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Clear Cell Sarcoma of the Kidney
Alessandro Pietro Aldera1, Komala Pillay1
1From the Division of Anatomical Pathology, University of Cape Town, Cape Town, and the National Health Laboratory Service, Western Cape, South Africa (Drs Aldera and Pillay); the Department of Anatomical Pathology, Groote Schuur Hospital, Western Cape, South Africa (Dr Aldera); and the Department of Anatomical Pathology, Red Cross War Memorial Children's Hospital, Cape Town, South Africa (Dr Pillay).
Archives of Pathology & Laboratory Medicine
|March 28, 2019
Summary
Clear cell sarcoma of the kidney is a rare pediatric cancer that can be misdiagnosed. New genetic markers and improved chemotherapy have led to better outcomes and fewer late relapses.
Area of Science:
- Pediatric Oncology
- Renal Neoplasms
- Molecular Pathology
Background:
- Clear cell sarcoma of the kidney (CCSK) is a rare pediatric renal neoplasm.
- CCSK exhibits diverse histological patterns, often leading to misdiagnosis.
- Diagnostic adjuncts like immunohistochemistry and molecular genetics were previously limited.
Purpose of the Study:
- To highlight recent advancements in diagnosing and managing CCSK.
- To emphasize the role of new molecular and immunohistochemical markers.
- To discuss the impact of improved chemotherapy on clinical outcomes.
Main Methods:
- Review of histological and molecular findings in CCSK.
- Evaluation of immunohistochemistry for BCOR as a diagnostic marker.
- Analysis of clinical data regarding treatment and relapse patterns.
Main Results:
- Internal tandem duplications in BCOR and the YWHAE-NUTM2B/E fusion gene are key molecular findings.
- BCOR immunohistochemistry is a sensitive and specific marker for CCSK.
- Intensive chemotherapy has reduced late relapses, with brain becoming the most common site.
Conclusions:
- Accurate diagnosis of CCSK is improved by molecular and immunohistochemical markers.
- Modern chemotherapy regimens enhance patient outcomes and alter relapse patterns.
- Continued research into pediatric renal tumors is crucial for advancing care.
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