儿童颞骨朗格汉斯组织细胞增生症7例临床分析

Langerhans cell histiocytosis of the temporal bone in children:7cases analysis

目的：分析儿童颞骨朗格汉斯组织细胞增生症（LCH）的临床表现、诊断、治疗及预后。方法：回顾性分析中南大学湘雅二医院耳鼻咽喉头颈外科2009-04-2014-04期间收治并经病理确诊的7例颞骨LCH患儿,研究其临床表现、影像学检查、诊断、治疗及预后等特点,并根据临床分类分组,比较组间预后差异。结果：7例LCH患儿中5例表现为单系统病变（3例为颞骨单灶型,2例为多灶型）,2例表现为多系统病变（1例眼部受累,1例肺部受累）。临床主要表现为颞部肿块、耳漏、听力下降、外耳道肉芽等,CT显示颞骨呈溶骨性骨质破坏,边界无硬化,可见弥漫性软组织密度影。随访1-5年,4例病情消退,1例病情稳定,2例病情进展。单系统组预后优于多系统组,差异有统计学意义（P〈0.05）。结论：儿童颞骨LCH临床表现多样,无特异性,主要表现为颞部肿块、耳漏、听力下降等,确诊主要靠病理学检查。手术、化疗及放疗是主要的治疗方法,其中单系统病变组的预后优于多系统病变组。

Objective： To analyze the clinical manifestations, diagnosis, therapy and prognosis of Langerhans cell histiocytosis （LCH） of the temporal bone in children. Method：Seven children with LCH of the temporal bone in our hospital were retrospectively summed up from April 2009 to April 2014. The patients were followed up 1-5 years, their clinical manifestations,imaging findings, diagnosis, therapy and prognosis were studied. Correlation between clinical classifications and prognosis was also analyzed. Result： Among the 7 patients, 4 were boys and 3 were girls. 5 cases belonged to the single system group and 2 cases belonged to the multisystem group. The most common clinical characters were temporal tumor, otorrhea, otalgia, hearing loss and granulation of external audi- tory canal. CT of the temporal bones showed extensive osteolytic destructions with diffuse soft tissure density, without border sclerotization. The cases were received different therapies. Followed up for 1-5 years,4 cases were regressive, 1 case kept stable, while 2 cases showed progressive. The two boys then received standard treatment combined steroids with vinblastine. The prognosis in the multisystem group was significantly differentfrom the single system group （P〈0.05）. Conclusion：The clinical manifestations of LCH vary a lot. The diagnosis is based on histological and immunophenotypic examination of lesion tissue. The main therapy includs surgery, chemotherapy and radiotherapy. The prognosis of the single system group is much better than the multisystem group.