Angiossarcoma primário de tecido mole em cavidade oral: um caso incomum.

Abstract

Angiosarcoma is a rare and aggressive malignant tumor that has a poor prognosis. Primary lesions in the oral cavity are extremely rare (0.0077%). Characteristically, they have a high rate of local recurrence and an early metastatic potential. We aimed to report the case of a 31-year-old male with angiosarcoma in alveolar ridge extending to hard palate as well as literature review. Clinically, there were ulcers with raised borders, with granulomatous characteristics and purplish coloration, measuring more than 24 mm. Histologically, the tumor consisted of spindle and polygonal cells with hyperchromatic nuclei with nucleoli. These cells are sometimes arranged in a stent-shaped pattern or forming numerous vascular structures of varying calibers. The tumor was invasive in the surrounding tissue, and lymphovascular permeation was noted. Immunohistochemically, being Grocott negative, tumor cells were positive for CD34 in vessels and scattered cells, FLI-1 in spindle cells and Ki-67 labeling mair than 15%. A pathological diagnosis of angiosarcoma was made. Metastatic lesions are found in upper limbs and thorax. Radical operation is now planned, associated with chemotherapy. For the review of the literature, an online database search was conducted for articles published in the last 10 years. Because of the rarity, it is essential to use vascular markers, such as CD34, CD31 and FLI1, for one of the histological diagnoses and for internal planning.