Andres C, Puchta U, Flaig M J
Department of Dermatology and Allergology, Ludwig-Maximilians-Universität, Munich, Germany.
Am J Dermatopathol. 2010 Dec;32(8):799-803. doi: 10.1097/DAD.0b013e3181dfcdff.
A clear etiopathogenetic concept for atypical fibroxanthoma (AFX) is not established yet. Nevertheless, AFX is known as a pleomorphic but indolent tumor primarily of the elderly and/or immunosuppressed patient occurring in severely sun- or radiation-damaged skin. These risk factors are almost identical to those of Merkel cell carcinoma (MCC), a highly malignant skin tumor being thought to be pathogenetically associated with the recently discovered Merkel cell polyomavirus (MCPyV). Because AFX and MCC share risk factors, the aim of this study was to evaluate presence of MCPyV DNA in 23 cases of AFX by PCR and direct DNA sequencing. Subsequently, we correlated clinical features with MCPyV DNA status in AFX. We detected MCPyV DNA in 4 of 23 AFX. All patients with MCPyV DNA-positive tumors were men. The mean age of patients with MCPyV DNA-positive AFX was 84.8 ± 8.7 years (vs. 75.2 ± 7.8 years of MCPyV DNA-negative AFX), the mean duration of tumor growth was 4.5 ± 2.3 months (vs. 5.1 ± 2.8 months) and the mean tumor diameter was 1.2 ± 0.3 cm (vs. 1.3 ± 0.7 cm). Ulceration was present in 75% of MCPyV DNA-positive tumors (vs. 65.2%). In conclusion, MCPyV DNA is present in 17% of AFX, in this cohort affecting predominantly male patients with higher age (>80 years). Clinical features seem to be independent of MCPyV DNA status. Although the role of MCPyV is unclear in this setting, it may act as a cofactor in the tumorigenesis of AFX in a subset of cases.
非典型纤维黄色瘤(AFX)的明确病因发病机制概念尚未确立。然而,AFX是一种多形性但生长缓慢的肿瘤,主要发生于老年人和/或免疫抑制患者,见于严重日晒或辐射损伤的皮肤。这些危险因素与默克尔细胞癌(MCC)几乎相同,MCC是一种高度恶性的皮肤肿瘤,被认为在发病机制上与最近发现的默克尔细胞多瘤病毒(MCPyV)相关。由于AFX和MCC有共同的危险因素,本研究的目的是通过聚合酶链反应(PCR)和直接DNA测序评估23例AFX中MCPyV DNA的存在情况。随后,我们将AFX的临床特征与MCPyV DNA状态进行了关联分析。我们在23例AFX中的4例中检测到了MCPyV DNA。所有MCPyV DNA阳性肿瘤患者均为男性。MCPyV DNA阳性AFX患者的平均年龄为84.8±8.7岁(MCPyV DNA阴性AFX患者为75.2±7.8岁),肿瘤生长的平均持续时间为4.5±2.3个月(MCPyV DNA阴性AFX患者为5.1±2.8个月),平均肿瘤直径为1.2±0.3 cm(MCPyV DNA阴性AFX患者为1.3±0.7 cm)。75%的MCPyV DNA阳性肿瘤存在溃疡(MCPyV DNA阴性AFX患者为65.2%)。总之,在该队列中,17%的AFX存在MCPyV DNA,主要影响年龄较大(>80岁)的男性患者。临床特征似乎与MCPyV DNA状态无关。虽然在这种情况下MCPyV的作用尚不清楚,但在一部分病例中它可能在AFX的肿瘤发生中作为一个辅助因子起作用。