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DermDx_020417_DermatofibrosarcomaProtuberans_v211.
A 44-year-old man presents for evaluation of a growth on his right shoulder. The lesion was first noted approximately 3 years previously and began as a “pimple that didn’t stop growing.” He denies spontaneous bleeding, but lately the growth has become tender as a result of irritation from clothing. The patient admits to both alcohol and cigarette use and has no history of skin cancer. Examination reveals a prominent erythematous nodule of the right scapula. Cervical and axillary lymph nodes are nonpalpable.
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Dermatofibrosarcoma protuberans (DFSP) is an uncommonly encountered soft tissue sarcoma that most often occurs on the trunk and proximal extremities.1 The majority of lesions begin as asymptomatic skin-colored to slightly erythematous plaques that slowly enlarge in size.2 Over time, nodules that infiltrate subcutaneous tissue develop and may invade tendons, fascia, muscle, and bone.3 Metastatic spread is quite rare.4 Histopathology results of DFSP reveal diffuse infiltration of the dermis and subcutis. The cellular origin of DFSP is postulated to be undifferentiated mesenchymal cells.5
Wide excision down to and including the fascia, with negative margins of at least 3 cm, is a long-standing treatment option.6 Excision with 5-cm borders results in a recurrence rate of less than 5%.7 Radiation therapy is sometimes administered postoperatively.8 Some clinicians favor Mohs micrographic surgery, especially when tumors are located on the head and neck. Mohs technique conserves tissue and is associated with a recurrence rate of less than 2%.9
Lawrence Schiffman, DO, is the director of Miami Skin Dr. in Doral, Florida. Stephen Schleicher, MD, is an associate professor of medicine at the Commonwealth Medical College in Scranton, Pennsylvania, and an adjunct assistant professor of dermatology at the Perelman School of Medicine at the University of Pennsylvania in Philadelphia. He practices dermatology in Hazleton, Pennsylvania.
References
- Cai H, Wang Y, Wu J, Shi Y. Dermatofibrosarcoma protuberans: clinical diagnoses and treatment results of 260 cases in China. J Surg Oncol. 2012;105:142-148.
- Bhambri S, Desai A, Del Rosso JQ, Mobini N. Dermatofibrosarcoma protuberans: a case report and review of the literature. J Clin Aesthet Dermatol. 2008;1:34-36.
- Stivala A, Lombardo GA, Pompili G, Tarico MS, Fraggetta F, Perrotta RE. Dermatofibrosarcoma protuberans: our experience of 59 cases. Oncol Lett. 2012;4:1047-1055.
- McArthur G. Dermatofibrosarcoma protuberans: recent clinical progress. Ann Surg Oncol. 2007;14:2876-2886.
- Dimitropoulos VA. Dermatofibrosarcoma protuberans. Dermatol Ther. 2008;21:428-432.
- Khatri VP, Galante JM, Bold RJ, Schneider PD, Ramsamooj R, Goodnight JE Jr. Dermatofibrosarcoma protuberans: reappraisal of wide local excision and impact of inadequate initial treatment. Ann Surg Oncol. 2003;10:1118-1122.
- Chang CK, Jacobs IA, Salti GI. Outcomes of surgery for dermatofibrosarcoma protuberans. Eur J Surg Oncol. 2004;30:341-345.
- Dagan R, Morris CG, Zlotecki RA, Scarborough MT, Mendenhall WM. Radiotherapy in the treatment of dermatofibrosarcoma protuberans. Am J Clin Oncol. 2005;28:537-539.
- Snow SN, Gordon EM, Larson PO, Bagheri MM, Bentz ML, Sable DB. Dermatofibrosarcoma protuberans: a report on 29 patients treated by Mohs micrographic surgery with long-term follow-up and review of the literature. Cancer. 2004;101:28-38.
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