Pleomorphic dermal sarcoma: a clinical and histological review of 49 cases

Pleomorphic dermal sarcoma (PDS) is a rare mesenchymal neoplasm that shares histopathological features with atypical fibroxanthoma (AFX), but has additional features of deep invasion of the superficial subcutis, tumour necrosis, and vascular and perineural invasion, which confers a risk of local recurrence and metastases. To study the clinical and pathological spectrum more comprehensively, we retrieved 49 cases of pleomorphic dermal sarcoma from our departmental files. There was a strong male predominance (n=45). The mean (range) age at presentation was 80 (47-97) years. The tumours had a median (range) maximum dimension of 23.5 (5-75) mm. There was a strong predilection for the head and face (n=47), with the scalp (n=32) being the most common site. Ulceration was observed in 21 patients, tumour necrosis in seven, perineural infiltration in six, and lymphovascular invasion in two. CD10 was expressed in all cases. Smooth muscle actin was expressed in 15 and CD68 in 14. Follow up was available in 41 cases (mean (range) 22.4 (2-90) months). Six patients had local recurrences, despite all having originally undergone primary surgical resections and having reports of clear margins. Progressive metastatic disease was observed in one patient who died from their disease. Based on the Kaplan Meier method, median overall survival was 70.8 (8.4-133.1) months. Although AFX and PDS may be part of a spectrum, distinction is important to emphasise the potential for malignant behaviour in PDS.