36例口腔颌面-头颈部朗格汉斯细胞组织细胞增多症临床及预后分析

doi:10.19438/j.cjoms.2021.01.011

摘要/Abstract

摘要： 目的: 探讨口腔颌面-头颈部朗格汉斯细胞组织细胞增多症（Langerhans cell histiocytosis, LCH）的临床特征、诊断、治疗方法及预后,为临床治疗提供更强的循证医学证据。方法: 回顾分析上海交通大学医学院附属第九人民医院口腔颌面-头颈肿瘤科收治的36例成人及儿童口腔颌面-头颈部朗格汉斯细胞组织细胞增多症患者的临床资料,采用SPSS 22.0软件包对其预后进行分析。结果: 36例LCH患者中,男女比例为5:1,中位年龄31岁（8个月~74岁）。主要临床表现为口腔黏膜溃疡或肉芽肿,并伴有疼痛、肿胀;颅颌面部骨损害呈穿凿样破坏;牙龈坏死、牙移位、脱落和牙槽骨破坏;22%的患者有尿崩症病史。根据Lavin. Osband计分标准,Ⅰ级26例,Ⅱ级9例,Ⅲ级1例,Ⅳ级0例。最常见的部位为颌骨、牙龈、腭部。23例患者接受单一治疗,13例接受联合治疗。中位随访时间为22.5个月,1例患者失访,1例死亡,初治后完全缓解率为75%,5年无复发生存率为53.78%。接受全身治疗的患者20例;单系统受累患者17例,多系统受累患者19例;24例患者检测BRAF-V600E突变,阳性6例;1例口服靶向药物,效果显著。结论: ①口腔颌面-头颈部朗格汉斯细胞组织细胞增多症初治疗效良好,治疗的最终目标是对症处理,延长患者的无复发生存时间。②治疗方式对患者的总生存期有显著影响,化疗可延长患者无复发生存时间;病理确诊后,建议行PET-CT,评估全身状况后,根据器官系统受累情况决定治疗方式。③对于一线、二线方案应答不明显的患者,可考虑检测BRAF-V600E基因突变,根据检测结果选择靶向药物。

关键词: 朗格汉斯细胞组织细胞增多症, 临床特征, BRAF-V600E, 预后

Abstract: PURPOSE: This retrospective study was performed to analyze and discuss the clinical characteristics, diagnosis, treatment and prognosis of Langerhans cell histiocytosis(LCH) in oral maxillofacial head and neck region, in order to provide evidence-based evidence for clinical treatment. METHODS: The clinical data of 36 cases of LCH of adults and children treated in the Department of Oromaxillofacial Head and Neck Oncology, Shanghai Ninth People's Hospital were retrospectively analyzed. The prognosis of LCH was analyzed by SPSS 22.0 software package. RESULTS: The median age of 36 LCH patients was 31 years (8 months to 74 years). The main clinical manifestations were oral mucosal ulcer or granuloma, accompanied by pain and swelling; craniomaxillofacial bone damage was chisel like destruction; gingival necrosis, tooth displacement, tooth loss and alveolar bone destruction. 22% patients had history of diabetes insipidus. According to the scoring standard of Lavin osband, 26 cases were classified as grade Ⅰ, 9 as grade Ⅱ, 1 as grade Ⅲ and 0 as grade Ⅳ. The most common sites were jaw, gum and palate. Twenty-three patients received single treatment, 13 patients received combined treatment. The median follow-up time was 22.5 months, 1 patient was lost to follow-up, 1 patient died. The complete remission rate was 75% after initial treatment. The 5-year relapse free survival rate was 53.78%; 20 patients received systemic therapy; 17 patients had single system involvement, 19 patients had multiple systems involvement, 24 patients underwent detection of BRAF-V600E mutation, 6 patients were positive, and 1 patient took targeted drug orally and the effect was significant. CONCLUSIONS: ① The initial treatment of oral and maxillofacial head and neck Langerhans cell histiocytosis is good, the ultimate goal of the treatment is to treat the disease and prolong the time of recurrence. ② Treatment has significant impacts on the overall survival of patients. Chemotherapy can prolong the survival time of patients without recurrence. After pathological diagnosis, it is suggested that the treatment should be determined according to the involvement of organ system after PET-CT evaluation of systemic condition. ③ For patients whose first-line and second-line responses are not obvious, detecting BRAF-V600E gene mutations and choosing targeted drug therapy according to the results.

Key words: Langerhans cell histiocytosis, Clinical characteristics, BRAF-V600E, Prognosis

中图分类号:

R739.8

杨冉冉, 鞠侯雨, 任国欣, 郭伟. 36例口腔颌面-头颈部朗格汉斯细胞组织细胞增多症临床及预后分析[J]. 中国口腔颌面外科杂志, 2021, 19(1): 52-58.

YANG Ran-ran, JU Hou-yu, REN Guo-xin, GUO Wei. Clinical and prognostic analysis of Langerhans cell histiocytosis in oral maxillofacial head and neck: report of 36 consecutive cases[J]. China J Oral Maxillofac Surg, 2021, 19(1): 52-58.

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