CNS tumor with BCOR internal tandem duplication: Clinicopathologic, molecular characteristics and prognosis factors

免疫组织化学 病理 基因复制 串联外显子复制 化疗 奥利格2 中枢神经系统 医学 生物 基因 内科学 生物化学 髓鞘 少突胶质细胞
作者
Ruifen Wang,Wenbin Guan,Meng Qiao,Ying Zhang,Meng Zhang,Kezhou Wang,Yin Wang,Lifeng Wang
出处
期刊:Pathology Research and Practice [Elsevier BV]
卷期号:236: 153995-153995 被引量:9
标识
DOI:10.1016/j.prp.2022.153995
摘要

The central nervous system tumor with BCOR internal tandem duplication (CNS tumor with BCOR ITD) is a recently identified rare tumor entity, the complete morphologic characteristic, genetic alteration, classification, clinical outcomes and optimal treatment for this tumor entity have not been fully clarified. Here, two new cases of CNS tumor with BCOR ITD were reported and the clinicopathologic, molecular characteristics, and prognosis were analyzed through reviewing of the reported literature. The histological features included a clear border with adjacent brain parenchyma, an extensively microcystic background, and the cells with round to oval nuclei containing delicate chromatin, ependymoma-like perivascular pseudorosettes, and palisading necrosis. Immunohistochemical features showed strong and diffuse positive expression for BCOR, scattered positive expression for OLIG2, and negative expression for GFAP, Syn, and EMA. PCR and direct DNA sequencing analysis identified exon 15 ITD of the BCOR gene in both cases. Interestingly, both cases revealed two duplication segments, which had not been reported in the literature. A review of the literature shows that CNS tumor with BCOR ITD has a poor prognosis with a median survival of 1.7 years; surgical gross total resection is a good prognostic factor. A combination of radiation treatment and chemotherapy, while trending towards significance, does not reach statistical significance. On the contrary, the OS is not associated with age, gender, and tumor location. In conclusion, CNS tumor with BCOR ITD is a rare tumor entity with characteristic morphologic and molecular features and a poor prognosis. The optimal treatment strategies need further studies.
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