软组织颗粒细胞瘤的临床特点  

Clinical features of granular cell tumor of Soft Tissue

作　　者：金韬[1] 刘巍峰[1] 郝林[1] 牛晓辉[1] JIN Tao;LIU Wei-feng;HAO Lin;NIU Xiao-hui(Department of Orthopedic Oncology Surgery,Beijing Jishuitan Hospital,Beijing,100035,China)

机构地区：[1]北京积水潭医院骨肿瘤科,100035

出　　处：《中国骨与关节杂志》2022年第5期339-344,共6页Chinese Journal of Bone and Joint

摘　　要：目的回顾分析10例软组织颗粒细胞瘤病例的临床特点,治疗与预后。方法2007年1月至2021年1月,我院骨肿瘤科经治软组织颗粒细胞瘤患者10例,患者年龄、性别、发病部位进行记录。所有患者术前完善MRI检查,后进行手术治疗,术后标本进行剖面分析切除边界。术后定期临床和影像学随访。结果本组10例,男4例,女6例;年龄33~71岁,平均46.5岁;肿瘤部位:下肢5例,上肢3例,躯干2例。10例获7~169个月的随访,平均68个月。本组10例中,8例病理为良性颗粒细胞瘤,1例为非典型颗粒细胞瘤,术后均未发现复发和转移。1例为恶性颗粒细胞瘤,术后31个月复发并进行截肢手术,现二次术后至今34个月,未见复发、转移。MRI是最重要的影像学检查,典型的MRI表现:与周围的肌肉相比,肿瘤在T_(1)相呈等信号、低信号,在T_(2)相呈等信号、高信号,在压脂T_(2)相上呈不均高低混杂信号,肿瘤周边可见水肿。典型病理表现为肿瘤有较薄包膜或无明显包膜,呈侵袭性生长,与周围组织边界不清,甚至侵犯周围正常组织。细胞大小较一致,胞浆较宽,细胞嗜酸性,颗粒状,伴有多少不等的单核、多核组织细胞样细胞和纤维细胞。肿瘤细胞呈巢状分布,间有多少不一的胶原纤维。肿瘤周围组织可有炎性反应,有淋巴细胞浸润。免疫组化:S-100及NSE阳性是其特点,CD68大部分可见阳性。Fanburg-Smith等所提的病理诊断标准可作为其良恶性的诊断参考。结论颗粒细胞瘤罕见,恶性颗粒细胞瘤更少见。基于颗粒细胞瘤的影像学特点,术前MRI可帮助诊断。病理诊断较难,免疫组化S-100及NSE阳性可帮助诊断。手术切除是主要的治疗方法,即使对于良性的颗粒细胞瘤,建议进行广泛切除。Objective To deepen the understanding and improve the diagnosis of Granular Cell Tumor in soft tissue by analyzing the epidemiological features,treatment and results.Methods The data of 10 patients with Granular Cell Tumor in soft tissue who were treated in our department from January 2007 to January 2021 were retrospectively studied.The age,gender of patients and location of tumor were recorded.All patients accepted a MRI examination before operation,gross specimens were analyzed to record the resection margin.All patients were followed up after surgery.Results There were 4 males and 6 females patients with mean age of 46.5(33-71)years.5 patients with tumor in lower extremities,3 in upper extremities and 2 in trunk.The median followup was 68 m(7-169 m)for all patients.8 patients were diagnosed as begin and 1 was atypical,who had no local recurrence or distant metastasis,whereas one patient who was diagnosed as malignant had a local recurrence 31 months after operation and accepted amputation,no relapse at the latest follow up.Preoperative MRI is the most important examination.A signal isointense or slight lower signal to the surrounding muscle on T1-weighted sequences and lower signal intensity than fat but slightly higher than muscle on T_(2)-weighted sequences was seen in most patients.All tumors had heterogeneous signal and high-intensity signal peripherally on this sequence.The typical pathological manifestations were tumor invasive growth,unclear boundary with surrounding tissues,and even invasion of surrounding normal tissues.The tumors had a thin capsule or no obvious capsule with a consistent cell size.The cells were eosinophilic and granular,the cytoplasm is wide.All tumors had varying numbers of mono nuclear and multinuclear histiocyte like cells and fibroblasts.The tumor cells were distributed in nests with varying amounts of collagen fibers.The tissues around the tumor may had inflammatory reaction and lymphocyte infiltration.Immunohistochemistry:S-100 and NSE were positive in all patients and CD68 w

关 键 词：颗粒细胞瘤 软组织肿瘤 肿瘤 磁共振成像 组织学类型肿瘤 

分 类 号：R738.6[医药卫生—肿瘤]