Open AccessPrimary Renal Sarcoma With BCOR-CCNB3 Gene Fusion in an 18-Year-Old Male: A Rare Lesion With a Diagnostic Quandary
Author(s) -
Sasan Setoodeh,
Doreen N. Palsgrove,
Jason Park,
Iván Pedrosa,
Payal Kapur,
Liwei Jia
Publication year - 2020
Publication title -
international journal of surgical pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.356
H-Index - 51
eISSN - 1940-2465
pISSN - 1066-8969
DOI - 10.1177/1066896920941087
Subject(s) - medicine , synovial sarcoma , pathology , sarcoma , fusion gene , immunohistochemistry , lesion , inferior vena cava , kidney , thrombus , radiology , gene , biology , biochemistry
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.