Primary Angiosarcoma of the Submandibular Gland

Abstract

Angiosarcoma of the head and neck is a rare tumor of vascular origin that occurs almost exclusively in the scalp and forehead region. Primary angiosarcomas of the submandibular gland are extremely rare. Here we report one such case. The patient was referred for evaluation of a neck mass in the right submandibular region that had rapidly enlarged within the previous half year. Computed tomography scanning showed a hypervascular tumor within the submandibular gland. Excision of the submandibular gland was attempted. The histopathology revealed characteristic features of high grade angiosarcoma, which revealed a predominant solid tumor mass with few vascular channels which composed with irregular and freely anastomosing channels lined by marked pleomorphic spindle endothelial cells. Positive CD34 and P53 expressions indicated malignant vascular origin tumor. Such a rare case of angiosarcoma occurs in the salivary gland presenting as a neck mass with variable imaging and cytological features make pre-operative diagnosis difficult. Spectrum of vascular tumors are variable, and excision biopsy should be performed to establish the diagnosis. The final diagnosis must be made according to histological examination, immunocytochemical studies and clinical behaviors.