Optimal Management of Ewing Sarcoma Family of Tumors: Recent Developments in Systemic Therapy

被引:8
作者
Owens, Cormac [1 ]
Abbott, Lesleigh S. [1 ]
Gupta, Abha A. [1 ]
机构
[1] Univ Toronto, Hosp Sick Children, Dept Pediat, Div Hematol Oncol, Toronto, ON M5G 1N6, Canada
关键词
PRIMITIVE NEUROECTODERMAL TUMOR; REFRACTORY SOLID TUMORS; GROWTH-FACTOR-RECEPTOR; PHASE-I TRIAL; TYROSINE KINASE INHIBITORS; FACTOR TYPE-1 RECEPTOR; IRINOTECAN PLUS TEMOZOLOMIDE; INITIAL TESTING STAGE-1; WHOLE-LUNG IRRADIATION; FRANCAISE-DES-CANCERS;
D O I
10.1007/s40272-013-0037-1
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
The Ewing sarcoma family of tumors (ESFT) is defined by cell surface expression of CD99 and a translocation involving EWS and an ETS partner. Cytotoxic chemotherapy remains the benchmark of first-and second-line therapy, and although the majority of patients with localized disease are cured, almost one third of patients relapse or progress from their disease. Moreover, cure remains elusive in most patients who present with distant metastases. In recent years, the ESFT literature has been dominated by reports of attempts at modulating the insulin-like growth factor (IGF) receptor (IGFR). Unfortunately, three phase II studies examining inhibiting antibodies to IGFR-1 published disappointing results. Whether these results were due to failure to modulate the pathway or other limitations in study design and/or patient selection remain unclear. Other novel strategies currently being investigated in ESFT include tyrosine kinase, mammalian target of rapamycin (mTOR), and poly(ADP-ribose) polymerase (PARP) inhibitors.
引用
收藏
页码:473 / 492
页数:20
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