儿童头面部局限性郎格罕细胞组织细胞增生症临床特点分析

Clinical characteristics of localized Langerhans cell histiocytosis in children

目的:探讨儿童头面部局限性郎格罕细胞组织细胞增生症(LCH)临床特点,提高诊疗水平。方法:回顾性分析8例儿童头面部局限性LCH临床资料,包括发病时间、发病部位、影像学资料、临床表现、治疗方法。结果:1例鼻背部病变初诊为慢性炎症,经过反复手术病理确诊;6例单纯头面部包块,无疼痛及红肿症状;1例颞部肿块并对侧耳道流脓。5例单纯手术切除病灶;3例手术切除病灶,病理确诊后继续化疗。随访2~3年,患儿情况良好。结论:儿童头面部局限性LCH容易误诊,应作为头颈肿块的重要鉴别诊断;手术切除联合辅助化疗,治疗效果良好。

Objective: To explore the clinical features of cephalic and facial limited langerhans cell histiocytosis(LCH) in children for improving its diagnosis and treatment. Methods: Clinical data of 8 children with cephalic and facial limited LCH were retrospectively analyzed, including the onset time of disease, lesion location, imaging data, clinical manifestations and treatment strategies. Results: One case was preliminarily diagnosed as chronic inflammation with nasal back lesions, then conformed by repeated surgical pathology. Six cases were found to have simple cephalic and facial lumps without pain and swelling. One case was found to have temporal lump with suppurate in the lateral auditory canal. Five cases were treated with surgical excision of lesions. Three cases were treated with surgical excision of lesions, and continued with chemotherapy after confirmed pathological diagnosis. All cases were followed up for 2-3 years with good prognosis. Conclusion: Cephalic and facial limited LCH in children was easy to be misdiagnosed and should be regarded as animportant differential diagnosis of cephalic and facial lumps. Good outcome is achieved by treatment with surgical resection combined with adjuvant chemotherapy.