Zhong Yan-ping, Wang Jian
Department of Pathology, Fudan University, Shanghai, China.
Zhonghua Bing Li Xue Za Zhi. 2013 Mar;42(3):153-7. doi: 10.3760/cma.j.issn.0529-5807.2013.03.003.
To study the clinicopathologic characteristics of cellular fibrous histiocytoma (CFH) with emphasis on diagnosis and differential diagnosis.
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.
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 cells 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 cells 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.
CFH is a cellular form of benign fibrous histiocytoma which 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 dermatofibrosarcoma protuberans.
研究细胞性纤维组织细胞瘤(CFH)的临床病理特征,重点关注其诊断及鉴别诊断。
回顾性分析2008年至2012年间收治的27例CFH患者的临床及病理特征,并进行预后分析。采用EnVision法进行免疫表型分析。
患者中男性13例,女性14例。发病年龄为15至61岁(平均34岁,中位数32岁)。肿瘤发生于四肢(14例)、头颈部(7例)及躯干(6例)。组织学上,肿瘤位于真皮层。部分病例呈楔形延伸至皮下脂肪组织。高倍镜下,肿瘤由密集的成纤维细胞和肌成纤维细胞组成。其他细胞成分如砂粒体样组织细胞、含铁血黄素巨噬细胞或 Touton 型巨细胞少见。梭形肿瘤细胞大多呈交错束状排列。局灶性席纹状结构并不少见。此外,少数病例表现出明显的血管外皮瘤样模式。未见明显细胞异型性,但易见核分裂象增多。2例出现坏死。免疫组化显示,肿瘤细胞α平滑肌肌动蛋白表达各异。部分病例CD34阳性细胞存在,但大多分布于病变周边或底部。所有病例结蛋白、h-钙调蛋白、S-100蛋白及细胞角蛋白均为阴性。19例随访结果显示,5例局部复发,1例反复复发后出现双侧肺转移。
CFH是良性纤维组织细胞瘤的一种细胞形式,不完全切除后有局部复发风险。罕见情况下可发生远处转移。然而,尚无形态学参数可预测复发或转移风险。提高对CFH临床病理特征及免疫表型的认识有助于避免将该病误诊为恶性肿瘤,尤其是隆突性皮肤纤维肉瘤。