BCORinternal tandem duplications in clear cell sarcoma of the kidney

2016 ◽  
Vol 55 (6) ◽  
pp. 549-550 ◽  
Author(s):  
Saskia L. Gooskens ◽  
Samantha Gadd ◽  
Marry M. van den Heuvel-Eibrink ◽  
Elizabeth J. Perlman
Keyword(s):  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma ◽  
Tandem Duplications

scholarly journals Clear Cell Sarcoma of the Kidney

2019 ◽  
Vol 144 (1) ◽  
pp. 119-123 ◽  
Author(s):  
Alessandro Pietro Aldera ◽  
Komala Pillay
Keyword(s):  
Clear Cell ◽  
Fusion Gene ◽  
Molecular Genetic ◽  
Clear Cell Sarcoma ◽  
Renal Tumors ◽  
Specific Marker ◽  
Renal Neoplasm ◽  
Cell Sarcoma ◽  
The Brain ◽  
Tandem Duplications

Clear cell sarcoma of the kidney is an uncommon malignant pediatric renal neoplasm that typically presents in the 2- to 3-year age group and has a propensity for aggressive behavior and late relapses. Histologically, this tumor exhibits a great diversity of morphologic patterns that can mimic most other pediatric renal neoplasms, often leading to confusion and misdiagnosis. Until recently, adjunct immunohistochemical and molecular genetic tests to support the diagnosis were lacking. The presence of internal tandem duplications in BCL-6 coreceptor (BCOR) and a translocation t(10;17) creating the fusion gene YWHAE-NUTM2B/E have now been well accepted. Immunohistochemistry for BCOR has also been shown to be a sensitive and specific marker for clear cell sarcoma of the kidney in the context of pediatric renal tumors. Improved intensive chemotherapy regimens have influenced the clinical course of the disease, with late relapses now being less frequent and the brain having overtaken bone as the most common site of relapse.

scholarly journals Clear Cell Sarcoma of the Kidney

2019 ◽  
Vol 143 (8) ◽  
pp. 1022-1026 ◽  
Author(s):  
Sze Jet Aw ◽  
Kenneth Tou En Chang
Keyword(s):  
Nerve Growth Factor ◽  
Clear Cell ◽  
Growth Factor Receptor ◽  
Clear Cell Sarcoma ◽  
Accurate Diagnosis ◽  
Egfr Mutations ◽  
Genetic Mutations ◽  
Cell Sarcoma ◽  
Molecular Studies ◽  
Tandem Duplications

Clear cell sarcoma of the kidney is the second most common primary renal malignancy in childhood. It is histologically diverse, making accurate diagnosis challenging in some cases. Recent molecular studies have uncovered BCOR exon 15 internal tandem duplications in most cases, and YWHAE-NUTM2 fusion in a few cases, with the remaining cases having other genetic mutations, including BCOR-CCNB3 fusion and EGFR mutations. Although clear cell sarcoma of the kidney has no specific immunophenotype, several markers including cyclin D1, nerve growth factor receptor, and BCOR (BCL6 corepressor) have emerged as potential diagnostic aides. This review provides a concise account of recent advances in our understanding of clear cell sarcoma of the kidney to serve as a practical update for the practicing pathologist.

scholarly journals Recurrent internal tandem duplications of BCOR in clear cell sarcoma of the kidney

2015 ◽  
Vol 6 (1) ◽  
Author(s):  
Angshumoy Roy ◽  
Vijetha Kumar ◽  
Barry Zorman ◽  
Erica Fang ◽  
Katherine M. Haines ◽  
...  
Keyword(s):  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma ◽  
Tandem Duplications

Mutually exclusiveBCORinternal tandem duplications andYWHAE-NUTM2fusions in clear cell sarcoma of kidney: not the full story

2016 ◽  
Vol 238 (5) ◽  
pp. 617-620 ◽  
Author(s):  
Colin Kenny ◽  
Sabrina Bausenwein ◽  
Antonio Lazaro ◽  
Rhoikos Furtwängler ◽  
Saskia LM Gooskens ◽  
...  
Keyword(s):  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma ◽  
Tandem Duplications

Consistent in-frame internal tandem duplications of BCOR characterize clear cell sarcoma of the kidney

2015 ◽  
Vol 47 (8) ◽  
pp. 861-863 ◽  
Author(s):  
Hitomi Ueno-Yokohata ◽  
Hajime Okita ◽  
Keiko Nakasato ◽  
Shingo Akimoto ◽  
Jun-ichi Hata ◽  
...  
Keyword(s):  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma ◽  
Tandem Duplications

An immunohistochemical study comparing clear cell sarcoma of the kidney and wilms' tumor

Pathology ◽  
1993 ◽  
Vol 25 (2) ◽  
pp. 106-109 ◽  
Author(s):  
Lai-Meng Lool ◽  
Phaik-Leng Cheah
Keyword(s):  
Clear Cell ◽  
Immunohistochemical Study ◽  
Wilms Tumor ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma
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