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clear cell sarcoma of kidney

Disease Summary
Associated Targets (6)
Tbio

3

Tdark

2

Tchem

1


Mondo Description A rare pediatric sarcoma affecting the kidney. It is characterized by the presence of epithelioid or spindle cells forming cords or nests, separated by fibrovascular septa. It is associated with internal tandem duplications in the BCOR gene. It metastasizes to lung, bone, brain and soft tissue.
Mondo Term and Equivalent IDs
MONDO:0005006:  clear cell sarcoma of kidney
EFO:0000350: 
NCIT:C4264: 
ONCOTREE:CCSK: 
Orphanet:457246: 
UMLS:C0334488: 
UMLS:CN242113: