Background: Pleomorphic dermal sarcoma (PDS) describes rare dermal-based malignant tumours that are morphologically similar to atypical fibroxanthoma (AFX). PDS may be differentiated from AFX by the presence of one or more of the following histologic features: subcutaneous invasion, tumour necrosis, lymphovascular invasion (LVI), and/or perineural infiltration (PNI).
Aims: To further define the clinicopathological features, surgical management, and outcomes of PDS primary tumours.
Methods and results: This study was a retrospective observational case series using a database search from 2012 to 2017. Inclusion criteria required all cases to meet the histopathologic criteria for PDS as confirmed by a specialist soft-tissue histopathologist. A total of n = 17 cases were included with a median age of 78 years (range 66-85). All tumours were located on the head and neck, with 13/17 located on the scalp. Primary treatment was with wide local excision (WLE) in all cases. Median follow-up was 48 months. Local recurrence occurred in 4/17 cases (24%) and distant metastasis in 2/17 cases (12%).
Conclusion: PDS behaves more aggressively than atypical fibroxanthoma with which it shares a biologic continuum. The optimal surgical management approach is yet to be determined.
Keywords: atypical; cutaneous sarcoma; fibroxanthoma; local recurrence; metastasis; pleomorphic dermal sarcoma; skin cancer.
© 2021 The Authors. Cancer Reports published by Wiley Periodicals LLC.