Lewoczko K B, Rohman G T, LeSueur J R, Stocks R M, Thompson J W
Department of Otolaryngology-Head and Neck Surgery, University of Tennessee Health Science Center, 910 Madison Avenue, Room 428, Memphis TN 38163, USA.
Department of Otolaryngology-Head and Neck Surgery, University of Tennessee Health Science Center, 910 Madison Avenue, Room 428, Memphis TN 38163, USA.
Int J Pediatr Otorhinolaryngol. 2014 Nov;78(11):1874-6. doi: 10.1016/j.ijporl.2014.08.015. Epub 2014 Aug 20.
Langerhan's cell histiocytosis (LCH) is an uncommon disease characterized by abnormal proliferation of polyclonal Langerhan's cells, most commonly presenting with head and neck manifestations. This is a report of a series of patients with LCH at St. Jude Children's Research Hospital over a 46-year period. The purpose was to examine the head and neck presentations of LCH, their treatments and outcomes, and to compare with other previously reported series.
This was a retrospective study of all patients with a diagnosis of LCH who presented to St. Jude Children's Research Hospital, Memphis, TN between 1962 and 2008. Patients who presented with an initial diagnosis of LCH but were later determined to not fit the diagnostic criteria were excluded from the study. IRB approval was obtained and a chart review was conducted to collect data regarding demographics, tumor site(s) and manifestations, pathology, treatment, surgical procedures, and outcomes. This data was compiled and compared to previously published results.
Eighty-eight cases of LCH with at least one head and neck manifestation were diagnosed during the period studied. There were 54 males and 34 females, with an average age of onset of 4.1 years. The most common lesions were those involving the skull (44.3%) and cervical lymph nodes (40.9%). The most common treatment was chemotherapy (80%) and corticosteroids (64%), with vinblastine being the most common chemotherapeutic agent. Surgical intervention occurred in 39% of cases. Total mortality due to LCH was 9.1%. The results were found to generally concur with other previously published studies.
This study represents the largest series of head and neck manifestations of LCH reported to date. Although LCH is a rare disease, it often mimics other common head and neck pathologies and therefore requires a high index of suspicion. Biopsy is required for definitive diagnosis and the mainstay of treatment is chemotherapy.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见疾病，其特征为多克隆朗格汉斯细胞异常增殖，最常见的表现为头颈部症状。本文报告了圣裘德儿童研究医院46年间一系列LCH患者的情况。目的是研究LCH的头颈部表现、治疗方法及结果，并与之前报道的其他系列进行比较。
这是一项对1962年至2008年间就诊于田纳西州孟菲斯市圣裘德儿童研究医院且诊断为LCH的所有患者的回顾性研究。最初诊断为LCH但后来被确定不符合诊断标准的患者被排除在研究之外。获得了机构审查委员会（IRB）的批准，并进行了病历审查，以收集有关人口统计学、肿瘤部位及表现、病理学、治疗、手术程序和结果的数据。对这些数据进行整理并与之前发表的结果进行比较。
在所研究期间，共诊断出88例至少有一处头颈部表现的LCH患者。其中男性54例，女性34例，平均发病年龄为4.1岁。最常见的病变部位是颅骨（44.3%）和颈部淋巴结（40.9%）。最常用的治疗方法是化疗（80%）和使用皮质类固醇（64%），长春碱是最常用的化疗药物。39%的病例进行了手术干预。LCH导致的总死亡率为9.1%。研究结果与之前发表的其他研究基本一致。
本研究是迄今为止报道的LCH头颈部表现病例数最多的系列研究。尽管LCH是一种罕见疾病，但它常与其他常见的头颈部疾病相似，因此需要高度怀疑。明确诊断需要进行活检，治疗的主要手段是化疗。
