DiseaseID 2199

横纹肌样瘤

disease

Atypical teratoid rhabdoid tumor (ATRT) is a highly malignant central nervous system (CNS) rhabdoid tumor (RT; see this term) found almost exclusively in children.

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Disease: 1Symptom: 12Target: 12Links: 24
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Record Fields

Scalar fields from the final disease record.

Disease Id
2199
Core Entity Id
2475
Source Entity Count
1
Preferred Name
Rhabdoid Tumor
Name Cn
横纹肌样瘤
Name Pinyin
Heng Wen Ji Yang Liu
Name En
Rhabdoid Tumor
Name Latin
Bilingual Status
complete
Disease Type
disease
Umls Disease Type
Disease or Syndrome
Disgenet Type
disease
Mesh Class
NeoplasmsNervous System Diseases; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases; Neoplasms
Do Class
disease of anatomical entity; disease of cellular proliferation
Hpo Class
Mesh Class Name
Neoplasms
Hpo Class Name
Do Class Name
disease of anatomical entity; disease of cellular proliferation
Disease Definition
Atypical teratoid rhabdoid tumor (ATRT) is a highly malignant central nervous system (CNS) rhabdoid tumor (RT; see this term) found almost exclusively in children.
Version
v1
Suppressed
No

Names

Preferred names, aliases, and source labels retained in the final schema.

Name
Rhabdoid Tumor
Role
preferred
Name
Atypical Teratoid/Rhabdoid Tumor
Role
preferred
Name
Rhabdoid Tumor Predisposition Syndrome 1
Role
preferred
Name
Rhabdoid Tumor Predisposition Syndrome 1 (Disorder)
Role
preferred
Name
Atypical Teratoid Rhabdoid Tumor
Role
preferred
Name
Malignant Rhabdoid Tumor, Somatic
Role
preferred
Name
Teratoid Tumor, Atypical
Role
preferred
Name
AT/RT
Role
alias
Name
BRAIN TUMOR, POSTERIOR FOSSA, OF INFANCY, FAMILIAL
Role
alias
Name
RDT
Role
alias
Name
RTPS1
Role
alias
Name
Rhabdoid Cancer
Role
alias

Cross References

Trusted external identifiers retained for this final record.

Herb
HBDIS004466HBDIS013013HBDIS028786HBDIS028787HBDIS029185
Me Sh
D018335
Omim
609322
Umls
C0206743C1266184C1836326C1836327C2750405
Sym Map
SMDE00348SMDE03514SMDE06175SMDE12866SMDE12868
Do Class
DOID:14566DOID:7
Dis Ge Net
C0206743C1266184C1836326C1836327C2750405
Orphanet
99966
Umls Sty
T047T191
Me Sh Class
C04C10C12C13
Tcmbank Disease
2076129764
Itcmdb Generated
ITX-DISEASE-B20AE4B7665A

Attributes

Merged source attributes and domain-specific metadata.

Version
v1v2
Suppress
0
Do Class Name
disease of anatomical entity; disease of cellular proliferation
Disease Type
disease
Do Disease Class
disease of anatomical entity; disease of cellular proliferation
Umls Disease Type
Disease or SyndromeNeoplastic Process
Disease Definition
Atypical teratoid rhabdoid tumor (ATRT) is a highly malignant central nervous system (CNS) rhabdoid tumor (RT; see this term) found almost exclusively in children.NCI2016_NCI-GLOSS_1602D:A malignant tumor of either the central nervous system (CNS) or the kidney. Malignant rhabdoid tumors of the CNS often have an abnormality of chromosome 22. These tumors usually occur in children younger than 2 years.|NCI2016_02D:An aggressive malignant embryonal neoplasm usually occurring during childhood. It is characterized by the presence of large cells with abundant cytoplasm, large eccentric nucleus, and a prominent nucleolus and it is associated with abnormalities of chromosome 22. It can arise from the central nervous system, kidney, and the soft tissues. The prognosis is poor.|MSH2017_2016_08_12:A rare but highly lethal childhood tumor found almost exclusively in infants. Histopathologically, it resembles RHABDOMYOSARCOMA but the tumor cells are not of myogenic origin. Although it arises primarily in the kidney, it may be found in other parts of the body. The rhabdoid cytomorphology is believed to be the expression of a very primitive malignant cell. (From Holland et al., Cancer Medicine, 3d ed, p2210)NCI2016_NCI-GLOSS_1602D:An aggressive cancer of the central nervous system, kidney, or liver that occurs in very young children.|NCI2016_02D:An aggressive malignant embryonal neoplasm arising from the central nervous system. It is composed of cells with a large eccentric nucleus, prominent nucleolus, and abundant cytoplasm. It may be associated with loss of chromosome 22. The vast majority of cases occur in childhood. Symptoms include lethargy, vomiting, cranial nerve palsy, headache, and hemiplegia.
Me Sh Disease Class
NeoplasmsNervous System Diseases; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases; Neoplasms
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Neoplasms
Umls Semantic Type Name
Neoplastic Process