Cesinaro Anna Maria, Mataca Ema, Gambini Claudio, Kutzner Heinz
Department of Anatomic Pathology, Azienda Ospedaliero-Universitaria, Modena, Italy.
Department of Anatomic Pathology, Clinica Montallegro, Genua, Italy.
J Cutan Pathol. 2016 Jul;43(7):589-593. doi: 10.1111/cup.12688. Epub 2016 Apr 28.
Dermatofibrosarcoma protuberans (DFSP) is a low grade, superficial sarcoma characterized by a proliferation of monomorphous, spindle cells arranged in a storiform pattern and infiltrating the subcutaneous tissue. The tumor is typically CD34 positive, and shows the characteristic COL1A1-PDGFB fusion gene, detectable either by florescent in situ hybridization (FISH) and polymerase chain reaction (PCR). We describe a case of DFSP with a focus of peculiar pleomorphic sarcomatous transformation. The focus constituted the entire bioptic tissue that was initially excised, raising considerable diagnostic problems for pathologist. The use of FISH as an ancillary technique allowed the right diagnosis.
隆突性皮肤纤维肉瘤（DFSP）是一种低级别浅表性肉瘤，其特征为单形性梭形细胞呈席纹状排列并浸润皮下组织。该肿瘤通常CD34阳性，并显示特征性的COL1A1-PDGFB融合基因，可通过荧光原位杂交（FISH）和聚合酶链反应（PCR）检测到。我们描述了一例伴有特殊多形性肉瘤转化灶的DFSP病例。该病灶构成了最初切除的全部活检组织，给病理学家带来了相当大的诊断难题。使用FISH作为辅助技术得以做出正确诊断。
