Dermatofibrosarcoma Protuberans Occurring in an Adult Presenting with Chronic Lymphedema: A Case Study and Literature Review

Abstract

BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a rare soft-tissue tumor typically located in the trunk. We report a unique case of DFSP in the right inguinal region of an adult presenting with chronic lymphedema. Only 1 case of DFSP and chronic lymphedema association has been previously reported in the literature. Since we could not provide adjuvant radiotherapy (RT), we conducted an extensive review of its application in similar cases, exploring various surgical treatments. CASE REPORT A 42-year-old Cameroonian man with unexplained chronic lymphedema presented with a tumor in the inguinal region of the affected limb. The patient underwent wide local excision (WLE) of the mass, including regional lymph node dissection. Pathological exam confirmed DFSP with a fibrosarcomatous component. Adjuvant RT was considered but not pursued due to the patient;s non-compliance. CONCLUSIONS This DFSP is reported for its rarity of site and the unique co-occurrence with chronic lymphedema. Considering both conditions are uncommon and the rarity of site of the DFSP, we assume that in this patient, chronic lymphedema was a contributing factor of occurrence of the DFSP. Remarkably, no prior reports have detailed an association between chronic lymphedema and DFSP onset. For that reason, we want to point out the value of better follow-up of chronic lymphedema and better knowledge of DFSP treatment options to improve patient healthcare and limit DFSP recurrence. In addition, we found adjuvant RT is an interesting treatment option that might be considered in all patients undergoing surgical excision, even in cases where negative surgical margins were achieved.

Figure 1.

Picture of elephantiasis in the right lower limb of the patient.

Figure 2.

Low-power view (×0.5) demonstrating a diffuse partially nodular infiltration of the deep dermis and subcutis. Note that the epidermis is spared. Arrows showing the typical honeycomb appearance of the infiltration of the subcutis.

Figure 3.

Power view (×5) demonstrating a diffuse infiltration of the deep dermis and subcutis. Arrows indicate the typical honeycomb appearance of the infiltration of the subcutis. The tumoral cells are spindles arranged in short fascicles with palisading. Plus sign: Focal feature of the tumor showing long fascicles and a herringbone pattern (fibrosarcoma-like).