Hayes-Jordan Andrea, Andrassy Richard
University of Texas MD Anderson Cancer Center, University of Texas Houston Health Sciences Center, Houston, TX 77030, USA.
Curr Opin Pediatr. 2009 Jun;21(3):373-8. doi: 10.1097/MOP.0b013e32832b4171.
Rhabdomyosarcoma is a rare childhood cancer that affects only approximately 300 children per year in the United States. The purpose of this review is to provide the reader a greater understanding of the complex diagnosis, assessment and treatment of rhabdomyosarcoma in children.
This review focuses on the new risk classification that is the foundation of all present rhabdomyosarcoma protocols developed by the Children's Oncology Group of the United States and Canada. The new risk classification of low, intermediate and high encompasses the staging and grouping categories that were previously utilized.
This review also provides a complete list of diagnostic tests and imaging required to identify rhabdomyosarcoma in any body site. Rapid diagnosis and recognition of this rare disorder will facilitate long-term survival. Rhabdomyosarcoma today has an overall survival of 70%, depending on the site, and in orbital and other sites survival is as high as 90%. The treatment approaches that have led to this doubling in survival over the last 25 years are reviewed. For a practitioner, this review can be used as a reference when a child with a suspicious mass is encountered.
横纹肌肉瘤是一种罕见的儿童癌症,在美国每年仅影响约300名儿童。本综述的目的是让读者更深入了解儿童横纹肌肉瘤的复杂诊断、评估和治疗。
本综述重点关注新的风险分类,这是美国和加拿大儿童肿瘤学组制定的所有现有横纹肌肉瘤治疗方案的基础。新的低、中、高风险分类涵盖了以前使用的分期和分组类别。
本综述还提供了在任何身体部位识别横纹肌肉瘤所需的诊断测试和影像学检查的完整列表。对这种罕见疾病的快速诊断和识别将有助于长期生存。如今,横纹肌肉瘤的总体生存率为70%,具体取决于发病部位,在眼眶及其他部位生存率高达90%。本文回顾了在过去25年中使生存率翻倍的治疗方法。对于从业者来说,当遇到有可疑肿块的儿童时,本综述可作为参考。