Systematic Review and Case Series of Pleomorphic Dermal Sarcoma: an Uncommon Cutaneous Pathology Predominantly in the Head and Neck

Pleomorphic dermal sarcoma is an uncommon mesenchymal neoplasm that shares clinical, immunohistochemical, and histopathologic features with atypical fibroxanthoma but with additional aggressive histopathologic features. This tumor is not well documented in the literature, its clinical features are poorly characterized, and diagnosis is still by exclusion. To better understand this disease, we performed a comprehensive systematic review and included clinical, immunohistochemical, and histopathologic data of five patients (median age: 81) from our own institution. Elderly men are typically affected more than women and the tumor presents as an exophytic growth on sun-damaged regions of the head and scalp, although our cohort had three men and two women. Histologically, the tumors display brisk mitotic activity with asymmetrical lesions; pleomorphic spindle cells; and large, multinucleated giant cells with vacuolated cytoplasm arranged in fascicular patterns. Eighty percent of patients displayed deep tissue invasion and 40% displayed perineural invasion. Immunohistochemical stains for CD10 and CD68 were positive in 60%, and all patients tested were negative for S-100, PanCK, desmin, MART-1, SOX-10 CD-34, P40, SMA, and CK-5/6. Recurrence and further clinical data were not collected due to loss of follow-up. Although there are no standardized treatment guidelines, wide local excision is recommended. The consensus is that pleomorphic dermal sarcoma has a more aggressive disease course than atypical fibroxanthoma with poorer outcomes. Our study creates the first comprehensive treatment recommendations in the literature for better risk stratification, prognoses, and outcomes.