Clear Cell Sarcoma of the Kidney

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 o...

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Published inArchives of pathology & laboratory medicine (1976) Vol. 144; no. 1; pp. 119 - 123
Main Authors Aldera, Alessandro Pietro, Pillay, Komala
Format Journal Article
LanguageEnglish
Published United States College of American Pathologists 01.01.2020
Subjects
Biomarkers, Tumor - analysis
Biomarkers, Tumor - genetics
Cancer
Carboplatin
Chemotherapy
Clear cell-type renal cell carcinoma
Cysts
Diagnostic errors
Diseases
Endometrium
Fusion protein
Genes
Genetic screening
Genetic testing
Hematuria
Hepatocytes
Humans
Immunohistochemistry
Kidney cancer
Kidney Neoplasms - diagnosis
Kidney Neoplasms - genetics
Kidney Neoplasms - pathology
Kidneys
Lymph nodes
Metastases
Metastasis
Morphology
Nucleotide sequence
Pediatrics
Recurrence (Disease)
Sarcoma
Sarcoma, Clear Cell - diagnosis
Sarcoma, Clear Cell - genetics
Sarcoma, Clear Cell - pathology
Tumors
Vincristine
South Africa
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Summary: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 ( ) and a translocation t(10;17) creating the fusion gene 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.
Bibliography:ObjectType-Article-2
SourceType-Scholarly Journals-1
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ISSN:0003-9985
1543-2165
1543-2165
DOI:10.5858/arpa.2018-0353-rs