血管瘤样纤维组织细胞瘤病理诊断与鉴别诊断

Angiomatoid fibrous histiocytoma： report of 5 cases with review of literature

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1．9am，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。

Objective To study the clinicopathologic features, immunophenotype and differential diagnosis of angiomatoid fibrous histiocytoma （AFH）. Methods The clinicopathologic 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 showed 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 pseudocapsule 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 CI）68. Two cases were epithelial membrane antigen and CD99-positive. Conclusions AFH is a rare tumor of intermediate malignant potential. Definitive diagnosis requires thorough histolagic 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.