儿童骨嗜酸性肉芽肿的临床特征、治疗及预后(附33例)

Clinical features,treatment and prognosis of eosinophilic granuloma of bone in children(33 cases)

目的:探讨儿童骨嗜酸性肉芽肿(eosinophilic granuloma,EG)的临床特征、治疗和预后。方法:回顾性分析2004年01月至2021年06月我院小儿骨科33例骨EG患者,分为单病灶受累和多病灶受累两组,收集患者的一般资料、临床特征、治疗和转归情况,采用Kaplan-Meier方法估计全组及两组的无事件生存率,并用对数秩检验(log-rank检验)进行两组比较。结果:男性25例(76%),女性8例(24%),诊断时的中位年龄2.1岁(0.2岁~7岁)。主诉多为骨局部肿块、疼痛、功能受限。其中四肢受累最多,其次为脊柱、骨盆、颅骨。单病灶组21例,其中12例行局部病灶切除手术治疗,有或没有使用局部类固醇;7例行手术及术后全身化疗,有或没有使用局部类固醇;2例行活检后全身化疗。多病灶组3例行手术联合全身化疗,9例行活检后全身化疗。单病灶组1例复发,多病灶组5例进展,两组均无患儿死亡。单病灶组较多病灶组EFS高,差异有统计学意义(P=0.009),中位随访时间7.3年(0.8年~17.5年)。结论:骨EG风险较低,无明显生命威胁,预后较好。单病灶受累较多病灶受累者的复发或进展较少、手术效果好、治愈率高;多病灶受累者化疗效果较好,进行积极的治疗并坚持长期随访可减少并发症的产生,提高患者的生存质量。

Objective:To investigate the clinical features,treatment and prognosis of eosinophilic granuloma of bone(EG)in children.Methods:From January 2004 to June 2021,33 patients with bone EG in the department of pe⁃diatric orthopaedics,of our hospital were retrospectively analyzed.They were divided into two groups:Single focus in⁃volvement group and multi-focus involvement group.The general data,clinical features,treatment and outcome of the patients were collected.The event-free survival(EFS)rate of the whole group and the two groups was estimated by Kaplan-Meier method,and the logarithmic rank test(log-rank test)was used to compare the two groups.Results:There were 25 males(76%)and 8 females(24%).The median age at the time of diagnosis was 2.1 years(0.2~7 years).The main complaints were local bone mass,pain and functional limitation.Among them,the limbs were the most involved,followed by the spine,pelvis and skull.In the 21 cases of the single focus group,12 cases underwent lo⁃cal resection with or without local steroids,7 cases underwent operation and postoperative systemic chemotherapy with or without local steroids,and 2 cases received systemic chemotherapy after biopsy.In the muliti-fous groups,3 pa⁃tients received surgery combined with systemic chemotherapy,and 9 patients recived systemic chemotherapy after bi⁃opsy.There was 1 case of recurrence in the single focus group and 5 cases in the multi-focus group.There was no death in both groups.The EFS rate in the single lesion group was higher than that in the multi-focus group,and the difference was statistically significant(P=0.009).The median follow-up time was 7.3 years(0.8~17.5 years).Conclusion:The risk of bone EG is low.There is no obvious life threat,and the prognosis is good.The patients with single focus and multiple lesions had less recurrence or progression,good surgical effect and high cure rate,and the chemotherapy effect of patients with multiple lesions was better.Active treatment and long-term follow-up can re⁃duce the occurrence of complications and improve the quality of life of patients.