Primary parotid angiosarcoma: A case report and literature review

Angiosarcoma is a rare, aggressive vascular malignancy that typically affects older men often following radiotherapy. Primary parotid angiosarcoma is uncommon, particularly in patients without known risk factors. We report the case of an 82-year-old woman who presented a parotid mass and a frontal skin lesion, without prior radiotherapy or relevant medical history. Imaging revealed a 4 cm parotid lesion with mandibular involvement but no nodal disease. Fine needle aspiration suggested a malignant epithelioid neoplasm, and PET confirmed uptake in the parotid gland, mandibular condyle, and frontal lesion. The patient underwent total parotidectomy with en bloc resection of the facial nerve and mandibular condyle, ipsilateral functional neck dissection, and excision of the frontal lesion with deep inferior epigastric perforator (DIEP) flap reconstruction. Histopathology confirmed poorly differentiated angiosarcoma with lymphovascular and perineural invasion. Immunohistochemistry was positive for vimentin, CD31, ERG, and CD34, with loss of H3K27me3 expression but no c-MYC amplification. Despite the adjuvant therapy recommendation, the patient developed pulmonary metastases and died three months postoperatively. Angiosarcoma presents with frequent pulmonary metastases and a dismal prognosis. Surgical resection with negative margins and radiotherapy remains the mainstay of treatment. However, survival outcomes remain poor. This case illustrates its aggressive clinical course and distinctive molecular profile, emphasizing the importance of early diagnosis and multidisciplinary treatment.