Dermatofibrosarcoma (DFSP) is a rare, locally invasive, low grade sarcoma. It most commonly presents as an asymptomatic skin-colored plaque on the trunk and upper extremities and is characterised by high rates of local recurrence after apparently adequate surgical resection. The head and neck is only very rarely affected, accounting for only 1.4% of soft tissue sarcomas of the head and neck and less than 0.01% of all head and neck malignancies. Published literature on these tumours is strikingly rare, consisting of scattered case reports and some case series, the largest of which (33 patients) was reported by Stojadinovic and colleagues over a 35 year period.
We report on an abnormal cluster of 5 patients presenting to the Plastic Surgery department at the Princess Alexandra Hospital with DFSP lesions arising in the Head and Neck area from 2015 - 2016. All of these patients had been referred to our department after either biopsy or attempted excision with involved margins.
Lesions were excised with margins ranging from 1cm-3cm. Of a total of 8 excisions performed in our department, there were 3 involved margins (37.5%) requiring re-excision. 2 patients underwent reconstruction with local flaps, 1 required a pedicled regional flap and 2 patients required free tissue transfer. All patients are free from recurrence at a minimum of 6 months follow up.
In our series, Peripheral margins of less than 3cm, or depth of excision that did not include the next anatomical fascial plane was associated with incomplete histological clearance. Our case series validates the assertion that DFSP requires excision with wide margins. It follows that DFSP arising on the Head and Neck represents a challenging reconstructive problem for surgeons. Ultimately early referral to a Plastic Surgery service with experience in Head and Neck reconstruction is required for optimal patient outcomes.