头面部郎格罕细胞组织细胞增生症的临床分析

Clinical analysis of Langerhans cell histiocytosis of head and face

目的:研究头面部郎格罕细胞组织细胞增生症(LCH)的诊断和治疗。方法:回顾分析19例经病理证实的头面部LCH患者的临床资料。对其临床表现、诊断和治疗进行探讨。结果:19例中发生于头皮颅骨5例,上颌骨5例,颞骨9例。Ⅰ型Ⅰ级8例(均为成人),Ⅱ型Ⅰ级7例(均为小儿),Ⅲ型Ⅱ级2例(小儿1例,成人1例),Ⅲ级1例(小儿),Ⅳ级1例(小儿)。临床主要表现为头皮包块、颅骨缺损、面颌部肿胀或包块、耳颞肿胀、耳漏、耳道肉芽等。15例接受手术加放疗后痊愈,4例手术后接受化疗治愈。遗留尿崩症1例。结论:根据临床表现、影像学及组织病理学特征可对LCH做出诊断。手术、放疗和化疗是治疗LCH的有效方法。只要不误诊,及时合理治疗,一般预后良好。

Objective： To study the diagnosis and treatment for Langerhans cell histiocytosis（LCH） of head and face. Method：Clinical data of 19 eases suffering from LCH of head and face verified by pathology was retro- spectively analyzed. The clinical presentation, diagnosis and treatment were discussed. Result：Of all the 19 cases, 5 patients had an involvement of the scalp or skull, 5 had the maxilla involvement and 9 temporal bone. Among the 19 patients, type Ⅰ stage Ⅰ was 8 cases（all adults）, typeⅡ stage Ⅰ 7 cases（all children）, type Ⅲ stageⅡ 2（1 adult, 1 child）, stage Ⅲ 1 and stage Ⅳ1（both child）. Their main clinical manifestations included scalp mass, de fect of skull, swelling or mass of facial maxilla and of ear or temporal bone, otorrhea, granuloma of auricular ca- nal, and so on. Fifteen cases were cured by surgical curettage combined with radiotherapy and 4 by surgical curet- lage combined with chemotherapy. One patient had the sequelae of diabetes insipidus. Conclusion： Diagnosis of LCH should be based on synthetical analyses of clinical manifestations, features of imaging and histopathology. Surgical curettage, radiotherapy and chemotherapy are effective treatment regimens. The prognosis is generally good if the patient gets correct diagnosis and timely rational treatment.