TY - JOUR
T1 - Primary Renal Sarcoma With BCOR-CCNB3 Gene Fusion in an 18-Year-Old Male
T2 - A Rare Lesion With a Diagnostic Quandary
AU - Setoodeh, Sasan
AU - Palsgrove, Doreen N.
AU - Park, Jason Y.
AU - Pedrosa, Ivan
AU - Kapur, Payal
AU - Jia, Liwei
N1 - Publisher Copyright:
© The Author(s) 2020.
PY - 2021/4
Y1 - 2021/4
N2 - Primary renal sarcoma with BCOR-CCNB3 gene fusion is a rare tumor with only 7 cases reported in the English literature. The morphologic features of this tumor strikingly overlap with clear cell sarcoma of the kidney and synovial sarcoma. Accurate diagnosis can be challenging. In this article, we report a case of an 18-year-old male who presented with hematuria. Subsequent imaging study showed a left renal mass with level II (infra-hepatic) inferior vena cava thrombus, which was resected. Detailed pathologic findings and immunohistochemical and molecular studies revealed an ovoid to spindle cell renal mass with a BCOR-CCNB3 gene fusion.
AB - Primary renal sarcoma with BCOR-CCNB3 gene fusion is a rare tumor with only 7 cases reported in the English literature. The morphologic features of this tumor strikingly overlap with clear cell sarcoma of the kidney and synovial sarcoma. Accurate diagnosis can be challenging. In this article, we report a case of an 18-year-old male who presented with hematuria. Subsequent imaging study showed a left renal mass with level II (infra-hepatic) inferior vena cava thrombus, which was resected. Detailed pathologic findings and immunohistochemical and molecular studies revealed an ovoid to spindle cell renal mass with a BCOR-CCNB3 gene fusion.
KW - BCOR-CCNB3 gene fusion
KW - primary renal sarcoma
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U2 - 10.1177/1066896920941087
DO - 10.1177/1066896920941087
M3 - Article
C2 - 32648496
AN - SCOPUS:85087764961
SN - 1066-8969
VL - 29
SP - 194
EP - 197
JO - International Journal of Surgical Pathology
JF - International Journal of Surgical Pathology
IS - 2
ER -