Dermatofibrosarcoma protuberans in children and adolescents: Clinical presentation, histology, treatment, and review of the literature

Abstract

Purpose: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant tumor. It is characterized by aggressive local infiltration, leading to a propensity for recurrence. In children, DFSP is even less common and likely misdiagnosed or underdiagnosed. This study is a review of DFSP in the pediatric population and aims to identify factors for successful treatment.

Materials and methods: From July of 1986 to 2011, a total of 159 patients were diagnosed with dermatofibrosarcoma protuberans at Kaohsiung Chang Gung Memorial Hospital, Taiwan. Subject to the age classification of our institution, patients under the age of 18 are defined in the pediatric category, of which159 cases were identified as our research subjects. Detailed data, including demographic data, imaging studies, pathology, treatment methods, and outcomes, of these identified patients were collected, reviewed, and analyzed.

Results: A total of 13 patients, consisting of six male and seven female patients, were identified based on our criteria. Two had the lesions noticed at birth. Most patients experienced a variable period of quiescence, followed by a rapid growth phase. All 13 patients underwent wide excisions. Post-excision reconstruction included direct closure in three cases, skin grafting in three cases, and local or free flap reconstruction in seven cases. Of 13 patients, four received postoperative radiotherapy. All patients survived without recurrence up to July 2011, with follow-up periods ranging from 20 months to 19 years.

Conclusion: Clinicians should be aware that DFSP is known to occur among children. Owing to its relatively low incidence, its presence may be confused with commoner lesions such as hemangioma, fibroma, or atrophic plaques without nodule. The confusing situation, as a result, frequently leads to delayed diagnosis. Vigilance in its diagnosis allows for treatment at manageable sizes as well as ensures complete excision. Reconstructive options, such as skin grafting, and modalities, such as adjuvant postoperative radiotherapy, are suggested to best complement each other. The former minimizes disfigurement while the latter minimizes recurrences.

Keywords: Adolescent; Children; Dermatofibrosarcoma protuberans; Treatment.