Hanft V N, Shea C R, McNutt N S, Pullitzer D, Horenstein M G, Prieto V G
Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA.
Am J Dermatopathol. 2000 Feb;22(1):17-21. doi: 10.1097/00000372-200002000-00003.
CD34 antigen is expressed in normal human skin on endothelium, in spindle cells located around adnexal structures, and in a subset of interstitial cells in the reticular dermis. CD34 expression has also been identified in a number of fibrohistiocytic neoplasms, such as dermatofibrosarcoma protuberans and solitary fibrous tumors of soft tissue. CD34 expression has not previously been described in sclerotic, or "plywood" fibromas. Here presented are three lesions from three patients, in which histologic examination revealed a well-circumscribed dermal nodule composed of spindled cells with focal nuclear pseudo-inclusions. There was extensive fibrosis with hypocellular, storiform areas, characteristic of sclerotic fibroma. The spindled cells strongly expressed CD34, but not factor XIIIa or markers of melanocytic, neural, or muscular differentiation. A diagnosis of Cowden syndrome was considered in one of the cases. These cases provide evidence that CD34 expression can occur in sclerotic fibromas, either solitary or associated with Cowden syndrome. When diagnosing a sclerotic fibroma, one should comment in the report regarding the possibility of Cowden syndrome.
CD34抗原在正常人类皮肤的内皮细胞、附属器结构周围的梭形细胞以及网状真皮中的一部分间质细胞中表达。CD34表达也已在一些纤维组织细胞瘤中得到确认，如隆突性皮肤纤维肉瘤和软组织孤立性纤维瘤。硬化性或“胶合板样”纤维瘤中此前尚未有CD34表达的描述。本文展示了来自三名患者的三个病变，其中组织学检查显示一个边界清楚的真皮结节，由具有局灶性核假包涵体的梭形细胞组成。存在广泛纤维化，伴有细胞稀少的席纹状区域，这是硬化性纤维瘤的特征。梭形细胞强烈表达CD34，但不表达因子ⅩⅢa或黑素细胞、神经或肌肉分化的标志物。其中一例考虑诊断为考登综合征。这些病例提供了证据，表明CD34表达可出现在硬化性纤维瘤中，可为孤立性或与考登综合征相关。在诊断硬化性纤维瘤时，应在报告中提及考登综合征的可能性。
