Objective:To summarize the clinical features, diagnosis and treatment of Langerhans histiocytosis（LCH） which first appeared in the nasal skull base. Method:Ten cases of LCH with nasal and skull base symptoms were analyzed retrospectively. The clinical characteristics of LCH with nasal and skull base symptoms were summarized. The correlation of other systems involved in LCH was analyzed. Result:Among the 10 patients, the youngest was 1 year and 5 months, and the oldest was 8 years, the average age was 3 years. The main imaging manifestations were osteolytic changes and soft tissue invasion. Seven patients were monofocal and three patients were multifocal. For localized lesions, radical resection and follow-up chemotherapy were performed, and conservative treatment was performed for patients with multiple system involvement and obvious systemic symptoms. Eight patients survived, 2 died. Conclusion:LCH occurs frequently in children and has certain clinical characteristics. Single system and single lesion surgery have a better therapeutic effect, and can achieve a greater survival rate with follow-up chemotherapy.
目的：总结首发于鼻颅底的朗格汉斯细胞增生症（LCH）的临床特点，探讨其诊断及治疗方案。 方法：回顾性分析以鼻颅底症状为首发表现的10例LCH患儿的资料，总结其鼻部及颅底症状的特点，并分析累及其他系统的相关性，总结手术治疗的特点及手术联合化疗的转归。 结果：10例患儿年龄1岁5个月~8岁，平均3岁。影像学表现主要为溶骨性改变及软组织侵犯，7例患儿为单灶性，3例患儿为多系统多灶性。对于局限性的病变患儿，采取彻底切除病变加后续化疗的方法；对于累及多系统并有明显全身症状的患儿，采取保守治疗。随访4~96个月，8例存活，2例死亡。 结论：LCH好发于儿童，具有一定的临床特征，单系统单病灶的手术治疗效果较好，配合后续的化疗可获得较大的生存率。.
Keywords: Langerhans histiocytosis; child; nasal skull base; surgical procedures, operative.
Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.