Angiosarcoma is a rare and highly malignant neoplasm which develops from the endothelium of blood vessels. A few cases of primary angiosarcoma of the parotid gland have been reported. However, there is no report of primary angiosarcoma of accessory parotid gland. In this case, we report a primary angiosarcoma of the accessory parotid gland in a 45-year-old man without prior irradiation of the head and neck region. Ultrasound finding revealed a 2.0¡¿2.6 cm sized homogeneous hypoechoic mass and CT scan showed a contrast enhanced homogeneous mass. Fine needle aspiration biopsy diagnosed a benign tumor. The mass was completely excised with a minimal vertical incision. The histologic diagnosis confirmed a angiosarcoma showing anastomosing vascular channels lined by atypical endothelial cells and many branching vessels with staghorn appearance with positive immunohistochemical staining for CD34, a highly specific endothelial marker. The patient underwent postoperative radiotherapy and was followed for 8 years without recurrence and metastasis.
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