成人孤立性骨嗜酸性肉芽肿45例临床分析

Clinical Analyse for 45 Cases of Solitary Eosinophilic Granuloma Bone Lesions in Adults

【目的】探讨成人孤立性骨嗜酸性肉芽肿(EG)的临床表现、影像与病理特点、治疗及预后。【方法】回顾性分析从2003年9月至2017年6月共45例成人孤立性骨EG患者的临床、影像学病理表现及治疗预后情况。【结果】男35例,女10例,中位年龄29岁,临床受累骨骼包括颅面骨(9例)、脊柱(11例)、肋骨(1例)、骨盆(8例)、锁骨(3例)、肩胛骨(2例)、肱骨(3例)及股骨(8例)等,平均随访81月。影像学特征为溶骨性破坏病灶,脊柱病灶中均未出现扁平椎改变,免疫组化多为CD1a、Langerin(CD207)及S100阳性。其中3例予保守观察,23例予手术治疗,8例接受小剂量化疗,其余11例行手术及术后辅助小剂量化疗,其中40例痊愈,5例复发后均再接受化疗,无进展生存时间(PFS)为3～170个月,中位PFS为67个月。【结论】孤立性骨EG临床发病率很低,尤其成人更为罕见,临床表现无明显特异性,影像学表现易误诊,需重视病理检查,因成人孤立性骨EG的生物学行为比儿童更具侵袭性,治疗需个体化的同时可采取比儿童EG更积极的策略。

【Background】To analyze the features of clinical,radiology,pathology,treatment and prognosis of solitary eosinophilic granuloma(EG)bone lesions in adults.【Methods】The records of 45 adult patients with solitary EG bone lesions at our institutions were retrospectively reviewed.The features of clinical,radiology,pathology,treatment and prognosis were analyzed.【Results】There were 35 males and 10 females,the median age was 29 years,the mean follow-up time was 81 months.The distribution of lesions sites were craniofacial bone(9 cases),spine(11 cases),rib(1 case),pelvis(8 cases),clavicle(3 cases),scapula(2 cases),humerus(3 cases),and femur(8 cases).Radiographic findings were characteristically osteolytic lesions,vertebra plana was not found in 11 spinal cases,immunohistochemistry showed positive in CD1a,Langerin(CD207)and S100.Of the 45 adult cases,3 cases received observation;surgery was performed in 23 cases,low dose chemotherapy was given in 8 cases,and the rest of 11 cases were administered surgery and adjuvant chemotherapy.Forty cases healed,while 5 cases developed progress or relapse and received chemotherapy again,the range of progress-free survival(PFS)was 3-170 months(median:67 months).【Conclusions】Solitary EG bone lesions are rare,particularly in adults.Insidious clinical presentation and aggressive radiology feature could easily develop misdiagnose,accordingly pathology is vital for diagnosis.Individualized management and more active intervention are recommended in adult EG due to its more aggressive behavior than children EG.