骨嗜酸细胞肉芽肿的临床特征与治疗

Clinical feature and treatment of eosinophilic granuloma of bone

目的探讨骨嗜酸细胞肉芽肿的临床表现、诊断和治疗效果。方法1991年3月至2006年3月收治骨嗜酸细胞肉芽肿28例,男19例,女9例;年龄1-61岁,平均22.4岁。病程20d～10个月。单发24例,多发4例。影像学表现为溶骨性改变,15例伴软组织肿物影。于外院确诊5例,入院后误诊为原发肿瘤及转移癌12例。所有病例均经病理学确诊。单发病例行化疗1例,手术治疗20例,手术后辅以放疗、化疗、激素综合治疗3例;多发病例采用放疗1例,激素治疗1例,综合治疗2例。结果全部病例随访7个月￣15年,平均5.3年。20例手术者手术治愈率100%;术后辅以放疗、化疗及激素治疗者无复发;植骨者6～12个月骨性愈合,随访功能不受限。1例行胸椎椎板减压术者截瘫部分恢复。4例多发病例中2例出现复发,再次给予激素及放疗、化疗后缓解。结论骨嗜酸细胞肉芽肿临床症状及影像学表现与肿瘤类似,误诊率极高。术前应尽可能行穿刺活检。对单发病例宜采用手术治疗,可辅以放疗或化疗;多发病例病灶多、范围广,不宜采用手术治疗,可选择放疗、化疗、激素治疗等综合疗法。

Objective Through analysing the clinical features of eosinophilic granuloma of bone, to study its diagnosis and therapeutics. Methods The clinical features, diagnosis and treatment of 28 patients with eosinophilic granuloma of bone in Cancer Hospital Affiliated Tianjin Medical University from March 1991 to March 2006 were retrospectively analyzed. The patients included 19 males, 9 females; age from 1 to 61 years old, with the average of 22.4 years old. The history of the disease was from 20 days to 10 months. Cases of unifocal lesion were 24, multifocal lesions were 4. The manifestation of radiograph was the evidence of bony erosion, and 15 cases had mass in soft tissue. 5 of 28 patients were diagnosed in other hospitals, and 12 of 28 patients were misdiagnosed as primitive tumor or metastatic tumor after checked. All the patients were performed by microscopic examination. 20 of 24 patients with solitary focal only underwent operation. 3 patients performed muhitherapy after operation, and 1 patient received chemotherapy. 2 patients with muhifocal lesions received radiotherapy and hormone therapy desperately, 2 patients received combined therapy. Results All the patients were followed up from 7 months to 15 years, with the average 5.3 years. The rate of cure was 100% in the 20 patients who underwent operation. No recurrent case was found in the other 3 cases treated with muhitherapy after operation. Bone union were got 6 to 12 months postoperatively in the patients with bone graft. Paraplegia recovered partially in 1 case after thoracic vertebral lamina depression. In those 4 patients with muhifocal lesions, 2 patients recurred after 1 year, consequently relieved after chemo-, radio-, and hormone therapy. Conclusion It is not easy to diagnoze eosinophilic granuloma of bone by clinical symptoms and radiological manifestations, due to the similar feature to tumor. Incisional biopsy and frozen-section should be done before operation. Operation is the best treatment and can be combined with chemotherapy and radiotherapy for solitary focal lesion. Muhifocal lesions can be combined by chemotherapy and radiotherapy and hormone therapy. Combination therapy appears to be an effective method of treating recurrent disease.