Bielack Stefan S, Carrle Dorothe, Hardes Jendrik, Schuck Andreas, Paulussen Michael
Klinik für Kinder- und Jugendmedizin, Pädiatrie 5 (Onkologie, Hämatologie, Immunologie), Klinikum Stuttgart, Olgahospital, Bismarckstr. 8, 70176, Stuttgart, Germany.
Curr Treat Options Oncol. 2008 Feb;9(1):67-80. doi: 10.1007/s11864-008-0057-1. Epub 2008 May 1.
Bone tumors, particularly osteosarcomas and members of the Ewing Sarcoma Family of Tumors (ESFT), are typical malignancies of adolescents and young adults. Current diagnostic and therapeutic guidelines for patients of all ages were developed in this specific age group. The aim of bone sarcoma therapy should be to cure the patient from both the primary tumor and all (micro-)metastatic deposits while maintaining as much (extremity) function and causing as few treatment-specific late effects as possible. Bone sarcoma therapy requires close multidisciplinary cooperation. Usually, it consists of induction chemotherapy, followed by local therapy of the primary tumor (and, if present, primary metastases) and further, adjuvant chemotherapy. Local treatment for osteosarcoma should be surgery whenever feasible. Surgery is also gaining importance in ESFT, which was long considered a domain of radiotherapy. Modern reconstructive techniques continue to expand the indications for limb salvage, particularly for patients who have not yet reached skeletal maturity. Treatment within the framework of prospective, multi-institutional trials should be considered standard of care not only for children, but also for affected adolescents and (young) adults. Such trials are essential in guaranteeing that all patients have access to appropriate care and that progress from biological studies can be translated into prognostic improvements without undue delay. The rarity of bone sarcomas increasingly requires trials to be multinational.
骨肿瘤，尤其是骨肉瘤和尤因肉瘤家族肿瘤（ESFT），是青少年和年轻成年人的典型恶性肿瘤。目前针对各年龄段患者的诊断和治疗指南是在这个特定年龄组制定的。骨肉瘤治疗的目标应该是在治愈原发性肿瘤和所有（微）转移灶的同时，尽可能多地保留（肢体）功能，并尽量减少治疗引起的晚期效应。骨肉瘤治疗需要密切的多学科合作。通常，治疗包括诱导化疗，随后是原发性肿瘤（以及如有原发性转移灶）的局部治疗，以及进一步的辅助化疗。对于骨肉瘤，只要可行，局部治疗应首选手术。手术在ESFT中的重要性也日益凸显，ESFT长期以来一直被认为是放疗的领域。现代重建技术不断扩大保肢手术的适应症，特别是对于尚未达到骨骼成熟的患者。不仅对于儿童，而且对于受影响的青少年和（年轻）成年人，在前瞻性多机构试验框架内进行治疗应被视为标准治疗。此类试验对于确保所有患者都能获得适当治疗，以及确保生物学研究的进展能够及时转化为预后改善至关重要。骨肉瘤的罕见性日益要求试验必须跨国进行。
