摘要目的 探讨非典型性纤维组织细胞瘤(AFH)的临床病理学特征及其鉴别诊断.方法 回顾性复习2007至2012年间收集的24例AFH临床资料、镜下形态和免疫学表型特点,分析预后资料.免疫组织化学标记采用EnVision法.结果 男性10例,女性14例,平均年龄和中位年龄分别为41岁和39岁(范围8～67岁).肿瘤发生于四肢(14例)、躯干(8例)和头颈部(2例).除1例位于黏膜下外,其他肿瘤均位于真皮层内,基本形态与经典的纤维组织细胞瘤相似,由短交织状或席纹状排列的原始间叶性细胞或纤维母细胞样细胞组成,局部可见泡沫样组织细胞或含铁血黄素性吞噬细胞,特征性形态表现为肿瘤内可见散在分布的核深染畸形细胞,呈胖梭形、多边形或多核样.部分病例内可见核分裂象,特别是在细胞丰富的区域内,偶见病理性核分裂象.免疫组织化学标记显示,AFH以表达波形蛋白为主,部分病例可灶性表达平滑肌肌动蛋白.随访14例,3例发生局部复发,无病例发生转移.结论 AFH是纤维组织细胞瘤的一种特殊亚型,尽管在组织学上显示不典型性,但临床上经完整切除后多呈良性经过,少数病例可发生局部复发.AFH易与非典型性纤维黄色瘤相混淆,应注意加以鉴别.

abstractsObjective To study the clinicopathologic characteristics of atypical fibrous histiocytoma (AFH),with emphasis on differential diagnosis.Methods The clinical and pathologic features were reviewed in 24 cases of AFH (from 2007 to 2012).The follow-up data were analyzed.Immunohistochemical study using EnVision method was carried out.Results There were 10 males and 14 females with age at presentation ranging from 8 to 67 years (mean =41 years and median =39 years).The tumor occurred in the extremities (number =14),trunk (number =8) or head and neck region (number =2).Apart from one case,all were located in the dermis.The clinical appearance was similar to those of classic fibrous histiocytoma.Histologically,the tumor was characterized by various number of hyperchromatic bizarre cells scattered in the background.Mitotic figures including atypical ones were noted,especially in the more cellular areas.Immunohistochemical study showed that the tumor cells expressed vimentin.Focal positivity for alpha-smooth muscle actin was demonstrated in some cases.Follow-up information was available in 14 cases.Three of them suffered local recurrence.None of these cases had distant metastasis.Conclusions Atypical fibrous histiocytoma represents a pleomorphic variant of fibrous histiocytoma.Although the tumor exhibits worrisome features,it usually pursuits a relatively benign course.Nevertheless,rare cases may recur,especially after incomplete excision.AFH is sometimes mistaken as atypical fibroxanthoma.A distinction between the two entities is warranted as they represent two different entities.