/clear cell sarcoma of kidney

clear cell sarcoma of kidney

Rare Disease

MONDO:0005006

Also Known As

CCSKchildhood clear cell sarcoma of the kidneychildhood kidney clear cell sarcomachildhood renal clear cell sarcomaclear cell sarcoma - kidneyclear cell sarcoma of kidneyclear cell sarcoma of the kidneykidney clear cell sarcomapaediatric kidney clear cell sarcomapaediatric renal clear cell sarcomapediatric kidney clear cell sarcomapediatric renal clear cell sarcomarenal clear cell sarcoma

Definition

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.