脊柱朗格汉斯细胞组织细胞增生症的临床病理分析  

Clinicopathologic analysis of langerhans cell histiocytosis of the spine

作　　者：熊文[1] 许霞 陈明 郑琼[1] 王明甫 Xiong Wen;Xu Xia;Chen Ming;Zheng Qiong;Wang Mingfu(Department of Orthopaedics,Wuhan Fourth Hospital,Puai Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wu Han 430000;Department of Pathology,Wuhan Union Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430022;Department of Radiology,The Third People‘s Hospital Of Hubei Province,Wuhan 430033,China)

机构地区：[1]武汉市第四医院骨科,华中科技大学同济医学院附属普爱医院,武汉430000 [2]华中科技大学同济医学院附属协和医院病理科,武汉430022 [3]湖北省第三人民医院放射科,武汉430033

出　　处：《中国组织化学与细胞化学杂志》2020年第4期339-344,共6页Chinese Journal of Histochemistry and Cytochemistry

基　　金：武汉市临床医学科研项目(WX17C14)。

摘　　要：目的探讨脊柱朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)的临床病理特征,分析BRAF V600E、CyclinD1及Ki-67在LCH中的表达情况及其意义。方法对27例脊柱LCH进行了临床和组织病理学的回顾性研究,以及免疫组化检测。结果本组27例均获得随访,临床资料齐全,其中男性20例,女性7例,患者的年龄5~62岁。21例为骨单发病灶,3例为骨多发病灶,3例为多系统病灶。颈椎13例,胸椎8例,腰椎6例,形成背部软组织肿块5例,临床表现为局部疼痛症状(27例),脊髓压迫症状(12例)及脊柱畸形(11例)。所有病例均表达CD1a、langerin、S-100;100%病例表达Cyclin D1,22.2%病例表达BRAF V600E,形成软组织包块5例Ki-67阳性率15%~20%,多系统病灶Ki-67阳性率15%~30%。采用ARMS法荧光定量PCR检测BRAF V600E,检出率为44.4%。结论脊柱LCH最常见的症状为疼痛,其次为脊髓压迫及脊柱畸形;cyclin D1可作为LCH的一个有效鉴别诊断指标;采用免疫组织化学染色检测BRAF V600E会使其检出率降低;Ki-67阳性率较高的病例更易发生周围组织的浸润。Objective To observe the clinicopathologic features of Langerhans cell histiocytosis(LCH)of the spine and to investigate the expression of BRAF V600E,CyclinD1 and Ki-67 protein in the spine LCH and its significance.Methods 27 cases of Langerhans cell histiocytosis of the spine were collected,The clinical and histopathological features were retrospectively studied and immunohistochemical staining was performed.Results In all 27 cases,20 were male and 7 were female.the ages of the patients ranged from 5 to 62 years old.Clinically,unisystem single-focal lesion of bone was predominant(21,77.8%),followed by unisystem multifocal lesion of bone(3,11.1%),multi-system disease(3,11.1%).The distribution of lesions revealed predominance in the cervical spine(13 cases),followed by thoracic spine(8 cases)and lumbosacral spine(6 cases).5 cases formed masses in the back soft tissue.The most common clinical manifestations of LCH of the spine was focal pain(27 cases),followed by spinal cord compression(12 cases)and deformity(11 cases).Immunohistochemically,100%(27/27)cases expressed CD1a,Langerin and S100 protein.The expression of BRAF V600E and cyclin D1 was 22.2%,and 100%,respectively.The positive rate of ki-67 in 5 cases with soft tissue mass formation was 15-20%,and the positive rate of ki-67 in multiple system lesions was 15-30%.BRAF V600E was detected by ARMS fluorescence quantitative PCR with a detection rate of 44.4%.Conclusion The most common clinical manifestation of LCH of the spine was pain,followed by spinal cord compression and deformity.Cyclin D1 can be used as an effective diagnostic indicator of LCH.The detection rate of BRAF V600E was decreased by using immunohistochemical staining.Cases with a higher positive rate of Ki-67 are more likely to invade the surrounding tissues.

关 键 词：LANGERHANS细胞 组织细胞增生症 Cyclin D1 KI-67 

分 类 号：R738.1[医药卫生—肿瘤]