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Gastrointestinal stromal tumor

ORPHA:44890DiseaseAutosomal dominant, Not applicableAdolescent, Adult, Childhood

Ассоциированные гены (7)

PDGFRA
platelet derived growth factor receptor alpha
Disease-causing germline mutation(s) in
OMIM: 173490
SDHA
succinate dehydrogenase complex flavoprotein subunit A
Disease-causing germline mutation(s) (loss of function) in
OMIM: 600857
SDHB
succinate dehydrogenase complex iron sulfur subunit B
Candidate gene tested in
OMIM: 185470
SDHC
succinate dehydrogenase complex subunit C
Candidate gene tested in
OMIM: 602413
KIT
KIT proto-oncogene, receptor tyrosine kinase
Disease-causing somatic mutation(s) in
OMIM: 164920
KIT
KIT proto-oncogene, receptor tyrosine kinase
Disease-causing germline mutation(s) (gain of function) in
OMIM: 164920
PDGFRA
platelet derived growth factor receptor alpha
Disease-causing somatic mutation(s) in
OMIM: 173490

Фенотипы (18)

Очень частый (80–99%)3
HP:0006753Neoplasm of the stomach
HP:0100242Sarcoma
HP:0100723Gastrointestinal stroma tumor
Частый (30–79%)6
HP:0002015Dysphagia
HP:0002017Nausea and vomiting
HP:0002019Constipation
HP:0002239Gastrointestinal hemorrhage
HP:0005214Intestinal obstruction
HP:0012378Fatigue
Периодический (5–29%)9
HP:0000988Skin rash
HP:0001392Abnormality of the liver
HP:0001903Anemia
HP:0007378Neoplasm of the gastrointestinal tract
HP:0007400Irregular hyperpigmentation
HP:0100273Neoplasm of the colon
HP:0100743Neoplasm of the rectum
HP:0100751Esophageal neoplasm
HP:0100833Neoplasm of the small intestine

Эпидемиология (8)

Point prevalence
1-5 / 10 000
Europe
Annual incidence
1-9 / 100 000
Europe
Annual incidence
1-9 / 100 000
Worldwide
Annual incidence
1-9 / 100 000
Korea, Republic of
Point prevalence
1-5 / 10 000
Hong Kong
Annual incidence
1-9 / 100 000
Hong Kong
Annual incidence
1-9 / 100 000
United States
Point prevalence
1-5 / 10 000
Sweden

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы