Darier and Ferrand's dermatofibrosarcoma: A carcinologic and cosmetologic challenge in a low-income country

Dermatofibrosarcoma (DFS) is a common dermic sarcoma. It is a local malignant tumor occurring in young adults. The recurrence potential justifies an R0-type surgery with a three centimeters margin. We report our experience of the management of locally advanced DFS by resection and reconstructive surgery.

It is an ongoing descriptive study spanned from June 2005 to December 2018. We included all DFS cases treated by curative resection and reconstruction. A total of eight cases of DFS among 108 soft tissue sarcomas were studied. All patients were males. The mean age was 41.8 years [32-60]. Carcinologic results, cosmetic results, and outcomes were analyzed.

R0-type resection was performed in six cases. In two cases, the resection was R1-type and resulted in amputation. In four cases, it was an iterative surgery. Average desease duration was 4 years [1-8]. Reconstructive surgery was needed for wound closure in six cases. Wounds healed in 28 days [18-90]. Outcomes showed hyperchromic keloid scars (N=2) at the trunk localization.

DFS is a common cancer with a good outcome if managed earliest. Delayed diagnoses and inadequate first-time surgery led to tumor extension and recurrences. Locally advanced tumors management needs extensive resections and reconstructive surgery. In addition to surgery, Imatinib and radiotherapy improve outcomes, but are not available in our context.