|
gptkbp:instanceOf
|
gptkb:disease
gptkb:rhabdomyosarcoma
gptkb:pediatric_cancer
|
|
gptkbp:affectsOrgan
|
gptkb:kidney
|
|
gptkbp:associatedSyndrome
|
gptkb:aniridia
gptkb:hemihypertrophy
genitourinary anomalies
|
|
gptkbp:canBeBilateral
|
yes
|
|
gptkbp:canBeSporadic
|
yes
|
|
gptkbp:commonIn
|
children under 5 years
|
|
gptkbp:commonMetastasisSite
|
lungs
liver
lymph nodes
|
|
gptkbp:diagnosedBy
|
gptkb:CT_scan
MRI
ultrasound
biopsy
|
|
gptkbp:firstDescribed
|
gptkb:Max_Wilms
1899
|
|
gptkbp:fiveYearSurvivalRate
|
over 90% (localized disease)
|
|
gptkbp:gender
|
slightly more common in females
|
|
gptkbp:hasOrphanetID
|
gptkb:ORPHA:324
|
|
gptkbp:hereditary
|
yes
|
|
gptkbp:histology
|
triphasic (blastemal, stromal, epithelial)
|
|
gptkbp:ICD-10_code
|
gptkb:C64
|
|
gptkbp:MeSH_ID
|
D009396
|
|
gptkbp:mostCommonAgeRange
|
3 to 4 years
|
|
gptkbp:mutationAssociatedWith
|
gptkb:WT1_gene
gptkb:WT2_gene
|
|
gptkbp:OMIM
|
194070
|
|
gptkbp:prevalence
|
about 1 in 10,000 children
|
|
gptkbp:prognosis
|
generally good with treatment
|
|
gptkbp:rareAfterAge
|
8 years
|
|
gptkbp:rareIn
|
adults
|
|
gptkbp:riskFactor
|
gptkb:Beckwith-Wiedemann_syndrome
gptkb:Denys-Drash_syndrome
gptkb:WAGR_syndrome
family history
|
|
gptkbp:symptom
|
fever
abdominal pain
hypertension
hematuria
abdominal mass
|
|
gptkbp:treatment
|
gptkb:radiation_therapy
surgery
chemotherapy
|
|
gptkbp:typicalAgeOfOnset
|
childhood
|
|
gptkbp:bfsParent
|
gptkb:Therapeutically_Applicable_Research_to_Generate_Effective_Treatments
gptkb:11p15.5
gptkb:Beckwith-Wiedemann_syndrome
gptkb:IGF2
|
|
gptkbp:bfsLayer
|
6
|
|
https://www.w3.org/2000/01/rdf-schema#label
|
Wilms tumor
|