Dasgupta Roshni, Rodeberg David A
Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Semin Pediatr Surg. 2012 Feb;21(1):68-78. doi: 10.1053/j.sempedsurg.2011.10.007.
Rhabdomyosarcoma (RMS) is a malignant childhood tumor of mesenchymal origin that currently has a greater than 70% overall 5-year survival. Multimodality treatment is determined by risk stratification according to pretreatment stage, postoperative group, histology, and site of the primary tumor. Pretreatment staging is dependent on primary tumor site, size, regional lymph node status, and presence of metastases. Unique to RMS is the concept of postoperative clinical grouping that assesses the completeness of disease resection and takes into account lymph node evaluation. At all tumor sites, the clinical grouping, and therefore completeness of resection, is an independent predictor of outcome. Overall, the prognosis for RMS is dependent on primary tumor site, patient age, completeness of resection, extent of disease, including the presence and number of metastatic sites and histology and biology of the tumor cells. Therefore, the surgeon plays a vital role in RMS by contributing to risk stratification for treatment, local control of the primary tumor, and outcome. The current state-of-the-art treatment is determined by treatment protocols developed by the Soft Tissue Sarcoma Committee of the children's Oncology Group.
横纹肌肉瘤（RMS）是一种间叶组织来源的儿童恶性肿瘤，目前其总体5年生存率超过70%。多模式治疗根据预处理阶段、术后分组、组织学类型以及原发肿瘤部位进行风险分层来确定。预处理分期取决于原发肿瘤部位、大小、区域淋巴结状态以及是否存在转移。RMS特有的概念是术后临床分组，该分组评估疾病切除的完整性并考虑淋巴结评估。在所有肿瘤部位，临床分组以及切除的完整性都是预后的独立预测因素。总体而言，RMS的预后取决于原发肿瘤部位、患者年龄、切除的完整性、疾病范围，包括转移部位的存在和数量以及肿瘤细胞的组织学和生物学特性。因此，外科医生在RMS治疗中发挥着至关重要的作用，通过参与治疗风险分层、原发肿瘤的局部控制以及影响治疗结果。目前的先进治疗方法由儿童肿瘤学组软组织肉瘤委员会制定的治疗方案决定。
