Ivana de Sousa Brandão; Karuza Maria Alves Pereira; Marcelo Bonifácio da Silva Sampieri; Filipe Nobre Chaves & Denise Hélen Imaculada Pereira de Oliveira
Summary
Angiosarcoma (AG) is a malignant mesenchymal neoplasm, which generally originates from endothelial cells, and the occurrence of primary lesions in the oral cavity being extremely rare. We report a case of primary intraoral AG manifesting at multiple intraoral sites in a 31-year-old male, with rapid progression resembling Kaposi sarcoma, and discuss the clinical, histological, and immunohistochemical aspects of primary intraoral AG, which led to an accurate diagnosis. Characteristically this tumor has an aggressive nature, metastatic potential and short survival rate, so we highlight the value of an early diagnosis of AG for a better prognosis. This clinical case shows an exceedingly rare primary AG with an unusual presentation involving multiple oral sites, wich was a challenge to definitive diagnosis, so histopathological analysis, combined with histochemistry, was essential to correctly identify the pathological entity of the lesion.