Le Breton C, Méziou M, Laredo J D, Amouroux J, Mazabraud A, Bigot J M, Tubiana J M
Service de Radiologie, Hôpital Tenon, Paris.
Rev Rhum Ed Fr. 1993 Jan;60(1):16-22.
Eight cases of spinal sarcoma complicating Paget's disease seen in different centers were reviewed. Clinical and radiological features of this condition were determined on the basis of these 8 cases and of 51 previously published cases. In the current series as well as in previous reports, most cases involved the lumbar and sacral spine and manifested as low back pain with sciatica and early development of neurological deficits. Roentgenographic diagnosis proved difficult because of the anatomic complexity of the spine and architectural changes characteristic of the pagetic bone. The most common radiological features was a mixed pattern with both sclerosis and central osteolysis. Computed tomography performed in one patient of current series disclosed osteolysis of the sacrum (not visible on plain roentgenograms) with tumor spread to adjacent soft tissues. Features shared by spinal and nonspinal sarcomas complicating Paget's disease include advanced mean age of patients, predominance of osteogenic sarcomas among histologic forms, increased risk of sarcoma in polyostotic Paget's disease, and a very grim prognosis. Development of a neurological deficit in a patient with Paget's disease is an unusual occurrence which should suggest sarcomatous transformation. Roentgenographic changes are difficult to identify on plain films of the spine and consequently CT scan studies are warranted whenever a sarcoma is suspected.
对不同中心所见的8例脊柱肉瘤合并佩吉特病的病例进行了回顾。基于这8例病例以及之前发表的51例病例,确定了这种疾病的临床和放射学特征。在当前系列病例以及之前的报告中,大多数病例累及腰椎和骶椎,表现为腰痛伴坐骨神经痛以及神经功能缺损的早期出现。由于脊柱的解剖复杂性以及佩吉特病骨的特征性结构改变,X线诊断颇具难度。最常见的放射学特征是硬化和中央骨质溶解并存的混合模式。在当前系列病例中的1例患者进行的计算机断层扫描显示骶骨骨质溶解(在普通X线片上不可见),肿瘤扩散至相邻软组织。脊柱肉瘤和非脊柱肉瘤合并佩吉特病的共同特征包括患者平均年龄较大、组织学类型中骨肉瘤占优势、多骨型佩吉特病中肉瘤风险增加以及预后极差。佩吉特病患者出现神经功能缺损是一种不常见的情况,应提示肉瘤样变。脊柱平片上难以识别X线改变,因此每当怀疑肉瘤时,进行CT扫描检查是必要的。