Ann Dermatol.  2015 Oct;27(5):597-600. 10.5021/ad.2015.27.5.597.

Congenital Dermatofibrosarcoma Protuberans: A Case Report and Literature Review

• Han HH
• Lim SY
• Park YM
• Rhie JW

• KMID: 2171460
• DOI: http://doi.org/10.5021/ad.2015.27.5.597

Abstract

Congenital dermatofibrosarcoma protuberans (DFSP) is an extremely rare skin tumor that is commonly misdiagnosed, or is often diagnosed long after the initial presentation. Although many cases of DFSP are diagnosed in adulthood, there are some differences between adult DFSP and congenital DFSP. We report a case of congenital DFSP that was initially misdiagnosed as a simple vascular lesion. The delay in diagnosis led to the considerable growth of the lesion, such that a huge scar was left after the surgical treatment. The major differences between adult and congenital DFSP are discussed through a literature review. Clinicians should be aware of the characteristics of congenital DFSP, to reduce misdiagnosis and the delay of diagnosis from the initial presentation.

Keyword

Age of onset; Congenital; Dermatofibrosarcoma; Diagnosis

MeSH Terms

Adult
Age of Onset
Cicatrix
Dermatofibrosarcoma*
Diagnosis
Diagnostic Errors
Humans
Skin

Figure

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  Fig. 1 An about 2.0×2.0 cm irregular erythematous-violaceous plaque showing an atrophic change with a scar tissue in the central portion on the posterior neck region. An area in the plaque shows a yellowish color.

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  Fig. 2 Typical spindle cells arranged in a storiform pattern infiltrating the dermis (H&E, ×400).

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  Fig. 3 Photograph at 4 years after surgery. The defect is covered with a bilobed transpositional fasciocutaneous flap.

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