TLE1 Positive Clear Cell Sarcoma of the Kidney: A Case Report and Review of the Literature

Clear cell sarcoma of the kidney (CCSK) is an uncommon malignant tumor of uncertain histogenesis that occurs most commonly in childhood. Histologically, CCSK can mimic myxoid variant of synovial sarcoma (SS); however, the double positivity for CD99 and TLE1 in SS helps in excluding CCSK. Herein, we...

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Main Author: Rana Naous
Format: Article
Language:English
Published: Wiley 2018-01-01
Series:Case Reports in Pathology
Online Access:http://dx.doi.org/10.1155/2018/3462096
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author Rana Naous
author_facet Rana Naous
author_sort Rana Naous
collection DOAJ
description Clear cell sarcoma of the kidney (CCSK) is an uncommon malignant tumor of uncertain histogenesis that occurs most commonly in childhood. Histologically, CCSK can mimic myxoid variant of synovial sarcoma (SS); however, the double positivity for CD99 and TLE1 in SS helps in excluding CCSK. Herein, we report a rare case of CCSK arising in the left kidney of a 3-year-old girl. The mass grossly measured 9.5 cm in maximum dimension and histologically showed the classic arborizing fibrovascular septae and background myxoid matrix that is usually seen in CCSK. The tumor however was double positive for CD99 and TLE1 which made it difficult to discriminate it from the myxoid variant of SS based on histopathological examination and immunophenotype alone, and genetic analysis for SYT gene rearrangement was required to reach a definitive diagnosis. Although there have been previous case reports of CCSK with positive expression of CD99 and negative TLE1, to our knowledge, this is the first case of CCSK expressing both CD99 and TLE1.
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spelling doaj-art-c071c3a94caa4469b4d9fa5aec63b7ce2025-02-03T01:10:42ZengWileyCase Reports in Pathology2090-67812090-679X2018-01-01201810.1155/2018/34620963462096TLE1 Positive Clear Cell Sarcoma of the Kidney: A Case Report and Review of the LiteratureRana Naous0Department of Pathology, SUNY Upstate Medical University, 750 East Adams Street, Syracuse, NY 13210, USAClear cell sarcoma of the kidney (CCSK) is an uncommon malignant tumor of uncertain histogenesis that occurs most commonly in childhood. Histologically, CCSK can mimic myxoid variant of synovial sarcoma (SS); however, the double positivity for CD99 and TLE1 in SS helps in excluding CCSK. Herein, we report a rare case of CCSK arising in the left kidney of a 3-year-old girl. The mass grossly measured 9.5 cm in maximum dimension and histologically showed the classic arborizing fibrovascular septae and background myxoid matrix that is usually seen in CCSK. The tumor however was double positive for CD99 and TLE1 which made it difficult to discriminate it from the myxoid variant of SS based on histopathological examination and immunophenotype alone, and genetic analysis for SYT gene rearrangement was required to reach a definitive diagnosis. Although there have been previous case reports of CCSK with positive expression of CD99 and negative TLE1, to our knowledge, this is the first case of CCSK expressing both CD99 and TLE1.http://dx.doi.org/10.1155/2018/3462096
spellingShingle Rana Naous
TLE1 Positive Clear Cell Sarcoma of the Kidney: A Case Report and Review of the Literature
Case Reports in Pathology
title TLE1 Positive Clear Cell Sarcoma of the Kidney: A Case Report and Review of the Literature
title_full TLE1 Positive Clear Cell Sarcoma of the Kidney: A Case Report and Review of the Literature
title_fullStr TLE1 Positive Clear Cell Sarcoma of the Kidney: A Case Report and Review of the Literature
title_full_unstemmed TLE1 Positive Clear Cell Sarcoma of the Kidney: A Case Report and Review of the Literature
title_short TLE1 Positive Clear Cell Sarcoma of the Kidney: A Case Report and Review of the Literature
title_sort tle1 positive clear cell sarcoma of the kidney a case report and review of the literature
url http://dx.doi.org/10.1155/2018/3462096
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