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Primary bone sarcoma with BCOR internal tandem duplication.

原发性骨肉瘤伴BCOR内部串联重复。

  • 影响因子:2.52
  • DOI:10.1007/s00428-019-02729-z
  • 作者列表:"Malik F","Zreik RT","Hedges DJ","Nakitandwe J","Lee S","Ward RA","McCarville MB","Pappo A","Bahrami A
  • 发表时间:2020-06-01
Abstract

:BCOR internal tandem duplications (ITDs) and rearrangements are implicated in the oncogenesis of a subset of undifferentiated sarcomas. To date, BCOR ITD sarcomas have been exclusively found in non-appendicular infantile soft tissues, whereas BCOR-rearranged sarcomas occur in both bones and soft tissues affecting a wider patient age range. Little is known about patient outcome in BCOR ITD sarcomas. We present a BCOR-expressing, primary bone, undifferentiated sarcoma case involving an adolescent male's left tibia that, unexpectedly, harbored a BCOR ITD instead of a BCOR rearrangement. Furthermore, the patient achieved a partial histologic response after receiving a Ewing sarcoma chemotherapy regimen. Our case expands the clinical spectrum of BCOR ITD sarcomas and suggests that childhood and adult BCOR-expressing sarcomas with an undifferentiated histology should be considered for both BCOR rearrangement and ITD screening. Accurate BCOR mutation identification in undifferentiated sarcomas is essential to define their clinical spectrum and to develop effective management strategies.

摘要

: BCOR内部串联重复 (itd) 和重排与未分化肉瘤子集的肿瘤发生有关。迄今为止,BCOR ITD肉瘤仅见于非阑尾婴儿软组织,而BCOR重排肉瘤发生于骨骼和软组织,影响更大的患者年龄范围。对BCOR ITD肉瘤的患者结局知之甚少。我们介绍了一例BCOR表达的原发性骨未分化肉瘤病例,涉及一名青少年男性的左胫骨,意外地携带BCOR ITD而不是BCOR重排。此外,患者在接受尤文肉瘤化疗方案后获得了部分组织学缓解。我们的病例扩大了BCOR ITD肉瘤的临床谱,并提示在BCOR重排和ITD筛查中应考虑儿童期和成人BCOR表达的未分化组织学肉瘤。未分化肉瘤中准确的BCOR突变鉴定对于确定其临床谱并制定有效的管理策略至关重要。

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发表时间:2020-03-01
DOI:10.1259/bjr.20180883
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发表时间:2020-03-01
DOI:10.1177/1078155219842277
作者列表:["Gyori DJ","Bullington SM","Crawford BS","Vernon VP"]

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骨肿瘤方向

骨肿瘤是发生于骨骼或其附属组织的肿瘤。有良性,恶性之分,良性骨肿瘤易根治,预后良好,恶性骨肿瘤发展迅速,预后不佳,死亡率高。

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