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目的:探讨口腔颌面部朗格汉斯细胞组织细胞增生症的临床特点及治疗方法。方法:采用病例回顾性研究方法,对30例口腔颌面部朗格汉斯细胞组织细胞增生症病例的临床表现、治疗和预后进行分析。以1997年WHO的临床分型为依据,采用以切除术或刮治术为主的治疗方法,病变累及范围较广者,辅以放疗和(或)化疗。对12例患者的随访结果进行分析。结果:男18例,女12例,中位年龄8岁;66.7%的病例病变累及下颌骨。影像学主要表现为骨组织破坏和软组织侵犯。对病变局限的14例患者采用单纯手术治疗;病变范围较广、累及骨外软组织的6例患者,采用手术加术后放疗;对有明显全身症状的5例患者,采用手术配合化疗。随访的12例患者中,无病生存10例。结论:口腔颌面部朗格汉斯细胞组织细胞增生症有一定临床特点,采取相应的治疗方法,预后较好。
Objective: To investigate the clinical features and treatment of Langerhans cell histiocytosis in oral and maxillofacial region. Methods: The clinical manifestations, treatment and prognosis of 30 cases of Langerhans cell histiocytosis in oral and maxillofacial region were analyzed by case retrospective study. Based on the clinical classification of WHO in 1997, the treatment method based on resection or curettage was used, and the patients with extensive lesions were treated with radiotherapy and / or chemotherapy. Follow-up results of 12 patients were analyzed. Results: There were 18 males and 12 females with a median age of 8 years. 66.7% of the cases involved the mandible. Imaging mainly manifested as bone tissue damage and soft tissue invasion. Fourteen patients with localized lesions were treated with surgery alone; six patients with extensive lesions involving extra-articular soft tissue were treated with surgery and postoperative radiotherapy; and five patients with significant systemic symptoms were treated with surgery and chemotherapy. Among the 12 patients who were followed up, 10 cases were disease-free. Conclusion: Oral and maxillofacial Langerhans cell histiocytosis has some clinical features, take the appropriate treatment, the prognosis is good.