About: Desmoplastic Small Round Cell Tumor

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Attributes	Values
rdf:type	Class
rdfs:label	Desmoplastic Small Round Cell Tumor
equivalentClass	nodeID://b631959
Concept_In_Subset	NCI CTEP Simplified Disease Classification Terminology NCI CTEP SDC Non-Rhabdomyosarcoma Soft Tissue Sarcoma Sub-Category Terminology
Semantic_Type	Neoplastic Process
Preferred_Name	Desmoplastic Small Round Cell Tumor
UMLS_CUI	C0281508
Contributing_Source	CTEP
ALT_DEFINITION	A tumor composed of small round tumor cells of uncertain histogenesis, associated with prominent stromal desmoplasia and polyphenotypic differentiation. There is a recurrent cytogenetic aberration: t(11;22)(p13;q12).NCI A rare, aggressive cancer that usually affects young males and usually is located in the abdomen.NCI-GLOSS
ICD-O-3_Code	8806/3
Neoplastic_Status	Malignant
Legacy_Concept_Name	Desmoplastic_Small_Round_Cell_Tumor
FULL_SYN	Desmoplastic small round cell tumorPTCTEP10064587 Desmoplastic Small Round Cell TumorPTNCI Desmoplastic Small Round-Cell TumorSYNCI DSRCTABNCI Desmoplastic Small Round-Cell NeoplasmSYNCI desmoplastic small round cell tumorPTNCI-GLOSSCDR0000044495 Desmoplas. small round cell tumorSYCTEP10064587
DEFINITION	An aggressive malignant neoplasm of uncertain lineage. It is characterized by 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.NCI
code	C8300
is rdf:first of	nodeID://b730117 nodeID://b730118
is someValuesFrom of	nodeID://b669699 nodeID://b669704
is sameAs of	Desmoplastic Small Round Cell Tumor

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