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成人期胸骨朗格汉斯细胞组织细胞增多症

Adult-onset Langerhans cell histiocytosis of the sternum.

作者信息

Huang Yi-Jhih, Lin Kuan-Hsun, Chao Tai-Kuang, Huang Tsai-Wang, Huang Hsu-Kai

机构信息

Division of Thoracic Surgery, Department of Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.

Department of Pathology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.

出版信息

J Thorac Dis. 2017 Sep;9(9):E783-E786. doi: 10.21037/jtd.2017.08.51.

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease with uncertain etiology that is more prevalent in children. LCH typically invades skeletal systems, but in rare cases, it has been reported in the ribs or sternum. Optimal treatment choices for single-site, skeletal LCH are still undefined. We report a case of adult-onset LCH of the sternum. The range of surrounding soft tissue invasion was confirmed by three-dimensional fusion and reconstruction of chest computed tomography and magnetic resonance images. Our patient was successfully treated by local surgical curettage and adjuvant radiation therapy. We concluded that postoperative adjuvant radiation therapy may be advantageous for single-site LCH of bones with soft tissue invasion.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种病因不明的罕见疾病,在儿童中更为常见。LCH通常侵犯骨骼系统,但在罕见情况下,也有在肋骨或胸骨中出现的报道。单部位骨骼LCH的最佳治疗选择仍不明确。我们报告一例成人胸骨LCH病例。通过胸部计算机断层扫描和磁共振图像的三维融合与重建确定了周围软组织的侵犯范围。我们的患者通过局部手术刮除和辅助放疗成功治愈。我们得出结论,术后辅助放疗可能对伴有软组织侵犯的单部位骨骼LCH有益。

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