A Comprehensive Literature Review on Diagnostic Strategies and Clinical Outcome of Intraoral Angiosarcoma and Kaposi Sarcoma

Abstract

This review analyzes the clinicopathological features, diagnostic challenges, and clinical outcomes of 60 intraoral angiosarcoma (InO-AS) and 20 intraoral Kaposi sarcoma (InO-KS) cases. These malignancies primarily affect adults, with mean ages of 52.3 years for InO-AS and 44 years for InO-KS, and are rare in children. Both show a male predilection, with InO-KS strongly linked to HIV infection. Metastatic InO-AS typically appears smaller and is located in the mandibular or maxillary gingiva. Most InO-KS cases occur in HIV-positive individuals, often in nodular form. Histological differentiation of InO-AS from poorly differentiated carcinoma and spindle cell carcinoma requires a comprehensive panel of immunohistochemical markers such as CK, CD31, and CD34, while HHV-8 antibody and CD34 help diagnose InO-KS. Treatment for InO-AS involves surgery with radiotherapy and/or chemotherapy, while InO-KS management may include antiretroviral therapy for AIDS patients. InO-AS is aggressive, with over half of patients dying from the disease, whereas InO-KS generally has a less severe course. Despite their rarity, both InO-AS and InO-KS behave similarly to their extraoral counterparts. A key limitation noted in this review is the inconsistent histopathological reporting of AS, particularly regarding histopathological grade, which complicates the assessment and comparison of treatment outcomes.