Congenital Dermatofibrosarcoma Protuberans: A Case Report.

Yun, Byung Min

Case Report

Journal of the Korean Society of Plastic and Reconstructive Surgeons 2010;37(3):285-288.
Published online May 1, 2010.

Congenital Dermatofibrosarcoma Protuberans: A Case Report.

Department of Plastic and Reconstructive Surgery, College of Medicine, Jeju National University, Jeju-si, Korea. almostfree@hanmail.net

Abstract

PURPOSE
Dermatofibrosarcoma protuberans is a rare tumor, accounting for 0.1% of all malignant tumors. Although metastasis is very uncommon, local recurrence occur frequently. Dermatofibrosarcoma protuberans occurring in children is even more rare; this is the first case report of congenital dermatofibrosarcoma protuberans in Korea. METHODS: The patient is a 14-month-old male infant with a lesion that was first thought to be a birthmark. The lesion grew larger, and a punch biopsy revealed dermatofibrosarcoma protuberans. A wide local excision was performed with a 2cm peripheral resection margin beyond the gross tumor lesion. Deep fascia and a portion of muscle underneath the central part of the lesion were also taken. The surgical defect was covered by a split-thickness skin graft.
RESULTS
There has been no clinical sign of recurrence over one year after the surgery.
CONCLUSION
A patient with congenital dermatofibrosarcoma protuberans detected at an early stage underwent a wide local excision of the tumor after accurate diagnosis was carried out by biopsy and immunohistochemical studies. There was no clinical evidence of tumor recurrence during over a 1-year follow-up.

Keywords: Congenital; Dermatofibrosarcoma protuberans; Wide excision