摘要目的 探讨血管瘤样纤维组织细胞瘤(angiomatoid fibrous histiocytoma)的临床病理特点、免疫表型、病理诊断与鉴别诊断要点.方法 收集5例血管瘤样纤维组织细胞瘤,分析其临床特点,观察病理组织学形态及免疫表型特征,并复习相关文献.结果 临床特点:5例患者中男性3例,女性2例,平均年龄21.4岁,平均病程约13个月.主要表现为真皮深层或皮下缓慢生长的无痛性肿块,3例位于头颈部,1例位于肘部,1例位于足部,均行完整切除,术后未予放疗和化疗等辅助治疗,随访10～29个月,无一例复发和转移.病理检查:5例均为单发肿块,瘤体最大径平均1.9 cm,周界清楚,切面呈灰褐色,质实,较硬,有不规则出血性囊腔.镜下观察:瘤细胞主要呈组织细胞样、梭形,略呈结节状分布,5例均有假血管样腔隙,3例有较厚纤维性假包膜及周围淋巴细胞浆细胞浸润.免疫组织化学:5例均表达波形蛋白,3例表达结蛋白,3例表达CD68,2例表达上皮细胞膜抗原,2例表达CD99,5例均不表达S-100蛋白、CKpan、CD34和CD31.结论 血管瘤样纤维组织细胞瘤是一种罕见的交界性肿瘤,其诊断主要依靠病理形态学特征,免疫组织化学标记有助于诊断和鉴别诊断;局部适当扩大切除,术后随访患者是主要处理原则.

abstractsObjective To study the clinicopathologic features,immunophenotype and differential diagnosis of angiomatoid fibrous histiocytoma(AFH).Methods The clinicepathologic features of 5 cases of AFH were analyzed.Immunohistochemical study was carried out and the literature was reviewed.Results There were a total of 3 males and 2 females.The average age of patients was 21.4 years old.The average duration of symptoms was 13 months.The patients primarily presented with a slowly enlarging painless deep dermal or subcutaneous mass.The mass was located in the head and neck region in 3 cases,elbow in 1 case and foot in 1 case.The patients underwent complete resection of the tumor,with no adjuvant chemotherapy and/or radiotherapy given.During a period of follow up for 10 to 29 months,all of them had no recurrence or distant metastasis.Gross examination shewed that the tumor was well-circumscribed and had a greycolored cut surface,with focal hemorrhagic cystic changes.The average tumor dimension was 1.9 cm.Histologically,the tumor was composed of histiocytoid or spindly cells arranged in nodular pattern.Fibrillary neuropil-type intercellular material was identified in all cases and a fibrous pseudocapsulc surrounded by lymphocytes and plasma cells was demonstrated in 3 cases.Immunohistochemical study showed that all of them were positive for vimentin and negative for S-100 protein,pan-cytokeratin,CD34 and CD31.Three of the cases expressed desmin and CD68.Two cases were epithelial membrane antigen and CD99-pesitive.Conclusions AFH is a rare tumor of intermediate malignant potential.Definitive diagnosis requires thorough histologic examination and clinical correlation.Immunohistochemistry is also helpful for diagnosis and differential diagnosis.Wide local excision with post-operative follow up is the main modality of treatment.