放射治疗头面部朗罕斯细胞组织细胞增生症9例疗效分析

Clinical analysis of the radiotherapy for Langerhans cell histiocytosis in the head and neck region

背景与目的:朗罕斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)是一种罕见的网状内皮组织疾病,常累及头面部。治疗手段尚无统一标准,但认为放射治疗可用于治疗局限性病灶。本研究旨在探讨放射治疗LCH的疗效。方法:对2000年7月—2007年5月期间本院放疗科收治的经病理证实的8例嗜酸性肉芽肿和1例韩-薛-柯病进行临床回顾性分析。结果:嗜酸性肉芽肿5例患者行肿块部分切除和放射治疗,3例活检后单纯放射治疗,韩-薛-柯病1例进行手术、化疗、再程放疗和内分泌治疗,所有病例随访1.5～8年,总有效率为89%。结论:朗罕斯细胞组织细胞增生症采用放射治疗具有良好的治疗效果,但放射剂量尚需进一步研究。

Background and purpose： Langerhans cell histiocytosis （LCH） is a rare complex reticuloendothelial disease that often invades the head and neck. There in no consensus of treatment. Radiation is one of the treatment options for the localized lesions. Our aim in the study was to analyze the radiotherapeutic efficacy for LCH in the head and neck region. Methods： 8 patients with eosinophilic granuloma （EG） and 1 case with Hand-SchullerChristian disease （HSC） were treated with radiotherapy in our hospital from July 2000 to May 2007. Their clinical data were retrospectively analyzed. Results： 5 cases of EG were treated with tumor partial excision and radiation, while the other 3 cases of EG were given radiation alone after biopsy. The HSC was administered with radiation and endocrine therapy. All the patients were followed up for 1.5-8 years. The response rate was 89%. Conclusion： Radiotherapy of LCH is an effective modality, but the optimal dose needs to be further studied.