Smith J, Botet J F, Yeh S D
Radiology. 1984 Sep;152(3):583-90. doi: 10.1148/radiology.152.3.6235535.
This is a comprehensive review of 85 patients who had bone sarcoma associated with Paget disease and who were seen at Memorial Sloan-Kettering Cancer Center between 1927 and 1982. There was an almost equal distribution of tumors in the axial and the appendicular skeletons. The pelvis, humerus, femur, and skull were the tumor sites in 80% of cases. The tumors were bulky large soft tissue masses. Lytic lesions were more common than sclerotic lesions. Mixed lytic and sclerotic lesions were much less common than either single type. Periosteal reaction was uncommon and found in less than 7%. Methylene diphosphonate scans of the bone often showed a cold area that was associated with marked increase in uptake on the gallium scan. Angiography, which was performed in 13 patients, was useful, but CT was much more helpful in showing the soft tissue mass as well as the extent of bony disease. Only three patients in this study survived for five years. Present chemotherapy protocols were disappointing in the treatment of this highly lethal tumor.
这是对85例患有与佩吉特病相关的骨肉瘤患者的综合回顾，这些患者于1927年至1982年间在纪念斯隆-凯特琳癌症中心接受诊治。肿瘤在轴向骨骼和附属骨骼中的分布几乎相等。骨盆、肱骨、股骨和颅骨是80%病例的肿瘤发生部位。肿瘤为体积巨大的软组织肿块。溶骨性病变比硬化性病变更常见。混合性溶骨和硬化性病变比单一类型的病变少见得多。骨膜反应不常见，发生率低于7%。骨的亚甲基二膦酸盐扫描常显示一个冷区，而镓扫描显示摄取明显增加。13例患者进行了血管造影，血管造影是有用的，但CT在显示软组织肿块以及骨病范围方面更有帮助。本研究中只有3例患者存活了5年。目前的化疗方案在治疗这种高度致命的肿瘤方面令人失望。
