Clinicopathological and survival analyses of primary cutaneous angiosarcoma in an Asian population: prognostic value of the clinical features of skin lesions.

Abstract

Primary cutaneous angiosarcoma (CA) is a rare but aggressive tumor with a high rate of local recurrence. This study was designed to analyze the clinicopathological features of primary CA and identify factors of cutaneous manifestations associated with the prognosis of angiosarcoma. Medical records of 55 patients with primary CA were retrospectively analyzed to investigate clinical features, survivals, and prognostic factors. Anatomical location of tumor was classified to the scalp, face, and neck, and sites outside the head and neck. Primary CA presented cutaneous nodules (31/55, 47.2%), patches (13/55, 23.6%), and indurated plaques (11/55, 20.0%). Nodular lesion was significantly more common in CA on the scalp compared to CA on sites outside the scalp. Histologically, tumors presenting as nodular lesions on the scalp was predominantly composed of solid sheets of large pleomorphic cells, whereas non-nodular lesions composed of tumor cells between collagen bundles forming irregular vascular spaces. Cutaneous angiosarcoma on the scalp showed a worse prognosis compared to CA on sites outside the scalp. Patients presenting clinical morphology with nodules and multiple skin lesions showed significantly reduced overall survival (OS). In primary CA, location on the scalp, morphology with nodules, and multiplicity of skin lesions significantly affected survival outcomes.