摘要目的 分析原发性骨骼恶性纤维组织细胞瘤(MFH)影像表现与病理的关系,以提高诊断准确性.方法 回顾性分析经手术病理证实的原发性骨骼MFH 13例,术前分别经X线平片、CT平扫、MR SE T1 WI、T2 WI和增强T1 WI检查,复习影像表现并与手术病理作对照.结果 13例原发骨骼MFH中,11例位于骨端,2例位于骨干,位于骨端者与骨性关节面的距离为1～5 cm.肿瘤呈溶骨性骨质破坏,直径5.3～12.7 cm.其中,破坏区偏心10例,破坏区内见少许分隔7例,周边不连续硬化11例,伪足样突起7例,骨膜增生2例,软组织肿块9例,所有患者软组织肿块体积较小.CT扫描显示肿瘤密度与肌肉密度相仿.在MR T1 WI肿瘤为等低信号,在T2 WI为混杂信号,T2 WI常见片状等低信号,病灶周围常见不完整的低信号环,环外见伪足样突起.动态增强呈进行性延迟强化,强化显著,且较均匀.大体病理显示病灶偏心,膨胀不明显,肿瘤多突破皮质形成范围较小的软组织肿块,呈结节状或假性包裹样,肿瘤内见纺锤形成纤维细胞和胞体较小的组织细胞样细胞,伴有数量不等的胶原纤维,细胞密集呈席纹状或放射状排列.肿瘤微血管丰富.结论 原发骨骼MFH好发于中年患者长骨骨端,T2 WI常见等低信号成分,增强扫描强化显著;MRI表现与病理关系密切.

abstractsObjective To explore the imaging features of primary malignant fibrous histiocytoma(MFH)of bone and correlate them with pathological findings.Methods Thirteen cases patients with primary MFH of bone confirmed by surgical pathology underwent radiography,spiral CT plain scanning and MR SE-T1 WI,T2 WI and SE-T1 WI enhancement scanning before operation.The imaging date was reviewed and analysed retrospectively in comparison with surgical and pathological results.Results Of 13 MFH,11 were located in the end of long bone,and 2 in the diaphysis.The distance between tumors in the end of long bone and adjacent joint surface was 1 to 5 cm.All lesions showed osteolytic destruction with the maximum diameter of the tumors from 5.3 to 12.7 cm.The tumors had eccentric aggressive osteolytic destruction in 10 lesions,internal crest within the lesions in 7,inconsecutive marginal osteosclerosis in 11,little periosteal reaction in 2 and small soft tissue masses in 9,respectively.The CT value of lesions was similar to muscle.MR imaging depicted low signal intensity with aggressive features on T1 WI,iso to slight high signal intensity on T2 WI,and middle or high degree contrast enhancement on enhanced T1 WI images.Macroscopically,MFH was usually located eccentrically within the bone and produced little or no osseous expansion.The soft tissue component appeared multi-nodules and pseudo-encapsulated.Histologically,they consisted of spindle-shaped fibroblasts,which radiated outward in a spiral array from a central focus and produced a nebula or storiform appearance,and cells,which were small and oval with little visible cytoplasm.Conclusions The imaging manifestations of MFH were specific to some extent.Combined utilization of plain X-ray,CT,and MRI is helpful for the diagnosis and differential diagnosis of MFH.