Pignatti G, Bacci G, Picci P, Dallari D, Bertoni F, Bacchini P, Capanna R
Department of Internal Medicine, Istituto Ortopedico Rizzoli, Bologna, Italy.
Clin Orthop Relat Res. 1991 Sep(270):99-106.
Seventeen patients with telangiectatic osteogenic sarcoma (TOS) of the extremities were treated with neoadjuvant chemotherapy, according to two different protocols. Preoperatively, the patients received high-dose methotrexate (HD-MTX) and cisplatinum (CPD) (HD-MTX/CPD) and doxorubicin. CPD was delivered intraarterially, the other drugs intravenously. Limb-salvage surgery was performed in 12 instances, and five patients were treated with amputation. Postoperative chemotherapy was tailored according to the grade of chemotherapy-induced necrosis. In 13 cases (86%), the resultant grade of necrosis was greater than 90% (good responders). The mean follow-up interval was 3.5 years with a range of 18 to 78 months. Fourteen patients (82%) remained continuously disease-free, while three patients developed lung metastases. Two of these died because of uncontrolled disease, whereas the third patient is alive and disease-free after metastasectomy. No local recurrences were observed. These results are better than those observed in 215 contemporary cases of conventional osteosarcoma treated with the same protocols. This study confirms that TOS is not a uniformly lethal tumor as suggested by prior reports. By employing neoadjuvant chemotherapy, a high percentage of patients with TOS can be cured, and in most of them, a limb-sparing surgery is possible and safe.
17例肢体毛细血管扩张性骨肉瘤（TOS）患者根据两种不同方案接受了新辅助化疗。术前，患者接受大剂量甲氨蝶呤（HD-MTX）和顺铂（CPD）（HD-MTX/CPD）以及阿霉素治疗。CPD通过动脉内给药，其他药物通过静脉给药。12例患者接受了保肢手术，5例患者接受了截肢治疗。术后化疗根据化疗诱导坏死的程度进行调整。13例患者（86%）坏死程度大于90%（反应良好）。平均随访时间为3.5年，范围为18至78个月。14例患者（82%）持续无病生存，3例患者发生肺转移。其中2例因疾病无法控制而死亡，第3例患者在转移灶切除术后存活且无病生存。未观察到局部复发。这些结果优于采用相同方案治疗的215例当代常规骨肉瘤患者的结果。本研究证实，TOS并非如先前报道所暗示的那样是一种一律致命的肿瘤。通过采用新辅助化疗，高比例的TOS患者可以治愈，并且在大多数患者中，保肢手术是可行且安全的。
