Donáth J, Szilágyi M, Fornet B, Bély M, Poór G
National Institute of Rheumatology and Physiotherapy, Budapest, Hungary.
Eur Radiol. 2000;10(10):1664-8. doi: 10.1007/s003300000456.
The authors describe the case of a male patient who had had Paget's disease since 1973 which led to the diffuse swelling of the proximal femur in 1993. The elevated alkaline phosphatase level and the destructive lytic lesions on the X-ray of the femur raised the possibility of malignancy. The bone scan showed increased accumulation in the proximal part of the left femur. On the MR the low T1 and the elevated T2 signal intensity suggested malignity despite that in some regions signals typical of adipose tissue could also be seen, which is usually interpreted as a sign of benignity. In the histological specimens no signs of malignant tumour cells were identified. The patient received bisphosphonate treatment which decreased his pain and swelling and also the alkaline phosphatase level. The patient died because of a rapidly progressing retroperitoneal fibrosarcoma and lung metastasis in 1996. The autopsy did not prove any malignant transformation of Paget's disease of bone in the proximal femur.
作者描述了一名男性患者的病例，该患者自1973年起患有佩吉特病，1993年导致股骨近端弥漫性肿胀。碱性磷酸酶水平升高以及股骨X线片上的溶骨性破坏病变增加了恶性肿瘤的可能性。骨扫描显示左股骨近端放射性摄取增加。磁共振成像（MR）显示T1加权像低信号、T2加权像高信号，提示为恶性，尽管在某些区域也可见典型脂肪组织信号，通常被解释为良性征象。组织学标本中未发现恶性肿瘤细胞迹象。患者接受了双膦酸盐治疗，疼痛、肿胀减轻，碱性磷酸酶水平也降低。1996年，患者因快速进展的腹膜后纤维肉瘤和肺转移死亡。尸检未证实股骨近端佩吉特骨病发生任何恶性转化。
