Key result
Case report details a rare primary renal BCOR-CCNB3 fusion sarcoma in a 16-year-old boy.
Why the study?
Primary renal BCOR-CCNB3 fusion sarcoma is very rare, accurate diagnosis is challenging, and there is no agreement on treatment due to its rarity.
Case Report (n=1)
Reports a rare case of primary renal BCOR-CCNB3 fusion sarcoma in a 16-year-old boy, adding to the limited literature on this entity.
May warrant molecular testing in atypical pediatric renal masses; leaves open management standards.
BCOR-CCNB3 fusion sarcoma is a recently described undifferentiated sarcoma with a novel recurrent inversion of 2 nearby genes BCOR and CCNB3. It typically affects bone and soft tissues of the pelvis, extremity, and paraspinal region and pursues variable clinical course. Primary renal BCOR-CCNB3 fusion sarcoma is very rare, and only a small number of cases have been documented. Accurate diagnosis is often challenging, and there is not any agreement for the treatment of this entity due to its rarity. We report findings of primary renal BCOR-CCNB3 fusion sarcoma in a 16-year-old boy with a brief review of the literature.
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Zhao et al. (2021) conducted a case report in Primary renal BCOR-CCNB3 fusion sarcoma (n=1). A case of primary renal BCOR-CCNB3 fusion sarcoma, a rare undifferentiated sarcoma, was reported in a 16-year-old boy.
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