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Adrenocortical carcinoma

ORPHA:1501DiseaseNot applicableChildhood

Ассоциированные гены (7)

TP53
tumor protein p53
Disease-causing germline mutation(s) in
OMIM: 191170
TP53
tumor protein p53
Disease-causing somatic mutation(s) in
OMIM: 191170
ZNRF3
zinc and ring finger 3
Disease-causing somatic mutation(s) in
OMIM: 612062
CTNNB1
catenin beta 1
Disease-causing somatic mutation(s) in
OMIM: 116806
CDKN2A
cyclin dependent kinase inhibitor 2A
Disease-causing somatic mutation(s) in
OMIM: 600160
TERT
telomerase reverse transcriptase
Disease-causing somatic mutation(s) in
OMIM: 187270
PRKAR1A
protein kinase cAMP-dependent type I regulatory subunit alpha
Disease-causing somatic mutation(s) in
OMIM: 188830

Фенотипы (29)

Облигатный (100%)1
HP:0006744Adrenocortical carcinoma
Частый (30–79%)27
HP:0000080Abnormality of reproductive system physiology
HP:0000737Irritability
HP:0000739Anxiety
HP:0000819Diabetes mellitus
HP:0000822Hypertension
HP:0000859Hyperaldosteronism
HP:0000975Hyperhidrosis
HP:0000998Hypertrichosis
HP:0001065Striae distensae
HP:0001324Muscle weakness
HP:0001824Weight loss
HP:0001939Abnormality of metabolism/homeostasis
HP:0001962Palpitations
HP:0002027Abdominal pain
HP:0002900Hypokalemia
HP:0003118Increased circulating cortisol level
HP:0003466Paradoxical increased cortisol secretion on dexamethasone suppression test
HP:0004324Increased body weight
HP:0011748Adrenocorticotropic hormone deficiency
HP:0012030Increased urinary cortisol level
HP:0025134Increased serum estradiol
HP:0025269Panic attack
HP:0025380Increased circulating androstenedione concentration
HP:0025436Elevated serum 11-deoxycortisol
HP:0030078Lung adenocarcinoma
HP:0030348Increased circulating androgen concentration
HP:0500022Abnormal serum dehydroepiandrosterone level
Периодический (5–29%)1
HP:0003110Abnormality of urine homeostasis

Эпидемиология (25)

Point prevalence
1-9 / 1 000 000
Europe
Annual incidence
<1 / 1 000 000
Europe
Annual incidence
<1 / 1 000 000
Portugal
Annual incidence
1-9 / 1 000 000
Austria
Annual incidence
1-9 / 1 000 000
Belgium
Annual incidence
1-9 / 1 000 000
Bulgaria
Annual incidence
1-9 / 1 000 000
Croatia
Annual incidence
1-9 / 1 000 000
Czech Republic
Annual incidence
1-9 / 1 000 000
Estonia
Annual incidence
1-9 / 1 000 000
Finland
Annual incidence
1-9 / 1 000 000
Germany
Annual incidence
1-9 / 1 000 000
Iceland
Annual incidence
1-9 / 1 000 000
Ireland
Annual incidence
1-9 / 1 000 000
Italy
Annual incidence
1-9 / 1 000 000
Latvia
Annual incidence
1-9 / 1 000 000
Lithuania
Annual incidence
1-9 / 1 000 000
Malta
Annual incidence
1-9 / 1 000 000
Norway
Annual incidence
1-9 / 1 000 000
Poland
Annual incidence
1-9 / 1 000 000
Slovakia
Annual incidence
1-9 / 1 000 000
Slovenia
Annual incidence
1-9 / 1 000 000
Spain
Annual incidence
1-9 / 1 000 000
Switzerland
Annual incidence
1-9 / 1 000 000
Netherlands
Annual incidence
1-9 / 1 000 000
United Kingdom

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы