Primary oral leiomyosarcoma: A systematic review and update

: The aim of this systematic review was to address the clinicopathologic inconsistencies noted with primary oral leiomyosarcoma in the literature by amassing the available data published into a comprehensive analysis. Eligibility criteria included publications of cases with dedicated immunohistochemical work-up along with radiographs to evaluate location. Based on these criteria, the systematic review compiled 29 cases. Four primary location sites were identified in the reported cases: soft tissue presentation only, soft tissue with bony involvement, bony involvement only, and bony involvement with a soft tissue component. The majority of primary oral leiomyosarcoma cases reviewed showed a soft tissue predilection, which is in contrast to prior reports of jawbones being the most common site. In addition, there was an improved 5-year survival rate for primary oral leiomyosarcoma limited to the oral cavity and gnathic bones without extension into paranasal sinuses.