富于细胞性纤维组织细胞瘤的临床病理分析

Cellular fibrous histiocytoma： a clinicopathologic analysis of 27 cases

目的探讨富于细胞性纤维组织细胞瘤（CFH）的临床病理学特征、诊断和鉴别诊断。方法回顾性复习2008至2012年27例CFH的临床资料、光镜形态和免疫学表型，分析预后资料。免疫组织化学染色采用EnVision法。结果男性13例，女性14例，平均年龄和中位年龄分别为34岁和32岁（范围15—61岁）。肿瘤发生于四肢（14例）、头颈部（7例）和躯干（6例）。镜下示肿瘤位于真皮层内，部分病例呈楔状伸入浅表皮下脂肪组织。瘤细胞成分较为单一，主要由致密增生的梭形至胖梭形纤维母细胞和肌纤维母细胞组成，其他细胞成分包括含铁血黄素性吞噬细胞、泡沫样组织细胞或杜顿巨细胞等均较少见。梭形瘤细胞多呈交织的条束状排列，部分病例内可呈席纹状，少数病例内还可见血管外皮瘤样结构。瘤细胞异型性不明显，但可见多少不等的核分裂象，少数病例内还可见局灶性坏死。免疫组织化学染色显示，瘤细胞可表达平滑肌肌动蛋白，但表达程度不一，常为灶性阳性。部分病例还可灶性表达CD34，但多分布于病变的周边或基底部。瘤细胞不表达结蛋白、高分子量钙调蛋白、S-100蛋白和细胞角蛋白。病变内少量的组织细胞表达KP-1。随访19例，5例发生局部复发，1例在多次局部复发后发生双肺转移。结论CFH是良性纤维组织细胞瘤的一种特殊亚型，切除不净可发生局部复发，极少数病例可发生区域淋巴结或肺转移。组织学形态不能评估局部复发或远处转移的危险性。熟悉CFH的临床病理形态学特点及其免疫表型可避免将其误诊为隆突性皮纤维肉瘤等恶性肿瘤。

Objective To study the clinicopathologic characteristics of cellular fibrous histiocytoma （CFH） with emphasis on diagnosis and differential diagnosis. Methods Clinical and pathologic features were reviewed in 27 cases of CFH （encountered during the period from 2008 to 2012） along with outcome analysis. Immunophenotyping was performed with EnVision method. Results The patients included 13 males and 14 females. The age at presentation ranged from 15 to 61 years （mean, 34 years; median, 32 years）. The tumor occurred in the extremities （n = 14）, head and neck （n =7）, and trunk （n =6）. Histologically, the tumors were located in the dermis. Some cases showed wedge like extension into the subcutaneous adipose tissue. On high power, they consisted of dense fibroblasts and myofibroblasts. Other cell components such as psammoma-like histiocytes, hemosiderin-containing macrophages or touton-type giant ceils were rare. The spindled tumor cells were arranged mostly in intersecting fascicles. Focal storiform architecture was not uncommon. In addition, a few cases showed prominent hemangiopericytoma-like pattern. There was no prominent cellular atypia but increased mitotic figures were not difficult to find. Two cases exhibited necrosis. By immunohistochemistry, the tumor cells showed variable expression of alpha smooth muscle actin. CD34 positive ceils were present in some cases, but were distributed mostly in the periphery or bottom of the lesions. They were all negative for desmin, h-caldesmon, S-100 protein and cytokeratin. Follow-up in 19 cases revealed local recurrences in 5 cases and bilateral pulmonary metastases in 1 case after repeated recurrences. Conclusions CFH is a cellular form of benign fibrous histiocytomawhich has a risk for local recurrence after incomplete excision. Distant metastasis can occur in rare examples. However, there were no morphological parameters predicting the risk Of recurrence or metastasis. Increased awareness of the clinocopathological features and immunophenotypes of CFH is helpful in avoiding misdiagnosing the disease as malignant tumors, especially derrnatofibrosarcoma protuberans.