← Назад

Meningioma

ORPHA:2495DiseaseNot applicableAll ages

Ассоциированные гены (12)

SMARCB1
SWI/SNF related BAF chromatin remodeling complex subunit B1
Disease-causing germline mutation(s) in
OMIM: 601607
PDGFB
platelet derived growth factor subunit B
Disease-causing germline mutation(s) in
OMIM: 190040
SUFU
SUFU negative regulator of hedgehog signaling
Disease-causing germline mutation(s) in
OMIM: 607035
NF2
NF2, moesin-ezrin-radixin like (MERLIN) tumor suppressor
Disease-causing somatic mutation(s) in
OMIM: 607379
PIK3CA
phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha
Disease-causing somatic mutation(s) in
OMIM: 171834
AKT1
AKT serine/threonine kinase 1
Disease-causing germline mutation(s) in
OMIM: 164730
TERT
telomerase reverse transcriptase
Disease-causing somatic mutation(s) in
OMIM: 187270
BAP1
BRCA1 associated deubiquitinase 1
Disease-causing germline mutation(s) in
OMIM: 603089
BAP1
BRCA1 associated deubiquitinase 1
Disease-causing somatic mutation(s) in
OMIM: 603089
SMARCE1
SWI/SNF related BAF chromatin remodeling complex subunit E1
Disease-causing germline mutation(s) (loss of function) in
OMIM: 603111
TRAF7
TNF receptor associated factor 7
Disease-causing somatic mutation(s) in
OMIM: 606692
SMO
smoothened, frizzled class receptor
Disease-causing somatic mutation(s) in
OMIM: 601500

Фенотипы (74)

Очень частый (80–99%)3
HP:0010997Chromosomal breakage induced by ionizing radiation
HP:0011133Increased sensitivity to ionizing radiation
HP:0100009Intracranial meningioma
Частый (30–79%)19
HP:0000044Hypogonadotropic hypogonadism
HP:0000141Amenorrhea
HP:0000802Impotence
HP:0000870Increased circulating prolactin concentration
HP:0001250Seizure
HP:0002017Nausea and vomiting
HP:0002315Headache
HP:0002920Decreased circulating ACTH level
HP:0007359Focal-onset seizure
HP:0008163Decreased circulating cortisol level
HP:0008214Decreased serum estradiol
HP:0008240Secondary growth hormone deficiency
HP:0008245Pituitary hypothyroidism
HP:0012658Abnormal brain FDG positron emission tomography
HP:0012691Focal T2 hypointense thalamic lesion
HP:0030341Decreased circulating follicle stimulating hormone concentration
HP:0030344Decreased circulating luteinizing hormone level
HP:0030521Bitemporal hemianopia
HP:0040171Decreased serum testosterone concentration
Периодический (5–29%)32
HP:0000238Hydrocephalus
HP:0000602Ophthalmoplegia
HP:0001067Neurofibromas
HP:0001085Papilledema
HP:0001251Ataxia
HP:0001269Hemiparesis
HP:0001317Abnormal cerebellum morphology
HP:0001513Obesity
HP:0002354Memory impairment
HP:0002516Increased intracranial pressure
HP:0003484Upper limb muscle weakness
HP:0004302Functional motor deficit
HP:0004408Abnormality of the sense of smell
HP:0006824Cranial nerve paralysis
HP:0007340Lower limb muscle weakness
HP:0007715Weak extraocular muscles
HP:0007924Slow decrease in visual acuity
HP:0008202Reduced circulating prolactin concentration
HP:0008237Hypothalamic hypothyroidism
HP:0010628Facial palsy
HP:0011442Abnormality of central motor function
HP:0011730Abnormality of central sensory function
HP:0011750Neoplasm of the anterior pituitary
HP:0012246Oculomotor nerve palsy
HP:0012285Abnormal hypothalamus physiology
HP:0012505Enlarged pituitary gland
HP:0030532Visual acuity test abnormality
HP:0030591Abnormal kinetic perimetry test
HP:0100010Spinal meningioma
HP:0100543Cognitive impairment
HP:0100661Trigeminal neuralgia
HP:0001288Gait disturbance
Очень редкий (1–4%)20
HP:0000020Urinary incontinence
HP:0000360Tinnitus
HP:0000520Proptosis
HP:0000618Blindness
HP:0000712Emotional lability
HP:0001262Excessive daytime somnolence
HP:0001279Syncope
HP:0001342Cerebral hemorrhage
HP:0002167Abnormality of speech or vocalization
HP:0002512Brain stem compression
HP:0003418Back pain
HP:0006520Progressive pulmonary function impairment
HP:0008069Neoplasm of the skin
HP:0010534Transient global amnesia
HP:0010828Hemifacial spasm
HP:0011752Neoplasm of the posterior pituitary
HP:0030766Ear pain
HP:0030878Abnormality on pulmonary function testing
HP:0045026Abnormality of the mediastinum
HP:0100648Neoplasm of the tongue

Эпидемиология (3)

Annual incidence
1-9 / 100 000
Germany
Point prevalence
Unknown
Europe
Annual incidence
1-9 / 100 000
United States

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы