Immenkamp M
Z Orthop Ihre Grenzgeb. 1975 Jun;113(3):331-43.
Report on 2 cases of poly-ostotic fibrous dysplasia combined with pigmented areas of the skin with secondary fibrosarcoma of the ulna or femur. They are analysed together with 49 cases published so far. The average age at the time of malignancy being diagnosed was 34.4 years. The average time from recognition of fibrous dysplasia to malignant changes was 17.7 years. In 14 cases local radiotherapy preceded the development of sarcoma. Femur and jaws were the most common sites. There were 30 osteosarcomas, 11 fibrosarcomas and 7 chondrosarcomas. The prognosis is bad: only 14 patients survived for 5 years. But malignant change is rare in fibrous dysplasia. It occurs in less than 1% of cases. Severe forms of poly-ostotic fibrous dysplasia which still progress in adults with a raised alkali phosphatase appear to show an increased tendency towards malignant degeneration.
两例多骨型骨纤维发育不良合并皮肤色素沉着区继发尺骨或股骨纤维肉瘤的病例报告。将这两例与迄今已发表的49例病例一起进行分析。诊断出恶性肿瘤时的平均年龄为34.4岁。从认识到骨纤维发育不良到发生恶性变化的平均时间为17.7年。在14例病例中，肉瘤发生前进行了局部放疗。股骨和颌骨是最常见的部位。有30例骨肉瘤、11例纤维肉瘤和7例软骨肉瘤。预后不良：只有14例患者存活了5年。但骨纤维发育不良中发生恶性变化的情况很少见。其发生率不到1%。在成年期仍有进展且碱性磷酸酶升高的严重多骨型骨纤维发育不良形式似乎显示出恶性退变的倾向增加。
