Pleomorphic dermal sarcoma: Clinicopathological features and outcomes from a 5‐year tertiary referral centre experience

Abstract

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.

Prognostic factors for pleomorphic dermal sarcoma: analysis of 1911 cases from the SEER database

“…Clinical and pathological data are presented for the SEER cohort of PDS in table 1. Most patients were septuagenarian males and most tumours involved the head/neck region, in accordance with prior cohorts 3 17. Tumours were generally small with a median size of 1.5 cm (75% were <2.5 cm) and <10% of patients received any adjuvant or neoadjuvant radiotherapy.…”

“…Histopathological characteristics such as local tumour extension (into deep subcutaneous tissues or adjacent structures) and lymphovascular invasion may be important for prognostication of not only PDS/AFX, but other types of cutaneous sarcoma as well 17 28–30. Unfortunately, as mentioned previously, these variables are not rigorously reported or defined in the SEER database and could not be evaluated here.…”

Fine needle aspiration cytopathology of pleomorphic dermal sarcoma

“…Given its propensity to arise in chronic ultraviolet (UV)-exposed skin, most examples have been reported in the head and neck region, particularly the scalp of elderly men. 6,8 PDS shares with AFX similar UV signature mutations in TP53, TERT promoter mutations, and frequent mutation in NOTCH1/2 and FAT1. Both have similar DNA methylation profiles and DNA copy number aberrations also.…”

“…PDS is an undifferentiated cutaneous mesenchymal neoplasm with a definite risk for local recurrence, and for distant metastases. 6,7 As a condition of its uncertain histogenesis, it fails to immunohistochemically express epithelial, melanocytic, vascular, or myogenic markers.…”

“…As a condition of its uncertain histogenesis, it fails to immunohistochemically express epithelial, melanocytic, vascular, or myogenic markers. Given its propensity to arise in chronic ultraviolet (UV)‐exposed skin, most examples have been reported in the head and neck region, particularly the scalp of elderly men 6,8 . PDS shares with AFX similar UV signature mutations in TP53, TERT promoter mutations, and frequent mutation in NOTCH1/2 and FAT1.…”