Imaging of a rare sarcoma: A case series of dermatofibrosarcoma protuberans treated in a Tertiary Care Center

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare, low-to-intermediate grade locally aggressive sarcoma. It typically arises in the dermis and infiltrates subcutaneous tissue, predominantly of the trunk and extremities. It has a propensity to recur locally. There are infrequent incidences of distant metastases. The purpose of this study was to know about different imaging modalities in the assessment and management of DFSP. A retrospective study was done on a series of four patients who presented to our institute with complaints of progressive swellings in different regions of the body. A clinical diagnosis of soft-tissue sarcoma was made. They underwent radiography/ ultrasonography/computed tomography/magnetic resonance imaging in our department followed by excisional biopsy. There were certain imaging features suggestive of DFSP. On excisional biopsy, all were proven to be DFSP. There was surgical resection in all the reported cases. After 6 months of follow-up, there was local recurrence in two of the cases. We came to the conclusion that, due to the superficial appearance of DFSP, common lesions are initially thought of which leads to misdiagnosis. Imaging techniques are not always utilized and there is a delay in their proper management. Knowledge of the variable imaging appearances of DFSP may help in the diagnosis of difficult and atypical case scenarios and help in proper early management.