A 40-year-old man presents with a nodular growth on his trunk that has been present for 10 years. It started as an indurated plaque and slowly enlarged to a size of 4 cm. The patient denies prior scar or nevus at the site. Physical examination reveals an exophytic, irregularly-shaped nodule with a reddish hue. The surrounding skin is unremarkable. No lymph nodes were palpable. Other than the nodule, the patient is in good health.
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Dermatofibrosarcoma protuberans (DFSP) is a dermal sarcoma that gradually increases in size and occurs primarily in young adults. It typically starts as an indurated plaque from which multiple firm nodules subsequently arise, sometimes with ulceration.1-3
Atrophic forms have been reported; these tend to be greyish-red to purplish-blue in color and resemble atrophoderma or anetoderma.4 The most common locations for DFSP are the trunk and limbs. The tumor is characterized by low metastatic potential, but significant subclinical infiltration and extension that often results in local structural destruction.5 DFSP has a high rate of local recurrence.1,2
Differential diagnosis includes other malignancies as well as benign neoplasms such as dermatofibroma. A history of local trauma including a prior tattoo may predate the occurrence of DFSP.6-8 Dermoscopy may aid in diagnosis. The most common dermoscopic patterns include delicate pigmented network and increased vasculature. Shiny white streaks, a pinkish background, and structureless depigmented areas are also encountered.9,10
Histopathology confirms the diagnosis and reveals high cellularity composed of uniform spindle cells with large nuclei, slight atypia, and low mitotic activity. Immunohistochemistry shows the positivity of CD34 monoclonal antibodies.10
Treatment of DFSP varies depending on the location and the size of the tumor. Mohs micrographic surgery, when available, may be preferred over wide surgical excision.11 The use of systemic therapy with tyrosine kinase inhibitors (imatinib) is indicated when the surgical excision is difficult or in cases of metastasis.12,13
Nejib Doss, MD, is a dermatologist practicing at the Department of Dermatology, Military Hospital of Tunis, Tunisia. Aleksandra Lesiak, MD, PhD, is a professor in the Department of Pediatric Dermatology and Dermatological Oncology at the Medical University of Lodz in Lodz, Poland. Asja Prohic, DMSc, is a professor of dermatovenereology at the University of Sarajevo and on staff at the Medicine Cekalusa in Sarajevo, Bosnia and Herzegovina. Stephen Schleicher, MD, is director of the DermDox Center for Dermatology in Pennsylvania, as well as an associate professor of medicine at Commonwealth Medical College and a clinical instructor of dermatology at Arcadia University and Kings College.
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13. Llombart B, Serra C, Requena C, et al. Guidelines for diagnosis and treatment of cutaneous sarcomas: dermatofibrosarcoma protuberans. Actas Dermosifiliogr. 2018;109(10):868-877.