伴有纤维肉瘤变的隆突性皮肤纤维肉瘤12例临床病理分析  被引量:6

Fibrosarcomatous dermatofibrosarcoma protuberans: a clinicopathological analysis of 12 cases

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作  者:张锦[1] 吴楠[1] 夏春[1] 魏雪[1] 石群立[1] 周晓军[1] 马捷[1] 

机构地区:[1]南京大学医学院临床学院南京军区南京总医院病理科,210002

出  处:《中华病理学杂志》2013年第11期753-757,共5页Chinese Journal of Pathology

基  金:国家自然科学基金(81171391);中国博士后科学基金面上资助(201150M1575);中国博士后科学基金特别资助(2012T50895)

摘  要:目的 探讨伴有纤维肉瘤变的隆突性皮肤纤维肉瘤(FS-DFSP)的临床病理学特征.方法 对1997年1月至2011年12月收集的12例FS-DFSP进行形态学观察,免疫组织化学EnVision法标记,并结合患者的临床资料及相关文献进行讨论.结果 12例患者,男10例,女2例,平均年龄53岁(41 ~70岁).临床表现为躯干和四肢皮下缓慢性生长的无痛性肿块,其中7例短期内肿块迅速增大,并伴有隐痛.3例为原发性,9例为复发性.镜下表现为经典的DFSP中出现纤维肉瘤样(FS)改变,且FS的区域超过肿瘤的5%.与经典的DFSP不同,FS区域内瘤细胞密度增高,常呈长束状、人字形或鱼骨样排列,而席纹状结构已不明显或完全消失,瘤细胞核异型性较明显,核分裂象(6~25)/10 HPF.免疫组织化学结果显示经典的DFSP区域瘤细胞CD34弥漫强阳性,而FS区域瘤细胞CD34明显减弱.FS区域Ki-67阳性指数约10% ~40%,显著高于经典DFSP区域的2% ~5%.12例患者均行手术切除治疗,并有2例行术后辅助放疗.9例获得随访,时间9 ~86个月,6例局部复发,2例患者发生转移,1例患者于术后9个月死亡.结论 FS-DFSP是一种罕见而独特的DFSP亚型,与潜在低度恶性/交界恶性的经典DFSP不同,FS-DFSP在临床、病理和生物学行为上均表现为明确的恶性肿瘤特点,具有较高的复发率及转移率.Objective To investigate the clinical pathological features of fibrosarcomatous dermatofibrosarcoma protuberans (FS-DFSP).Methods The clinical history,histopathological features and immunohistochemical characteristics were analyzed in twelve cases of FS-DFSP from January 1997 to February 2011,and related literature were reviewed.Results Age of the patients (2 females,10 males) at diagnosis ranged from 41 to 70 years (mean 53 years).Among the 12 cases of FS-DFSP,9 cases aroused in recurrent ordinary DFSP.Histologically,FS areas in FS-DFSP were characterized by a fascicular and highly cellular histology,frequently showing a characteristic herringbone pattern.FS-DFSP showed diminishment of CD34 staining in FS areas.The labeling index of Ki-67 was much higher in the FS areas (10%-40%) than that in the conventional DFSP areas (2%-5%).All the patients were treated by operation with local excision or wide excision.Postoperative radiotherapy and chemotherapy was administered in two cases respectively.Follow-up information in 9 of 12 patients (9 to 86 months) revealed local recurrence in 6 patients.Distant metastases were seen in two patients.One patient was died in the follow up period.Conclusions FS-DFSP is a rare and unique subtype of DFSP and is associated with significant elevated risk of both local and distance metastasis,usually followed by poor outcome.Compared to ordinary DFSP as a borderline neoplasm,FS-DFSP should be considered as a malignant tumor.

关 键 词:皮肤纤维肉瘤 诊断 鉴别 免疫组织化学 

分 类 号:R739.5[医药卫生—肿瘤]

 

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