LaQuaglia M P
Department of Surgery (Pediatric Surgery), Memorial Sloan-Kettering Cancer Center, New York, New York 10021.
Semin Surg Oncol. 1993 Nov-Dec;9(6):510-9. doi: 10.1002/ssu.2980090609.
Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and adolescence with 20% of the primary tumors anatomically located on extremities. It is a complicated entity that requires careful planning and coordination between the surgical oncologist and other members of the therapeutic team for successful treatment. Even with the most effective regimens more than 50% of patients will die from progressive, usually distant disease. Further progress may require new therapeutic agents or techniques. The surgical oncologist is a necessary and often prominent member of the team. Elements of the biological behavior, histopathology, clinical staging, and treatment of extremity rhabdomyosarcomas occurring in children are discussed. In particular, the importance of the alveolar subtype in determination of prognostic risk as well as new findings regarding the molecular biologic determinants of phenotypic behavior are mentioned. Finally, innovative methods of local control like regional arterial perfusion and rapid intraoperative brachytherapy are addressed.
横纹肌肉瘤是儿童和青少年时期最常见的软组织肉瘤，20%的原发性肿瘤位于四肢。它是一个复杂的疾病实体，需要外科肿瘤学家与治疗团队的其他成员进行仔细规划和协调，才能成功治疗。即使采用最有效的治疗方案，仍有超过50%的患者会死于进展性疾病，通常是远处转移。进一步的进展可能需要新的治疗药物或技术。外科肿瘤学家是治疗团队中必不可少且通常很突出的成员。本文讨论了儿童四肢横纹肌肉瘤的生物学行为、组织病理学、临床分期及治疗等方面。特别提到了肺泡型亚型在判断预后风险中的重要性以及关于表型行为分子生物学决定因素的新发现。最后，还探讨了局部控制的创新方法，如区域动脉灌注和术中快速近距离放疗。
