Overview

NCI 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. [1]

Significant Genes in Clear Cell Sarcoma of the Kidney

MLH1 +

MLH3 +

MSH2 +

MSH3 +

MSH6 +

PMS1 +

PMS2 +

Disease Details

Synonyms
Clear Cell Sarcoma of Kidney, Pediatric Renal Clear Cell Sarcoma, Clear cell sarcoma - kidney, Childhood Clear Cell Sarcoma of the Kidney, Childhood Kidney Clear Cell Sarcoma, Childhood Renal Clear Cell Sarcoma, Kidney Clear Cell Sarcoma, Renal Clear Cell Sarcoma, Pediatric Kidney Clear Cell Sarcoma, CCSK
Parent(s)
Kidney Sarcoma
OncoTree Name
Clear Cell Sarcoma of Kidney
OncoTree Code
CCSK

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.