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目的 对长骨孤立性朗格罕细胞肉芽肿的临床特点、影像学和病理学特征进行分析 ,探讨其诊断、鉴别诊断 ,治疗。方法 收集我院 1985～ 2 0 0 1年经临床病理证实的长骨孤立性朗格罕细胞肉芽肿 2 3例资料 ,对其临床表现、X线片和病理学特征以及治疗进行回顾性综合分析。其中 18例标本进行了 S- 10 0蛋白免疫组织化学染色。结果 临床表现主要为局部疼痛伴全身发热、局部肿胀或患部功能障碍。病变部位 :股骨 9例 ,肱骨 7例 ,胫骨 3例 ,腓骨 2例 ,桡骨和尺骨各 1例。X线片主要表现为孤立性边界清楚的溶骨性骨破坏。病理组织学是以朗格罕细胞增生为特点 ,免疫组化染色朗格罕细胞表达 S- 10 0蛋白。治疗均采取单纯刮除术加植骨或骨水泥填塞。平均随访 3.5年 ,无 1例复发。结论 原发于长骨的孤立性郎格罕细胞肉芽肿是一种相对少见而原因不明的骨瘤样病变 ,其临床诊断要与慢性骨髓炎、骨尤文氏肉瘤以及其他骨病或骨肿瘤等鉴别 ,最后确诊要依靠病理组织学检查证实 ,并采取以手术为主的综合治疗。
OBJECTIVE: To analyze the clinical features, imaging features and pathological features of isolated long Langer cell granuloma and to investigate its diagnosis, differential diagnosis and treatment. Methods Clinical data of 23 patients with long bone isolated from Langerhans cell granuloma confirmed by clinicopathology from 1985 to 2001 were collected and analyzed retrospectively for their clinical manifestations, X-ray and pathological features and treatment. Eighteen specimens were immunohistochemically stained with S-10 protein. Results The main clinical manifestations of local pain with general fever, local swelling or affected part dysfunction. Lesions: 9 cases of femur, 7 cases of humerus, 3 cases of tibia, 2 cases of fibula, 1 case of radius and ulna. X-ray film is mainly characterized by isolated osteolytic bone destruction border. Histopathology is characterized by the proliferation of Langerhans cells, immunohistochemical staining Langerhans cells express S- 10 0 protein. Treatment were taken by a simple curettage plus bone graft or bone cement. The average follow-up of 3.5 years, no recurrence in 1 case. Conclusions The isolated long granulomatous granulomatosis from primary long bone is a relatively rare and unexplained osteosarcoid lesion whose clinical diagnosis is related to chronic osteomyelitis, bone Ewing’s sarcoma and other bone diseases or bone tumors , The final diagnosis to rely on histopathological examination confirmed, and to take the surgery-based comprehensive treatment.