Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Mar 1;88(3):319-322.
doi: 10.1097/SAP.0000000000002970.

Diagnostic and Management Considerations in Pediatric Dermatofibrosarcoma Protuberans

Daniel J Reilly  1 , Yew Loong Loo  1 , William M Alexander  1 , Daniel J Wilks, Duncan MacGregor, Christopher J Coombs
Affiliations

Diagnostic and Management Considerations in Pediatric Dermatofibrosarcoma Protuberans

Daniel J Reilly et al. Ann Plast Surg. .

Abstract

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare fibrohistiocytic tumor of dermal origin. Six percent of all cases present in children, with a childhood incidence of 1 per million.

Methods: This is a retrospective review of all cases of pediatric DFSP managed at a single institution over a 23-year period.

Results: Seventeen patients (10 male; mean age, 9.9 years) were managed during the study period. The median follow-up was 29 months. All patients had surgical excision. Three patients required further excision to achieve uninvolved final margins. There were no recurrences observed.

Conclusions: Pediatric DFSP should be managed by a soft tissue tumor multidisciplinary team, with experienced pathologists and reconstructive surgeons. Where R0 resections are obtained, patients can experience recurrence-free survival.

PubMed Disclaimer

Conflict of interest statement

Conflicts of interest and sources of funding: none declared.

References

    1. Valdivielso-Ramos M, Hernanz JM. Dermatofibrosarcoma protuberans in childhood. Actas Dermosifiliogr . 2012;103:863–873.
    1. Kornik RI, Muchard LK, Teng JM. Dermatofibrosarcoma protuberans in children: an update on the diagnosis and treatment. Pediatr Dermatol . 2012;29:707–713.
    1. Durack A, Gran S, Gardiner MD, et al. A 10-year review of surgical management of dermatofibrosarcoma protuberans. Br J Dermatol . 2021;184:731–739.
    1. Thornton SL, Reid J, Papay FA, et al. Childhood dermatofibrosarcoma protuberans: role of preoperative imaging. J Am Acad Dermatol . 2005;53:76–83.
    1. Rubio GA, Alvarado A, Gerth DJ, et al. Incidence and outcomes of dermatofibrosarcoma protuberans in the US pediatric population. J Craniofac Surg . 2017;28:182–184.

LinkOut - more resources