Atrophic dermatofibrosarcoma protuberans: a clinicopathological study of 16 cases

We present a case series of atrophic dermatofibrosarcoma protuberans (DFSP) to further characterise its clinical and pathological features. Sixteen cases were enrolled in the study. There were five males and 11 females with a median age of 28 years. The vast majority occurred in the trunk (12/16, 75%), whereas a minority involved the upper limb or limb gird (2/16, 12.5%), and head and neck region (2/16, 12.5%). The most common presentation was a depressed plaque-like lesion with a greyish-red to purplish-blue colour. Histologically, the lesion was dermal-based consisting of monomorphous spindle cells arranged in parallel fascicles with focal areas displaying storiform architecture. In addition, one case showed remarkable hyalinisation of the matrix, two cases contained scattered pigmented dendritic cells and one case had admixed giant cell fibroblastoma-like component, respectively. The diagnosis was confirmed by immunohistochemical study, and by further fluorescence in situ hybridisation analysis in six cases. Follow-up thus far has revealed a relatively low rate of local recurrence (1/10, 10%). Familiarity with the distinctive clinical and pathological features of atrophic DFSP helps avoid misdiagnosis. Like a classical DFSP, morphological variants can also occur in an atrophic DFSP, including pigmented, sclerosing and hybrid subtypes, albeit rare.