长骨旺炽性反应性骨膜炎5例临床病理分析  

Clinicopathological features of florid reactive periostitis of the long bone

作　　者：谢乐[1] 莫超华 袁健祥[2] 李红玲 黄颖欣 毛荣军[1] XIE Le;MO Chaohua;YUAN Jianxiang;LI Hongling;HUANG Yingxin;MAO Rongjun(Department of Pathology,Foshan Hospital of Traditional Chinese Medicine,Foshan 528000,China;Department of Radiology,Foshan Hospital of Traditional Chinese Medicine,Foshan 528000,China)

机构地区：[1]广东省佛山市中医院病理科,佛山528200 [2]广东省佛山市中医院放射科,佛山528200

出　　处：《临床与实验病理学杂志》2023年第10期1236-1240,共5页Chinese Journal of Clinical and Experimental Pathology

基　　金：广东省佛山市十四五医学重点专科建设项目(FSZDZK 145010)。

摘　　要：目的探讨长骨旺炽性反应性骨膜炎(florid reactive periostitis,FRP)的临床病理学特征。方法回顾性分析5例长骨FRP的临床病理学特征、影像学表现、免疫表型及分子学特征等,行HE及免疫组化染色,FISH法检测基因突变情况,并复习相关文献。结果长骨FRP影像学表现为与骨膜相连的肿块影,可见程度不等的骨膜反应。镜下主要包含成熟程度不一的反应性新生骨和增生活跃的成纤维细胞两种成分,成纤维细胞表达vimentin、SMA等纤维母细胞、肌纤维母细胞标志物。Ki-67增殖指数为1%~4%。FISH检测可见USP6基因断裂重排。结论长骨FRP属罕见的良性皮质旁骨病变,以显著的骨膜反应和骨旁软组织纤维骨性病变为主要特征。由于鉴别诊断的可能性较大,特别是与骨旁骨肉瘤等恶性病变的鉴别,其正确诊断具有挑战性,需要全面的临床、影像和病理学评估。治疗方式包括抗炎休息、肿块完整切除等,需长期随访。Purpose To investigate the clinicopathologic features of florid reactive periostitis of(FRP)the long bone.Methods The clinicopathological features,imaging findings,immunophenotype and molecular characteristics of 5 cases of long bone FRP were analyzed retrospectively,HE and immunohistochemical staining were performed,gene mutation was detected by FISH,and the related literatures were reviewed.Results Long bone FRP radiographically appeated as a mass shadow connected to the periosteum with a variable degree of periosteum reaction.Microscopically,it mainly consisted of two components:reactive new bone with different degrees of maturity and fibroblasts with active proliferation.Fibroblasts expressed fibroblast and myofibroblast markers,such as vimentin and SMA,The proliferation index of Ki-67 ranged from 1%to 4%.Molecular detection showed the fracture and reorganization of USP6 gene.Conclusion Long bone FRP is a rare benign paracortical bone disease which characterized by significant periosteal reaction and paracortal soft tissue fibroskeletal lesions.Due to the high possibility of differential diagnosis,especially from malignant lesions such as parosteosarcoma,the correct diagnosis is challenging and requires comprehensive clinical,imaging,and pathological evaluation.The treatment methods include anti-inflammatory rest,complete resection,etc.,which requires long-term follow-up.

关 键 词：旺炽性反应性骨膜炎 长骨 临床病理特征 免疫组织化学 FISH 

分 类 号：R681[医药卫生—骨科学]