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Pediatric Disease Annotations & Medicines



   desmoplastic small round cell tumor
  

Disease ID 346
Disease desmoplastic small round cell tumor
Definition
A rare, aggressive soft tissue sarcoma that primarily affects adolescents and young adults. It is most commonly found in the abdomen.
Synonym
desmoplas. small round cell tumor
desmoplastic small cell tumor
desmoplastic small round cell tumor (morphologic abnormality)
desmoplastic small round cell tumor [disease/finding]
desmoplastic small round cell tumour
desmoplastic small round-cell neoplasm
desmoplastic small round-cell tumor
desmoplastic small-cell tumor
desmoplastic small-cell tumors
dsrct
polyphenotypic small round cell tumor
small-cell tumor, desmoplastic
small-cell tumors, desmoplastic
tumor, desmoplastic small-cell
tumors, desmoplastic small-cell
Orphanet
DOID
UMLS
C0281508
MeSH
Comorbidity
UMLS | Disease | Sentences' Count(Total Sentences:8)
C1261473  |  sarcoma  |  2
C0019829  |  hodgkin lymphoma  |  1
C0024305  |  non-hodgkin lymphoma  |  1
C0020538  |  hypertension  |  1
C0155616  |  secondary hypertension  |  1
C0024299  |  lymphoma  |  1
C0034072  |  cor pulmonale  |  1
C0700251  |  brachial plexopathy  |  1
Curated Gene
Entrez_id | Symbol | Resource(Total Genes:2)
EWSR1  |  2130  |  ORPHANET
WT1  |  7490  |  ORPHANET
Inferring Gene(Waiting for update.)
Text Mined Gene(Waiting for update.)
Locus
Symbol | Locus(Total Locus:2)
EWSR1  |  22q12.2
WT1  |  11p13
Disease ID 346
Disease desmoplastic small round cell tumor
Integrated Phenotype
HPO | Name(Total Integrated Phenotypes:18)
HP:0002240  |  Hepatomegaly
HP:0001903  |  Anemia
HP:0003270  |  Abdominal distention
HP:0004326  |  Cachexia
HP:0100242  |  Sarcoma
HP:0100721  |  Mediastinal lymphadenopathy
HP:0002585  |  Abnormality of the peritoneum
HP:0001824  |  Weight loss
HP:0002595  |  Ileus
HP:0002894  |  Neoplasm of the pancreas
HP:0002017  |  Nausea and vomiting
HP:0001541  |  Ascites
HP:0100526  |  Neoplasm of the lung
HP:0002027  |  Abdominal pain
HP:0010788  |  Testicular neoplasm
HP:0100006  |  Neoplasm of the central nervous system
HP:0100615  |  Ovarian neoplasm
HP:0002716  |  Lymphadenopathy
Text Mined Phenotype
HPO | Name | Sentences' Count(Total Phenotypes:7)
HP:0100242  |  Sarcoma  |  2
HP:0012539  |  Non-Hodgkin lymphoma  |  1
HP:0012189  |  Hodgkin disease  |  1
HP:0002665  |  Lymphoma  |  1
HP:0000822  |  Hypertension  |  1
HP:0002664  |  Neoplasia  |  1
HP:0001648  |  Cor pulmonale  |  1
Disease ID 346
Disease desmoplastic small round cell tumor
Manually Symptom
UMLS  | Name(Total Manually Symptoms:1)
C0494165  |  hepatic metastasis
Text Mined Symptom(Waiting for update.)
Manually Genotype(Total Text Mining Genotypes:0)
(Waiting for update.)
All Snps(Total Genotypes:0)
(Waiting for update.)
GWASdb Annotation(Total Genotypes:0)
(Waiting for update.)
GWASdb Snp Trait(Total Genotypes:0)
(Waiting for update.)
Mapped by lexical matching(Total Items:1)
HP ID HP Name MP ID MP Name Annotation
HP:0002585Abnormality of the peritoneumMP:0000662abnormal branching of the mammary ductal tree;HP:0000035Abnormality of the testis
Mapped by homologous gene(Total Items:1)
HP ID HP Name MP ID MP Name Annotation
HP:0100615Ovarian neoplasmMP:0000260abnormal angiogenesis;HP:0001541Ascites
Chemical(Total Drugs:3)
CUI ChemicalName ChemicalID CasRN DiseaseName DiseaseID DirectEvidence PubMedIDs
C0281508cyclophosphamideD00352050-18-0desmoplastic small round cell tumorMESH:D058405therapeutic10555010
C0281508ifosfamideD0070693778-73-2desmoplastic small round cell tumorMESH:D058405therapeutic10555010
C0281508vincristineD014750-desmoplastic small round cell tumorMESH:D058405therapeutic10555010
FDA approved drug and dosage information(Total Drugs:0)
(Waiting for update.)
FDA labeling changes(Total Drugs:0)
(Waiting for update.)