Tantcheva-Poor Iliana, Marathovouniotis Nicos, Kutzner Heinz, Mentzel Thomas
Department of Dermatology, University of Cologne, Cologne, Germany.
Am J Dermatopathol. 2012 Jun;34(4):e46-9. doi: 10.1097/DAD.0b013e318234e704.
We describe a case of congenital dermatofibrosarcoma protuberans (DFSP) that masqueraded as a vascular tumor both clinically and histologically. Based on the infiltrative growth pattern, presence of capillary-sized vessels, and spindle cell areas with slit-like vascular spaces and numerous thin-walled vessels at the periphery of the tumor, a kaposiform hemangioendothelioma was initially diagnosed. Strong diffuse CD34 positivity and the extension into the subcutaneous fat with a sieve-like effect prompted the fluorescence in situ hybridization analysis, which demonstrated a reciprocal t(17;22) translocation. According to our knowledge, this is the first report of a vascular histological variant of DFSP. This unique variant represents a potential pitfall for dermatopathologists and underlines the importance of cytogenetic diagnostics in unusual cases of DFSP.
我们描述了一例先天性隆突性皮肤纤维肉瘤（DFSP），该病例在临床和组织学上均伪装成血管肿瘤。基于浸润性生长模式、毛细血管大小血管的存在以及肿瘤周边具有裂隙样血管腔隙和众多薄壁血管的梭形细胞区域，最初诊断为卡波西样血管内皮瘤。弥漫性强CD34阳性以及向皮下脂肪呈筛状延伸促使进行荧光原位杂交分析，结果显示存在相互的t(17;22)易位。据我们所知，这是DFSP血管组织学变异型的首例报告。这种独特的变异型是皮肤病理学家可能面临的一个潜在陷阱，并强调了细胞遗传学诊断在DFSP不寻常病例中的重要性。
