朗格汉斯细胞组织细胞增多症表现为下颌骨的单一嗜酸性肉芽肿:一个罕见的报告

P. Shah, S. Mukherji, B. Hathiram, Vicky Khatter
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引用次数: 1

摘要

朗格汉斯细胞组织细胞增生症(LCH)是一种相对罕见和独特的良性疾病,其特征是未成熟树突状细胞的异常增殖,通常发生在儿童和年轻人身上。只有不到10%的儿童患有这种疾病。目的:在文献报道的基础上,增加一例罕见且独特的骨肿瘤病例,并探讨其口腔表现及治疗方法。地点:私立颌面外科研究所,加尔各答,西孟加拉邦。患者:男,2岁8个月大,左下颚单发肿胀1个半月来。病变固定于下骨,下颌骨体部颊皮质板扩张。结果:在评估病史、临床和放射学细节并考虑患者年龄后,对病变进行了去核。术后活检结果显示LCH表现为单灶性嗜酸性肉芽肿。适当的手术干预使患者的临床状况得到显著改善。目前,患者无病无症状。结论:手术摘除或刮除是治疗上颌、下颌骨病变的首选方法。由于这种疾病是不可预测的,因此不应低估单灶性疾病发展为多灶性疾病的可能性。由于病情的不确定性,我们建议长期随访。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Langerhans Cell Histiocytosis Presenting as a Unifocal Eosinophilic Granuloma in the Body of the Mandible: A Rare Report
Introduction: Langerhans cell histiocytosis (LCH) is a relatively rare and unique benign disease characterized by an abnormal proliferation of immature dendritic cells which usually affects children and young adults. Jaws are involved in less than 10% of children with the disease. Objective: To add on to the literature one more case of this rare and unique disease which accounts for less than 1% of all bone tumors and also to discuss its oral manifestation and management. Setting: Private maxillofacial surgery institute, Kolkata, West Bengal. Patient: A 2 years and 8 months old male child with solitary swelling over the lower left jaw since one and half months. Lesion was fixed to underlying bone and there was an expan­ sion of buccal cortical plate at the body of mandible. Results: Enucleation of the lesion performed after assessing history, clinical and radiological dertails and also considering patient’s age. Postoperative biopsy result came as LCH pre­ senting as a unifocal eosinophil granuloma. Appropriate surgical inter vention resulted in a dramatic improvement in patient’s clinical condition. At present, patient is disease­free and asymptomatic. Conclusion: Surgical enucleation or curettage is a preferred treatment option for maxillary and mandibular lesion. Potential for the unifocal disease to become multifocal should not be under estimated as the disease can be unpredictable. We recom­ mend long­term follow­up due to uncertainty of the disease.
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