Probing for a deeper understanding of rhabdomyosarcoma: insights from complementary model systems

被引：97

作者：

Kashi, Venkatesh P. ^{[1
]}

Hatley, Mark E. ^{[2
]}

Galindo, Rene L. ^{[1
,3
,4
]}

机构：

[1] Univ Texas SW Med Ctr Dallas, Dept Pathol, Dallas, TX 75390 USA

[2] St Jude Childrens Res Hosp, Dept Oncol, Memphis, TN 38105 USA

[3] Univ Texas SW Med Ctr Dallas, Dept Biol Mol, Dallas, TX 75390 USA

[4] Univ Texas SW Med Ctr Dallas, Dept Pediat, Dallas, TX 75390 USA

来源：

NATURE REVIEWS CANCER | 2015年 / 15卷 / 07期

关键词：

CHILDRENS ONCOLOGY GROUP; NEGATIVE ALVEOLAR RHABDOMYOSARCOMA; EMBRYONAL RHABDOMYOSARCOMA; MYOBLAST FUSION; MOUSE MODEL; HEDGEHOG PATHWAY; PEDIATRIC RHABDOMYOSARCOMA; COSTELLO-SYNDROME; SKELETAL-MUSCLE; GENE FUSIONS;

D O I：

10.1038/nrc3961

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Rhabdomyosarcoma (RMS) is a mesenchymal malignancy composed of neoplastic primitive precursor cells that exhibit histological features of myogenic differentiation. Despite intensive conventional multimodal therapy, patients with high-risk RMS typically suffer from aggressive disease. The lack of directed therapies against RMS emphasizes the need to further uncover the molecular underpinnings of the disease. In this Review, we discuss the notable advances in the model systems now available to probe for new RMS-targetable pathogenetic mechanisms, and the possibilities for enhanced RMS therapeutics and improved clinical outcomes.

引用

页码：426 / 439

页数：14

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