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Angiosarcoma is a rare, highly malignant mesenchymal tumor characterized by endothelial cell differentiation and formation of abnormal blood-filled vascular channels. Although it more commonly affects the skin and deep soft tissues, angiosarcoma can occur in the oral and maxillofacial region, where it poses significant diagnostic and therapeutic challenges due to its aggressive behavior and deceptively benign clinical appearance in early stages.
Definition and Classification
Angiosarcoma is defined as a malignant neoplasm arising from vascular endothelial cells. It may be classified based on site and etiologic association, including:
- Cutaneous angiosarcoma (head and neck skin)- Soft tissue angiosarcoma
- Radiation-induced angiosarcoma
- Lymphedema-associated angiosarcoma (Stewart–Treves syndrome)
Oral angiosarcomas typically fall under soft tissue angiosarcoma.
Epidemiology and Etiology
- Rare tumor, accounting for <2% of soft tissue sarcomas- Predominantly affects older adults
- Slight male predilectiong
= Chronic lymphedema
= Prior radiation therapy
= Chemical exposure (e.g., vinyl chloride, arsenic)
= Trauma (rarely implicated)
Common Sites of Occurrence
In the head and neck region, angiosarcoma most commonly affects:
- Scalp and face skin- Oral cavity sites such as:
= Gingiva
= Buccal mucosa
= Floor of the mouth
Clinical Presentation
Clinical features are variable and often misleading:
- Rapidly enlarging, ill-defined mass- Bluish-red or purplish discoloration
- Tendency to bleed easily
- Ulceration in advanced lesions
- Pain or paresthesia
- Facial swelling or asymmetry
Early lesions may resemble benign vascular lesions such as hemangioma or pyogenic granuloma.
Diagnostic Approach
Early diagnosis is critical but challenging.
Key steps include:
- Detailed clinical evaluation- Imaging (MRI/CT) to assess extent and vascularity
- Incisional biopsy (performed with caution due to bleeding risk)
- Histopathologic examination
- Immunohistochemical confirmation of endothelial differentiation
Immunohistochemistry
Angiosarcoma typically shows positivity for:
- CD31 (most sensitive and specific)- ERG
- Factor VIII–related antigen
High Ki-67 proliferation index reflects aggressive behavior.
Histopathological Features
Microscopic features vary with tumor grade:
- Irregular, anastomosing vascular channels- Atypical endothelial cells lining vascular spaces
- Nuclear pleomorphism and hyperchromasia
- Increased mitotic figures
- Solid sheets of malignant endothelial cells in high-grade lesions
- Areas of hemorrhage and necrosis
Poorly differentiated tumors may lack obvious vascular channels.
Differential Diagnosis
Differential diagnoses include:
- Hemangioma
- Kaposi sarcoma
- Epithelioid hemangioendothelioma
- Pyogenic granuloma
- Melanoma (amelanotic)
- Poorly differentiated carcinoma
Immunohistochemistry is essential for accurate diagnosis.
Management and Treatment
Management is aggressive and multidisciplinary:
- Wide surgical excision with clear margins- Chemotherapy for advanced or metastatic disease
- Palliative care in unresectable cases
Due to high recurrence rates, close follow-up is mandatory.
Prognosis
- Poor prognosis overall- High rates of local recurrence and distant metastasis
- Common metastatic sites: lungs, liver, bone
- Five-year survival rates are generally <40%
- Tumor size, depth, and surgical margin status significantly influence outcome
Role of Dental and Medical Professionals
Dental professionals should:
- Maintain high suspicion for rapidly growing, bleeding oral lesions
- Avoid assuming benign vascular pathology without biopsy
- Refer urgently to specialized centers
- Manage oral complications of treatment and provide long-term surveillance
For students, angiosarcoma highlights the importance of recognizing vascular malignancies and the role of immunohistochemical markers in diagnosis.
Conclusion
Angiosarcoma is a rare but highly aggressive malignant vascular tumor of the oral and maxillofacial region. Its variable clinical presentation often leads to delayed diagnosis. Early recognition, histopathologic confirmation, and aggressive multidisciplinary management are essential to improve patient outcomes.