gonadotropowy | Czynny gonadotropowy gruczolak przysadki |
| cs | Hormonálně aktivní/funkční gonadotropní adenom | Hormonálně aktivní/funkční gonadotropní adenom hypofýzy |
| uk | Функціонуюча гонадотропна аденома | Функціонуюча гонадотропна аденома гіпофіза · Гонадотрофна аденома |
| zh | 功能性促性腺腺瘤 | 功能性垂体促性腺腺瘤 |
| tr | İşlevsel gonadotropik adenom | Gonadotrop adenom · İşleyen hipofiz gonadotropik adenomu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D35.2 | NTBT | attributed | validated |
| ICD-11 | 2F37.Y | NTBT | attributed | validated |
| MONDO | 0019612 | E | validated | |
| UMLS | C0346304 | 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 (2):
- ORPHA:314753Functioning pituitary adenoma
rare endocrine diseases; rare neoplastic diseases - ORPHA:715120Hyperpituitarism
rare endocrine diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
34 annotations · validated 2016-07-25| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0011759 | Pituitary gonadotropic cell adenoma | |
| Frequent (79-30%) | ||
| HP:0040086 | Abnormal prolactin level | |
| HP:0000140 | Abnormality of the menstrual cycle | |
| HP:0000830 | Anterior hypopituitarism | |
| HP:0030018 | Decreased female libido | |
| HP:0012378 | Fatigue | |
| HP:0000802 | Impotence | |
| HP:0030088 | Increased serum testosterone level | |
| HP:0000789 | Infertility | |
| HP:0001123 | Visual field defect | |
| HP:0000505 | Visual impairment | |
| Occasional (29-5%) | ||
| HP:0009888 | Abnormality of secondary sexual hair | |
| HP:0011748 | Adrenocorticotropic hormone deficiency | |
| HP:0000141 | Amenorrhea | |
| HP:0001541 | Ascites | |
| HP:0000863 | Central diabetes insipidus | |
| HP:0000824 | Decreased response to growth hormone stimulation test | |
| HP:0002625 | Deep venous thrombosis | |
| HP:0000823 | Delayed puberty | |
| HP:0002750 | Delayed skeletal maturation | |
| HP:0008675 | Enlarged polycystic ovaries | |
| HP:0100829 | Galactorrhea | |
| HP:0002315 | Headache | |
| HP:0000238 | Hydrocephalus | |
| HP:0000837 | Increased circulating gonadotropin level | |
| HP:0008236 | Isosexual precocious puberty | |
| HP:0002050 | Macroorchidism, postpubertal | |
| HP:0012246 | Oculomotor nerve palsy | |
| HP:0000798 | Oligozoospermia | |
| HP:0000938 | Osteopenia | |
| HP:0000939 | Osteoporosis | |
| HP:0000138 | Ovarian cyst | |
| HP:0000871 | Panhypopituitarism | |
| HP:0008245 | Pituitary hypothyroidism | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 8656706[PMID]_25166722[PMID]_26072284[PMID]_9768644[PMID]_767353[PMID]_11502789[PMID]_2991325[PMID]_402377[PMID]_334787[PMID]_21196695[PMID]_20960265[PMID]_2627747[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/91348
- This record as JSON: /data/d/91348.json