原发性骨内Rosai–Dorfman病的影像学表现及其病理相关性分析

Radiological Characteristics and Pathological Correlation Analysis of Primary Rosai-Dorfman Disease of Bone

目的探讨原发性骨内Rosai–Dorfman病(RDD)的影像学特征及病理特点。方法经手术病理证实的4例原发性骨RDD病的临床及影像学特征,同时复习国内外文献病例分析。结果骨内RDD病中本组4例,文献23例;其中男性13例,女性14例,年龄2-72岁,平均年龄(33.55±19.38)岁。6例椎体及附件,5例股骨,4例胫骨,4例颅骨及颅底,3例桡骨,2例肱骨,1例骰骨,1例舟骨,1例肋骨,26例(96%)显示溶骨性骨质破坏,24例(89%)未见骨膜反应,23例(85%)边缘未见硬化,7例突破骨皮质,侵犯周围软组织,27个病例其中仅10例做了增强,有9例呈明显强化,病灶密度均匀,周围水肿不明显。结论骨内RDD患者不同年龄均可发病,影像上呈溶骨性骨质破坏,骨膜反应及边缘骨质硬化少见,部分可突破骨皮质侵犯周围软组织,增强明显强化,软组织肿块更加少见,部分与病理有一定相关性,该病的确诊主要依靠细胞形态学及免疫组织化学检查。

Objective To investigate the imaging and pathological features of primary intraosseous Rosai-Dorfman disease(RDD).Methods Clinical and imaging characteristics of 4 cases of primary bone RDD disease confirmed by surgical pathology,and reviewed the literature and case analysis at home and abroad.Results There were 4 cases of intraosseous RDD disease in this group and 23 cases in literature;13cases were males and 14 cases were females,aged 2-72 years old,with an average age of(33.6±19.4)years old.Six cases of vertebral body and appendages,5 cases of femur,4 cases of tibia,4 cases of skull and skull base,3 cases of radius,2 cases of humerus,1 case of cuboid bone,1 case of navicular bone,and 1 case of ribs were collected.Twenty six cases(96%)Showed osteolytic bone destruction,24 cases(89%)had no periosteal reaction,23 cases(85%)had no marginal sclerosis.The bone cortex were broke through and surrounding soft tissues were invaded in 7 cases.Only 10 cases of 27 cases had enhanced examination.Among them,9 cases showed obvious enhancement,the density of the lesions was uniform,and the surrounding edema was not obvious.Conclusion Intraosseous RDD patients can develop the disease at different ages.The image shows osteolytic bone destruction.Periosteum reaction and marginal bone sclerosis are rare.Some can break through the cortex and invade the surrounding soft tissues,enhance obvious enhancement.Soft tissue masses are more rare,and some are pathological There is a certain correlation,the diagnosis of the disease mainly depends on cell morphology and immunohistochemical examination.