Recurrent Dermatofibrosarcoma Protuberans of the Head and Neck: a Case Series

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma that develops from dermal fibroblasts and spreads within the dermis and subcutaneous fat. It is locally aggressive, with a high local recurrence rate after excision but has extremely low metastatic potential. In the case of recurrent tumors, surgical excision with adequate margins is the gold standard treatment and may require adjuvant radiotherapy or chemotherapy in some cases. We conducted a retrospective analysis of individuals with dermatofibrosarcoma protuberance of the head and neck region that had treatment at our facility between 2016 and 2021. We gathered the data on the surgical techniques, reconstructive techniques used, histopathological features, adjuvant therapy, and outcomes. We treated three patients with head and neck dermatofibrosarcoma protuberance: one scalp lesion and two on the cheek. All three patients had recurrent tumors, two of whom were treated elsewhere for the primary lesion. One patient underwent surgery for a benign spindle cell tumor of the right cheek, but a final histopathological examination revealed dermatofibrosarcoma protuberance, and the tumor recurred within 3 months. The duration of recurrence is between 3 and 24 months. The size of the tumor ranges from 7.2 to 10.5 cm. The wide local excision margins range from 2 to 4 cm. Reconstruction ranges from split skin graft to regional flap. Inadequate margins raise the possibility of local recurrence in dermatofibrosarcoma protuberance.

Dermatofibrosarcoma protuberans challenges: a case series and review of the literature

“…The trunk and extremities are the most common locations for DFSP, while recent studies have shown that about 40% of lesions were found in the head and neck region. Moreover, the recurrence rate of head and neck lesions was higher in some surveys, albeit lacking statistical difference [6,13].…”

“…Moreover, years after the initial diagnosis, a minority of nearly 6% can invade distant organs such as the lungs [16]. However, one study reported 15% lung metastasis in DFSP cases in correlation with other forms of high-grade sarcomas [6,13,[17][18][19]. Age and gender are not risk factors for DFSP and its recurrence.…”

“…Histologically, DFSP consists uniformly of spindle cell fascicles positioned in a storiform pattern. However, due to various CD34 immunoreactivity in DFSP, its presentation can be mistaken with other malignancies or even benign lesions [4][5][6][7]. The majority of DFSP cases have a t (17; 22) (q22; q13) which results in the creation of COL1A1-PDGFB fusion transcripts [8][9][10][11].…”

Fibrosarcomatous Dermatofibrosarcoma Protuberans of the Head

“…On immunohistochemistry, DFSP shows strong and diffuse positive staining for CD34 while being negative for factor XIIIA, desmin, D2-40, or CD163 (markers more commonly seen in benign dermato broma). Features of rare variants, and pitfalls to their Fibrosarcomatous transformation of DFSP is seen in less than a quarter of all cases, and have been described to be locally aggressive, has high propensity to recur, and capacitates metastatic spread 9 due to its increased cellularity, high mitotic activity, and deep tissue involvement. Although the mechanism for brosarcomatous transformation has not been fully elucidated, this process is considered as a form of tumor progression.…”

“…Fibrosarcomatous DFSP of the head, particularly those involving the scalp, represents the farthest end of the spectrum of DFSP. Left untreated, the aggressive behavior of the tumor can jeopardize underlying neurovascular structures 9 . In the cohort analysis by Go, et al (2022) 3 , factors such as advanced age, tumor size, and socioeconomic status are the most signi cant predictors of survival in head DFSP.…”