Greditzer H G, McLeod R A, Unni K K, Beabout J W
Radiology. 1983 Feb;146(2):327-33. doi: 10.1148/radiology.146.2.6571760.
The most serious complication of Paget disease is the development of sarcoma. Forty-one cases of bone sarcoma in Paget disease, which represented our institution's entire experience, were reviewed. The relative frequency of sarcomatous degeneration was 0.9%. Histologically, 35 of 41 lesions were osteogenic sarcomas, and six were fibrosarcomas. The distribution of lesions was similar to that expected with uncomplicated Paget disease, except for a disproportionately high number of lesions in the humerus; only one lesion involved the vertebrae. The tumors were categorized radiographically as lytic, mixed, and sclerotic, in descending order of frequency. The vast majority of lesions occurred in the pelvis, humerus, and femur. Overall prognosis for these patients was poor, with a five-year survival of 8%. Finding areas of cortical destruction is particularly useful in the detection of malignant degeneration. The radiologist must maintain a high index of suspicion when evaluating radiographs of patients with Paget disease, especially of those who present with pain or a palpable mass.
佩吉特病最严重的并发症是肉瘤的发生。对41例佩吉特病骨肉瘤病例进行了回顾，这些病例代表了我们机构的全部经验。肉瘤变性的相对发生率为0.9%。组织学上，41个病变中有35个为骨肉瘤，6个为纤维肉瘤。病变分布与单纯佩吉特病预期的分布相似，但肱骨中的病变数量不成比例地高；只有一个病变累及椎体。肿瘤在放射学上分为溶骨性、混合性和硬化性，按频率从高到低排列。绝大多数病变发生在骨盆、肱骨和股骨。这些患者的总体预后较差，五年生存率为8%。发现皮质破坏区域对检测恶性变性特别有用。放射科医生在评估佩吉特病患者的X光片时必须保持高度的怀疑指数，尤其是那些伴有疼痛或可触及肿块的患者。
