raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:95613

Pituitary apoplexy

Synonyms: Pituitary tumor apoplexy

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
All ages
Status
active

A rare pituitary disease characterized by hemorrhagic or non-hemorrhagic necrosis of the pituitary gland. Clinical manifestations typically comprise sudden and severe headache (often with nausea and vomiting), visual disturbances (visual-field defects, loss of visual acuity), oculomotor palsies, and variable degrees of altered consciousness, ranging from lethargy to coma. Acute endocrine dysfunction may also be present, most commonly corticotropic deficiency with severe hypotension and hyponatremia as well as secondary adrenal failure, but also thyrotropic and gonadotropic deficiency.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enPituitary apoplexyPituitary tumor apoplexy
frApoplexie hypophysaireApoplexie tumorale hypophysaire
deHypophyseninfarktHypophysentumor-Infarkt
esApoplejía hipofisariaApoplejía hipofisaria tumoral
itApoplessia ipofisaria
nlApoplexie van hypofyseHypofysetumor-apoplexie
ptApoplexia da pituitária
plApopleksja przysadki= en
csPituitární apoplexie= en
ukАпоплексія гіпофізаАпоплексія пухлини гіпофіза
zh垂体卒中垂体瘤卒中
trHipofiz apopleksiHipofiz tümörü apopleksi

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10E23.6NTBTindexvalidated
ICD-115A61.0NTBTindexvalidated
MONDO0006908Evalidated
MeSHD010899Evalidated
UMLSC0032001Evalidated
MedDRA10056447Evalidated

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):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

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

41 annotations · validated 2017-03-20
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0030521Bitemporal hemianopia
HP:0030907Thunderclap headache
Frequent (79-30%)
HP:0030591Abnormal kinetic perimetry test
HP:0030595Abnormal static automated perimetry test
HP:0011748Adrenocorticotropic hormone deficiency
HP:0006824Cranial nerve paralysis
HP:0000651Diplopia
HP:0002315Headache
HP:0000815Hypergonadotropic hypogonadism
HP:0000822Hypertension
HP:0001943Hypoglycemia
HP:0002902Hyponatremia
HP:0002615Hypotension
HP:0000802Impotence
HP:0002017Nausea and vomiting
HP:0000876Oligomenorrhea
HP:0002893Pituitary adenoma
HP:0008245Pituitary hypothyroidism
HP:0008202Reduced circulating prolactin concentration
Occasional (29-5%)
HP:0002921Abnormality of the cerebrospinal fluid
HP:0000622Blurred vision
HP:0001259Coma
HP:0000824Decreased response to growth hormone stimulation test
HP:0012378Fatigue
HP:0001945Fever
HP:0100829Galactorrhea
HP:0040075Hypopituitarism
HP:0000870Increased circulating prolactin concentration
HP:0011499Mydriasis
HP:0000980Pallor
HP:0000613Photophobia
HP:0000508Ptosis
HP:0007663Reduced visual acuity
Very rare (<4-1%)
HP:0002339Abnormal caudate nucleus morphology
HP:0000863Central diabetes insipidus
HP:0001289Confusion
HP:0000845Elevated circulating growth hormone concentration
HP:0001262Excessive daytime somnolence
HP:0003118Increased circulating cortisol level
HP:0001895Normochromic anemia
HP:0100661Trigeminal neuralgia

Run the differential on the 2 obligate / very frequent terms

Annotation source: 26414232[PMID]_21044119[PMID]_26812964[PMID]_25859804[PMID]_2436600[PMID]_22491696[PMID]_20842779[PMID]_15879908[PMID]_12546370[PMID]_11357158[PMID]_10684913[PMID]_9596029[PMID]_7608291[PMID]_2878231[PMID]

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Sources for this card