[Clinical characteristics of localized Langerhans cell histiocytosis in children]

Abstract

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.

Keywords: Langerhans cell histocytosis; cephalic and facial; children.

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1例额部包块病变患儿的临床资料

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1例鼻背部肿块病变患儿的临床资料 2a：鼻背部手术后溃破；2b、2c：轴位CT示鼻骨、上颌骨破坏；2d：术后1年CT示病变部位已愈合 图 3  1例颞部包块病变患儿的临床资料 3a：颞部包块；3b：轴位CT示病灶；3c：冠状位MRI示病灶；3d：术后3个月CT示愈合;  图 4  1例单系统多发骨损害患儿的CT表现 4a：轴位CT示左顶部溶骨性骨质破坏；4b：轴位CT示右颞部骨质破坏；

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病理学检查