Dermatofibrosarcoma Protuberans Presenting as a Subcutaneous Cystic Nodule

Chris Logas,Austin Dunn,Eli Saleeby,Danielle Lazarra,Justin Rubin,Brad Glick

doi:10.25251/skin.5.6.16

Abstract

A 41-year-old African American male complains of a painful cyst located on his right shoulder and lasting 2 years. The specimen was excised and stained positive for CD34 and negative for SOX-10, confirming the diagnosis of Dermatofibrosarcoma Protuberans (DFSP). The initial specimen had positive extension at both the deep and lateral margins. Complete removal of the tumor required 3 stages of MMS resulting in a 10 cm x 7.6 cm final defect, extending to muscle. As demonstrated in this case, DFSP has an erratic growth pattern and requires meticulous histopathological examination to ensure clear margins. MMS is the preferred method of treatment. Cases of DFSP have high rates of recurrence and require regular follow-up for 3-5 years after treatment.

Highlights

Dermatofibrosarcoma Protuberans (DFSP) is a rare soft tissue tumor that was first described by Derier and Ferrand in 19241
Occurrence of DFSP is preceded by trauma in approximately 10% of cases, it is unclear if there is a direct correlation with development 4
We report a clinical case of a patient diagnosed with DFSP and subsequently treated with Mohs micrographic surgery (MMS)

Summary

INTRODUCTION

Dermatofibrosarcoma Protuberans (DFSP) is a rare soft tissue tumor that was first described by Derier and Ferrand in 19241. It most often occurs on the trunk of young to middle aged patients and has an incidence of 4.1 per million adults accounting for 0.1% of all malignancies 2–4. It often presents as a slow growing, indurated plaque and progresses into a violaceous to red-brown firm nodule fixed to the skin with occasional reddish blue to reddish-yellow surrounding discoloration. Complete removal of the tumor required 3 stages of MMS resulting in a 10 cm x 7.6 cm final defect, extending to muscle, (figure 1B). The patient underwent genetic, PET/CT imaging, and tumor marker testing which were all negative for any pathological abnormalities or metastatic disease

DISCUSSION

Findings

CONCLUSION