Mesenchymal Chondrosarcoma: a Review with Emphasis on its Fusion-Driven Biology

被引：30
|
作者：
El Beaino, Marc [1 ]
Roszik, Jason [2 ,3 ]
Livingston, John A.
Wang, Wei-Lien [4 ]
Lazar, Alexander J. [4 ]
Amini, Behrang [5 ]
Subbiah, Vivek [6 ]
Lewis, Valerae [1 ]
Conley, Anthony P. [3 ]
机构：
[1] Univ Texas MD Anderson Canc Ctr, Dept Orthopaed Oncol, Houston, TX 77030 USA
[2] Univ Texas MD Anderson Canc Ctr, Dept Melanoma Med Oncol, Houston, TX 77030 USA
[3] Univ Texas MD Anderson Canc Ctr, Dept Sarcoma Med Oncol, Houston, TX 77030 USA
[4] Univ Texas MD Anderson Canc Ctr, Dept Pathol, Houston, TX 77030 USA
[5] Univ Texas MD Anderson Canc Ctr, Dept Diagnost Radiol, Houston, TX 77030 USA
[6] Univ Texas MD Anderson Canc Ctr, Dept Invest Therapeut, Houston, TX 77030 USA
来源：
CURRENT ONCOLOGY REPORTS | 2018年 / 20卷 / 05期
关键词：
Mesenchymal chondrosarcoma; Chondrosarcoma; Fusion; Translocation; Notch; HEY1; NCOA2; IRF2BP2; CDX1; Chromatin remodeling; TGF beta; Apoptosis; Genomics; Pathways; HEY1-NCOA2; FUSION; TRANSCRIPTION FACTORS; SIGNALING PATHWAYS; UBIQUITIN LIGASE; PROGENITOR CELLS; GENE FUSIONS; RECURRENT; NOTCH; DIFFERENTIATION; EXPRESSION;
D O I：
10.1007/s11912-018-0668-z
中图分类号：
R73 [肿瘤学];
学科分类号：
100214 ;
摘要：
Mesenchymal chondrosarcoma is a rare but deadly form of chondrosarcoma that typically affects adolescents and young adults. While curative intent is possible for patients with localized disease, few options exist for patients in the unresectable/metastatic setting. Thus, it is imperative to understand the fusion-driven biology of this rare malignant neoplasm so as to lead to the future development of better therapeutics for this disease. This manuscript will briefly review the clinical and pathologic features of mesenchymal chondrosarcoma followed by an appraisal of existing data linked to the fusions, HEY1-NCOA2 and IRF2BP2CDX1, and the associated downstream pathways.
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页数：11
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