Case reportIntracranial recurrence of the scalp dermatofibrosarcoma
Introduction
Dermatofibrosarcoma protuberans (DFSP) is a rare monoclonal cutaneous soft tissue sarcoma, first described by Darier and Ferrand in 1924 [1]. The most common sites of origin are the skin of the trunk (50%–60%), proximal extremities (20%–30%), and the head and neck (10%–15%) [2], [3]. In spite of its slowly infiltrative growth and tendency towards local recurrence, metastasis is very unusual [4], [5], [6]. The mainstay of treatment for DFSP is surgical resection. Resection with conservative margins has led to unacceptable local tumor control, with local recurrence rates varying from 26% to 60%. To improve local control after excision, many physicians have recommended wide excision to include more than 2 cm grossly uninvolved margins of skin and underlying deep fascia. This treatment approach has reduced local recurrence rates to a mean of approximately 20% [2]. Surgery followed by post-operative radiation therapy (RT) may effectively reduce the local recurrence rate and preserve more tissue than dose radical surgery [7], [8]. Herein, we report a rare case of recurrent scalp DFSP followed by intracranial infiltration following four surgical procedures and radiation therapy.
Section snippets
Case report
A 30-year-old woman was referred to our institution because of a recurrent scalp mass in the parieto-occipital area. The patient was initially operated on at another hospital with a presumed benign lesion. However, the tumor recurred 1 year later. Despite salvage excision at a second hospital, a second recurrence followed 2 years later. A third operation was performed, and the patient received radiotherapy following surgery. Finally, the patient was referred to our clinic, presenting with a
Discussion
Dermatofibrosarcoma protuberans (DFSP), a low-grade spindle cell neoplasm involving both the dermis and subcutis and thus, mobile on the fascia, is an unusual, locally aggressive, cutaneous neoplasm of low-grade malignancy [5], [6]. It affects mainly the trunk and proximal extremities of young and middle-aged adults. Males are affected four times as often as females, and approximately 5% of DFSP cases occur in the pediatric population. DFSP typically appears during the third or fourth decade of
Conclusion
The primary objective in treating patients with DFSP is to achieve aggressive surgical clearance. Adjuvant radiotherapy with or without chemotherapy may provide a useful treatment option when adequate margins cannot be obtained. We recommend regular follow-up for a long period of time in order to guard against late recurrences.
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