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作者复习了22例Paget肉瘤的临床、X线及病理特征,以期确定哪些Paget病人最可能发生肉瘤,以及哪些X线表现能提示诊断。男12例,女10例,年龄48～87岁。临床表现包括:疼痛和/或肿块11例,病理性骨折7例,神经症状4例。20例病人的存活时间为活检后5天～2.5年。2例病人分别在第2年和第8年时中断随访。22例中16例为骨肉瘤、3例为软骨肉瘤、2例为纤维肉瘤、1例为恶性纤维组织细胞瘤。X线上成骨性改变均为骨肉瘤,肿块内有软骨钙化为软骨肉瘤,溶骨改变或溶骨成骨混合改变对确定肿瘤组织类型价值不大。21例单发肿瘤中以股骨最常见(8例),亦可见于其他少见部位。1例多灶性骨肉瘤病变仅限于具有Paget病变的骨骼。17例病人肿瘤发生前就有Paget症状存在,其中15例病人系多骨发病。4例肉瘤位于2个月至15年前发生骨折部位的附近。21例病人
The author reviewed the clinical, X-ray, and pathological features of 22 cases of Paget’s sarcoma in order to determine which Paget patients were most likely to have sarcoma and which X-ray findings could suggest a diagnosis. There were 12 males and 10 females, aged 48-87 years old. Clinical manifestations included 11 cases of pain and/or masses, 7 cases of pathological fractures, and 4 cases of neurological symptoms. The survival time of 20 patients was 5 days to 2.5 years after biopsy. Two patients were discontinued during the second and eighth years, respectively. Of the 22 cases, 16 were osteosarcoma, 3 were chondrosarcoma, 2 were fibrosarcoma, and 1 was malignant fibrous histocytoma. Osteochondral changes in the X-rays were all osteosarcoma. Cartilage in the tumor calcified into chondrosarcoma. Osteolytic changes or mixed osteoblastic changes were of little value in determining the type of tumor tissue. In 21 cases of single tumors, the femur was the most common (8 cases), and it was also found in other rare sites. One case of multifocal osteosarcoma was limited to bone with Paget lesions. Seventeen patients had Paget symptoms before tumorigenesis, and 15 of them had bone disease. Four cases of sarcoma were located near the fracture site from 2 months to 15 years ago. 21 patients