Diaz-Cascajo C, Weyers W, Borghi S
Center for Dermatopathology, Freiburg, Germany.
J Cutan Pathol. 1998 Sep;25(8):440-4. doi: 10.1111/j.1600-0560.1998.tb01771.x.
Dermatofibrosarcoma protuberans is a low-grade sarcoma of the skin with well characterized clinical and histopathological features. The lesion frequently appears as a plaque on the trunk or on the extremities of middle-aged adults. Microscopically, the tumor is composed of monomorphous spindle cells arranged in storiform pattern and embedded in a sparse to moderately dense fibrous stroma. We report on two unusual cases of dermatofibrosarcoma protuberans in which neoplastic cells were diffusely replaced by sclerotic tissue in more than 50% of the entire tumor. In both cases, no external trauma or radiotherapy were recorded. Since tumor regression has been defined as a loss of tumor mass in the absence of any treatment (or trauma) we believe that sclerosis in DFSP may represent a manifestation of regression and not, as previously suggested, a new variant of the neoplasm.
隆突性皮肤纤维肉瘤是一种皮肤低度恶性肉瘤，具有明确的临床和组织病理学特征。该病变常表现为中年成人躯干或四肢的斑块。显微镜下，肿瘤由呈车辐状排列的单一形态梭形细胞组成，并嵌入稀疏至中度致密的纤维性间质中。我们报告了两例不寻常的隆突性皮肤纤维肉瘤病例，其中肿瘤细胞在整个肿瘤的50%以上被硬化组织弥漫性取代。两例均未记录到外部创伤或放疗史。由于肿瘤消退被定义为在没有任何治疗（或创伤）的情况下肿瘤体积的缩小，我们认为隆突性皮肤纤维肉瘤中的硬化可能代表消退的一种表现，而不是如先前所认为的肿瘤的一种新变体。
