Angiosarcoma of the oral cavity: a clinicopathological study and a review of the literature

Soft tissue sarcomas are uncommon in the head and neck. Primary angiosarcomas of the oral cavity area are extremely rare, and have mostly been presented as case reports. This paper presents the clinical and histological features of three such cases. All patients were diagnosed based on the presence of rapidly extending masses involving the tongue, maxillary gingiva, or mandibular gingiva; bone destruction was present in two cases. The resected specimens revealed clustered large, pleomorphic, and spindle-shaped cells with a markedly haemorrhagic background. Tumour cells showed expression of vascular endothelial markers, such a CD31, CD34, and factor VIII-related antigen. Despite undergoing radical surgery, distant metastases developed in all three cases. We also studied the clinicopathological features of a series of oral angiosarcomas. This article therefore reports the clinicopathological features of the three new cases and provides a review of the cases of primary oral angiosarcoma reported during the past 20 years.