耳颞部朗格汉斯组织细胞增生症(附10例报告)

目的研究耳颞部朗格汉斯组织细胞增生症(LCH)的诊断和治疗。方法回顾分析10例经病理证实的耳颞部LCH病例。10例中男女各5例,年龄1~60岁,平均18.3岁。3例为韩-薛-柯病,7例为嗜酸性肉肿。临床主要表现为耳颞肿胀、颅骨缺损、耳漏、鼓膜穿孔、耳道肉芽、耳鸣、眩晕、耳聋、头痛、尿崩症等。结果8例接受手术加放疗后痊愈,2例接受手术后化疗治愈。遗留尿崩症、侏儒症各1例。结论LCH根据临床表现、影像学及组织病理学特征可做出诊断。手术、放疗和化疗是治疗LCH的有效疗法。尽管LCH病情危重,只要未累及重要生命器官、不误诊、及时合理治疗,一般预后良好,但其后遗尿崩症或侏儒症残疾则顽固难治。

Langerhans cell histiocytosis of temporal bone(Report of 10 cases)

Objective To study the diagnosis and treatment for Langerhans cell histiocytosis of temporal bone. Methods Clinical data of 10 cases suffering from LCH of temporal bone verified by pathology was retrospectively analyzed. Of all the cases, male and female were equal in number, aged from 1 to 60 years with an average age of 20. 4 years. As for the pathological classification, 3 cases were Hand-Schuller-Christian and 7 were eosinophilic granuloma. Their clinical manifestations included swelling of ear and temporal bone, defect of skull, otorrhea, perforation of ear drum, granuloma of auricular canal, tinnitus, vertigo, hearing loss, headache and diabetes insipidus. Results Eight cases were cured by surgical curettage combined with radiotherapy and 2 by surgical curettage combined with chemotherapy. The sequelae as diabetes insipidus and dwarfism was 1 respectively. 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. Although LCH is very critical, the prognosis is generally good if vital organs are not involved with correct diagnosis and timely rational treatment But residuals of diabetes insipidus or dwarfism of LCH are obstinate.