Angiosarcoma of the tongue: A case series and literature review.

PURPOSE: Angiosarcoma of the tongue is an exceedingly rare malignancy of the head and neck. Such lesions can be primary in nature or occur in a previously irradiated field. We examine a series of cases with relation to clinical presentation, diagnosis, management, and outcomes. MATERIALS AND METHODS: Retrospective chart review of all patients with angiosarcoma of the tongue at a tertiary academic institution yielded a single case between 2005 and 2016. The MEDLINE database was additionally searched for all case series or reports of angiosarcoma arising in the tongue, and pertinent clinical data were extracted. RESULTS: The clinical presentation, disease course, and management of a patient with angiosarcoma of the tongue are presented. Institutional and literature search yielded a total of eight patients with angiosarcoma of the tongue. The most common primary sites were dorsal and lateral oral tongue. Treatment consisted of surgical resection in 63% of cases with adjuvant therapy administered in 75% of cases. Follow-up times varied per patient, but 63% had persistent or recurrent disease and 67% died of or with disease within two years of index presentation. CONCLUSION: Angiosarcoma of the tongue is a rare and highly aggressive tumor, accounting for fewer than 1% of all head and neck malignancies. The mainstay of treatment is surgical resection with negative margins followed by adjuvant chemoradiation for high-risk features. Due to rarity of the disease, consensus on optimal treatment approach is lacking, and multi-center prospective studies would be helpful to set clinical guidelines.