Chen Tiffany, Ashman Peter E, Bojrab Dennis I, Johnson Andrew P, Benson Brian, Hong Robert S, Svider Peter F
Hackensack Meridian School of Medicine, Nutley, New Jersey, USA.
Department of Otolaryngology-Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, New Jersey, USA.
Otolaryngol Head Neck Surg. 2022 Jan;166(1):48-59. doi: 10.1177/01945998211004590. Epub 2021 May 4.
To perform a systematic review to investigate common otologic manifestations of Langerhans cell histiocytosis, the incidence of these findings, methods for diagnosis, as well as medical and surgical management.
PubMed/MEDLINE, Embase, and Cochrane Library.
A search of PubMed/MEDLINE, Embase, and Cochrane Library for all articles published between 1963 to 2020 was performed with variations and combinations of the following search terms: Langerhans cell histiocytosis, eosinophilic granuloma, Letterer-Siwe, Hand-Schüller-Christian, otitis, otologic, ear. A review of the references of all included articles was also conducted.
Sixty-two articles encompassing 631 patients met inclusion criteria. Otologic symptoms at presentation were found in 246 (39%) patients in the reported studies with 48% reporting bilaterality. The mean age was 14.8 years with a male predominance (64%). The most common otologic presenting symptom was otorrhea (46%). A majority had the multisystem variant (52%). The most common treatment modalities were chemotherapy (52%), followed by surgery (50%), systemic steroids (45%), and radiotherapy (31%). Surgery was performed in 75.8% with unisystem involvement and in 50.6% with multisystem involvement. The most effective treatments included radiotherapy (56% success rate, 17% of treated patients), systemic steroids (44% success, 20% treated), chemotherapy (41% success, 21% treated), and surgical modalities (36% success, 19% treated).
Otologic manifestations that occur with the multisystem variant or are at high risk for central nervous system involvement necessitate systemic treatment. For unifocal lesions, surgery is recommended. Lastly, radiotherapy should be reserved for extensive lesions involving vital structures or presenting in older patients.
进行一项系统评价，以研究朗格汉斯细胞组织细胞增多症的常见耳科表现、这些表现的发生率、诊断方法以及内科和外科治疗。
PubMed/MEDLINE、Embase和Cochrane图书馆。
在PubMed/MEDLINE、Embase和Cochrane图书馆中检索1963年至2020年期间发表的所有文章，使用以下检索词的变体和组合：朗格汉斯细胞组织细胞增多症、嗜酸性肉芽肿、莱特勒-西韦病、汉-许-克病、中耳炎、耳科、耳朵。还对所有纳入文章的参考文献进行了综述。
62篇文章纳入631例患者，符合纳入标准。在报告的研究中，246例（39%）患者在就诊时有耳科症状，48%报告为双侧性。平均年龄为14.8岁，男性占优势（64%）。最常见的耳科就诊症状是耳漏（46%）。大多数患者为多系统型（52%）。最常见的治疗方式是化疗（52%），其次是手术（50%）、全身用类固醇（45%）和放疗（31%）。单系统受累患者手术治疗率为75.8%，多系统受累患者为50.6%。最有效的治疗方法包括放疗（成功率56%，占治疗患者的17%）、全身用类固醇（成功率44%，占治疗患者的20%）、化疗（成功率41%，占治疗患者的21%）和手术方式（成功率36%，占治疗患者的19%）。
多系统型出现的耳科表现或有中枢神经系统受累高风险的表现需要全身治疗。对于单发病变，建议手术治疗。最后，放疗应保留用于累及重要结构或在老年患者中出现的广泛病变。
