Diaz-Cascajo C, Weyers W, Rey-Lopez A, Borghi S
Center for Dermatopathology Freiburg, Germany.
Histopathology. 1998 Jun;32(6):552-5.
Dermatofibrosarcoma protuberans (DFSP) is a distinctive cutaneous spindle cell neoplasm that invariably infiltrates the subcutaneous tissue. Other reports have suggested that exceptional cases of DFSP may be confined to the subcutaneous tissue and lack dermal involvement. We wish to confirm this observation by describing cases of a rare variant of DFSP confined to the subcutaneous tissue, and analyse possible histogenetic implications.
Three cases of DFSP located in the subcutaneous tissue are reported. Multiple step sections demonstrated the lack of dermal involvement in two of them, whereas the third case infiltrated the dermis at the junction with the subcutis minimally in one of five blocks. Immunohistochemical studies using a battery of monoclonal antibodies were performed. All the tumours stained strongly for vimentin and CD34.
Because of the lack of dermal involvement in two cases and only minimal dermal involvement in one case, we called this variant deep DFSP. Except for deep setting of the tumour, deep DFSP is indistinguishable from typical DFSP clinically, histologically and immunohistochemically. The existence of deep DFSP provides evidence that specific structures of the skin may be not involved in this tumour's histogenesis.
隆突性皮肤纤维肉瘤（DFSP）是一种独特的皮肤梭形细胞瘤，总是浸润皮下组织。其他报告表明，DFSP的特殊病例可能局限于皮下组织，而无真皮受累。我们希望通过描述局限于皮下组织的DFSP罕见变体病例来证实这一观察结果，并分析可能的组织发生学意义。
报告了3例位于皮下组织的DFSP病例。多步切片显示其中2例无真皮受累，而第3例在5个切片块中的1个中，在与皮下组织交界处有最小程度的真皮浸润。使用一系列单克隆抗体进行了免疫组织化学研究。所有肿瘤波形蛋白和CD34染色均呈强阳性。
由于2例无真皮受累，1例仅有最小程度的真皮受累，我们将此变体称为深部DFSP。除肿瘤位置较深外，深部DFSP在临床、组织学和免疫组织化学上与典型DFSP无法区分。深部DFSP的存在提供了证据，表明皮肤的特定结构可能不参与该肿瘤的组织发生。
