|
gptkbp:instanceOf
|
gptkb:cancer
gptkb:disease
gptkb:rhabdomyosarcoma
malignant tumor
|
|
gptkbp:affects
|
gptkb:Humans
gptkb:bone
gptkb:Nerves
gptkb:Blood_vessels
Animals
trunk
fat
collagen
blood vessels
Muscle
genitourinary tract
head and neck
skeletal muscle tissue
nerves
tendons
extremities
soft tissues
muscles
Connective tissue
lining of joints
|
|
gptkbp:affectsOrgan
|
gptkb:kidney
|
|
gptkbp:alsoKnownAs
|
gptkb:Wilms_tumor
|
|
gptkbp:alternativeName
|
Sarcoma
chondrosarcoma
nephroblastoma
sarcoma
soft_tissue_sarcoma
|
|
gptkbp:canBeBenign
|
False
|
|
gptkbp:canBeMalignant
|
True
|
|
gptkbp:canMetastasizeTo
|
lungs
liver
lymph nodes
bones
bone marrow
other bones
|
|
gptkbp:canOccurAtAnyAge
|
true
|
|
gptkbp:commonIn
|
adults
children
children under 5
|
|
gptkbp:diagnosedBy
|
gptkb:X-rays
gptkb:CT_scan
imaging
MRI
ultrasound
biopsy
Biopsy
|
|
gptkbp:fieldOfStudy
|
Oncology
Pathology
|
|
gptkbp:firstDescribed
|
gptkb:Max_Wilms
1804
1854
1899
19th century
|
|
gptkbp:frequency
|
about 500 cases per year in the US
most common kidney cancer in children
|
|
gptkbp:has5YearSurvivalRate
|
about 50%
|
|
gptkbp:hasDiseaseOntologyID
|
DOID:3360
|
|
gptkbp:hasGeneticAlteration
|
gptkb:PAX3-FOXO1_fusion
gptkb:PAX7-FOXO1_fusion
|
|
gptkbp:hasMalignancyGrade
|
high grade
intermediate grade
low grade
|
|
gptkbp:hasOrphanetID
|
ORPHA:28447
|
|
gptkbp:hasRecurrenceRisk
|
high if not completely excised
|
|
gptkbp:hasTreatmentProtocol
|
gptkb:IVA_(ifosfamide,_vincristine,_actinomycin_D)
gptkb:VAC_(vincristine,_actinomycin_D,_cyclophosphamide)
|
|
gptkbp:histology
|
triphasic (blastemal, stromal, epithelial)
|
|
https://www.w3.org/2000/01/rdf-schema#label
|
rhabdomyosarcoma
|
|
gptkbp:ICD-10_code
|
gptkb:C49
gptkb:C64
C41
|
|
gptkbp:incidence
|
rare
1 in 200,000 per year
|
|
gptkbp:isMostCommonMalignantCartilageTumor
|
true
|
|
gptkbp:isNotSensitiveTo
|
radiation therapy
chemotherapy
|
|
gptkbp:marking
|
gptkb:MyoD1
gptkb:desmin
gptkb:myogenin
|
|
gptkbp:MeSH_ID
|
D012595
D012509
|
|
gptkbp:mortalityRate
|
varies by grade
|
|
gptkbp:mutationAssociatedWith
|
gptkb:WT1_gene
gptkb:WT2_gene
|
|
gptkbp:occurredIn
|
gptkb:meat
gptkb:humerus
adults
children
femur
pelvis
spine
|
|
gptkbp:originatedIn
|
mesenchymal cells
cartilage cells
|
|
gptkbp:peakIncidenceAge
|
2-6 years
15-19 years
|
|
gptkbp:prevalence
|
rare
|
|
gptkbp:prognosis
|
gptkb:theatre
place
variable
size
grade
metastasis
generally good with treatment
age at diagnosis
histologic subtype
tumor size
tumor grade
tumor location
|
|
gptkbp:rareIn
|
children
|
|
gptkbp:rarelyTreatedBy
|
chemotherapy
|
|
gptkbp:riskFactor
|
gptkb:Paget's_disease_of_bone
gptkb:Li-Fraumeni_syndrome
gptkb:Neurofibromatosis_type_1
gptkb:Beckwith-Wiedemann_syndrome
gptkb:Denys-Drash_syndrome
gptkb:WAGR_syndrome
gptkb:Maffucci_syndrome
gptkb:Ollier_disease
radiation exposure
chemical exposure
Genetic syndromes
Radiation exposure
genetic syndromes
previous radiation exposure
Chemical exposure
|
|
gptkbp:stageName
|
gptkb:TNM
gptkb:IRS_(Intergroup_Rhabdomyosarcoma_Study)
National Wilms Tumor Study Group (NWTSG)
|
|
gptkbp:subspecies
|
gptkb:alveolar_rhabdomyosarcoma
gptkb:pleomorphic_rhabdomyosarcoma
gptkb:spindle_cell/sclerosing_rhabdomyosarcoma
gptkb:Angiosarcoma
gptkb:Chondrosarcoma
gptkb:Osteosarcoma
gptkb:embryonal_rhabdomyosarcoma
gptkb:rhabdomyosarcoma
gptkb:Ewing_sarcoma
gptkb:angiosarcoma
gptkb:clear_cell_chondrosarcoma
gptkb:conventional_chondrosarcoma
gptkb:dedifferentiated_chondrosarcoma
gptkb:mesenchymal_chondrosarcoma
gptkb:Rhabdomyosarcoma
synovial sarcoma
liposarcoma
leiomyosarcoma
malignant peripheral nerve sheath tumor
Leiomyosarcoma
Liposarcoma
Synovial sarcoma
|
|
gptkbp:symptom
|
gptkb:Pain
gptkb:Lump
fever
marina
pain
abdominal pain
hypertension
hematuria
lump
swelling
Swelling
abdominal mass
Limited movement
|
|
gptkbp:synonym
|
cartilage cancer
|
|
gptkbp:treatment
|
surgery
radiation therapy
Surgery
chemotherapy
Chemotherapy
targeted therapy
Targeted therapy
|
|
gptkbp:typicalAgeOfOnset
|
childhood
|
|
gptkbp:bfsParent
|
gptkb:The_Cancer_Genome_Atlas
gptkb:cancer
|
|
gptkbp:bfsLayer
|
4
|