Dermatofibrosarcoma- An uncommon entity, commonly mismanaged: a case report

Dermatofibrosarcoma protuberans (DFS) is a slow-growing, recurrent, cutaneous soft tissue sarcoma with low metastatic potential. It is not uncommon for DFS to get misdiagnosed and treated like commoner parietal wall swellings, viz., epidermal cysts, lipomas and fibromas. Suboptimal management often leads to recurrence, which may be difficult to manage.

A 33-year male was referred to us with a lump in his lower abdomen for 15 years, without any symptoms. The patient underwent medical management for 6 years, followed by excision. The swelling recurred four years later and progressed in size till the next 5 years. At our centre, the patient underwent imaging and aspiration cytology, which established diagnosis of DFS, followed by wide local excision. Biopsy was conclusive and margins negative.

DFS presents commonly as an asymptomatic indurated plaque that slowly enlarges over months to years. Untreated, DFS can attain massive dimensions, producing the large "protuberant" nodules, and hence the name. In the current report, 15 years elapsed before the correct diagnosis was established. In this case, recurrence first appeared after four years of excision. The possibility of primary wound closure after wide excision decreases with every subsequent excision, and reconstructive options may not be readily available.

The current report highlights a diagnostic delay of 15 years in a case of DFS. Asymptomatic, indolent nature combined with low awareness among community doctors contribute to delay in timely diagnosis. Community doctors should consider DFS as a differential in any long-standing, indolent, asymptomatic parietal wall swelling, especially with a history of recurrence.