含铁血黄素沉着性纤维脂肪瘤样肿瘤1例并文献复习

Haemosiderotic Fibrolipomatous Tumor:A Report of 1 Case and Literature Review

目的:观察含铁血黄素沉着性纤维脂肪瘤样肿瘤(haemosiderotic fibrolipomatous tumor,HFLT)临床病理特征。方法:回顾性分析1例HFLT的病理学形态、免疫表型并复习相关文献。结果:患者中年男性,右足内踝皮下肿块四十余年,时有疼痛,呈黑褐色。显微镜下表现为梭形细胞和脂肪组织密切混合,部分区域梭形细胞分隔单个脂肪细胞,包绕血管;巨噬细胞和梭形细胞内含铁血黄素沉积明显;伴淋巴细胞、浆细胞及少量肥大细胞浸润。免疫组化梭形细胞弥漫强表达CD34、Vimentin,不表达S-100、P63、HMB45、Melan-A、SOX10、Calponin、SMA、Desmin、CD31、CD68等标记。普鲁士蓝染色显示含铁血黄素呈蓝色。肿瘤完整切除后,采用自体皮肤移植,恢复良好,随访2年余,未见复发。复习文献可知,该肿瘤多见于中老年人,常见于踝和足部,易复发,应与弥漫型腱鞘巨细胞肿瘤、隆突性皮肤纤维肉瘤、丛状纤维组织细胞瘤、非典型脂肪瘤和梭形细胞脂肪瘤等肿瘤进行鉴别。结论:HFLT是一种生长缓慢、局部侵袭、无转移的中间型软组织肿瘤,治疗一般采用手术完整切除肿瘤且切缘阴性。正确诊断对选择适当的治疗措施具有重要临床意义。

Objective:To describe the clinicopathologic features of haemosiderotic fibrolipomatous tumor(HFLT).Methods:The morphology and immunoprofiles of HFLT were retrospectively analyzed,and relevant literature was reviewed.Results:A middle-aged man was presented with subcutaneous mass located on the medial ankle of right foot,and it had been presented for more than 40 years.It was painful sometimes,and dark-brown in color.It showed that the spindle cells and adipose tissue were closely mixed,and that the spindle cells separated the single fat cells and surrounded the blood vessels in some areas.Significant hemosiderin deposits were in macrophages and spindle cells,with infiltration of lymphocytes,plasma cells and a few of mast cells in the stroma.Immunohistochemically,the spindled cells were strong positive for CD34 and Vimentin,but were negative for S-100,P63,HMB45,Melan-A,SOX10,Calponin,SMA,Desmin,CD31 and CD68.Hemosiderin showed blue in Perls staining.The patient was treated with tumor resection followed by autologous skin transplantation.The patient was in good health without recurrence after followed-up for more than two years.This tumor is mostly common in middle-aged and elderly people,often in the ankle and foot,and is prone to recurrence.It should be differentiated from tumors such as diffuse giant cell tumor of the tendon sheath,derma-tofibrosarcoma protuberans,plexiform fibrohistiocytic tumor,atypical lipoma and spindle cell lipoma.Conclusion:Haemosiderotic fibrolipomatous tumour is a slow-growing,locally invasive,and non-metastatic intermediate soft tissue tumor.Complete resection with negative margin is required.Precise diagnosis is very important for choosing appropriate treatment.