loading...| Preferred Name |
Desmoplastic Small Round Cell Tumor |
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| Synonyms |
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| Definitions |
An aggressive malignant soft tissue neoplasm of uncertain differentiation. It is characterized by a recurrent chromosomal translocation t(11;22)(p13;q12) and the presence of small round cells in a desmoplastic stroma. It usually affects children and young adults. The most common site of involvement is the abdomen. Patients usually present with abdominal distention, pain, ascites, and a palpable abdominal mass. The prognosis is usually poor. |
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| ID |
http://purl.obolibrary.org/obo/NCIT_C8300 |
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| ALT_DEFINITION |
A rare, aggressive cancer that usually affects young males and usually is located in the abdomen. |
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| code |
C8300 |
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| Contributing_Source |
CTEP CTRP GDC |
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| definition |
An aggressive malignant soft tissue neoplasm of uncertain differentiation. It is characterized by a recurrent chromosomal translocation t(11;22)(p13;q12) and the presence of small round cells in a desmoplastic stroma. It usually affects children and young adults. The most common site of involvement is the abdomen. Patients usually present with abdominal distention, pain, ascites, and a palpable abdominal mass. The prognosis is usually poor. |
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| Display_Name |
Desmoplastic Small Round Cell Tumor |
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| ICD-O-3_Code |
8806/3 |
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| in_subset |
http://purl.obolibrary.org/obo/NCIT_C168658 http://purl.obolibrary.org/obo/NCIT_C168656 http://purl.obolibrary.org/obo/NCIT_C168657 http://purl.obolibrary.org/obo/NCIT_C168655 http://purl.obolibrary.org/obo/NCIT_C102905 http://purl.obolibrary.org/obo/NCIT_C168662 http://purl.obolibrary.org/obo/NCIT_C168661 http://purl.obolibrary.org/obo/NCIT_C177537 http://purl.obolibrary.org/obo/ncit#oncotree_slim http://purl.obolibrary.org/obo/NCIT_C103117 http://purl.obolibrary.org/obo/NCIT_C116977 |
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| Is_Value_For_GDC_Property | ||
| label |
Desmoplastic Small Round Cell Tumor |
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| Legacy Concept Name |
Desmoplastic_Small_Round_Cell_Tumor |
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| Maps_To |
Desmoplastic small round cell tumor 8806/3 |
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| Neoplastic_Status |
Malignant |
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| Preferred_Name |
Desmoplastic Small Round Cell Tumor |
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| prefixIRI |
NCIT:C8300 |
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| prefLabel |
Desmoplastic Small Round Cell Tumor |
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| Semantic_Type |
Neoplastic Process |
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| UMLS_CUI |
C0281508 |
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| subClassOf |
http://purl.obolibrary.org/obo/NCIT_C3746 |