Histologic and Immunopathologic Variability in Primary Intraoral Angiosarcoma: A Case Report and Review of the Literature

Abstract

Primary intraoral angiosarcoma is an exceptionally rare malignancy of vascular origin which can be challenging to diagnose due to microscopic and immunohistochemical variability. A histopathologically challenging case of primary intraoral angiosarcoma, occurring in a pediatric patient is presented. A comprehensive review of the literature reveals that primary intraoral angiosarcomas occur with nearly equal frequency in males and females, affect the gingiva and the tongue most commonly and are treated primarily with surgery. As with angiosarcoma in other sites, primary intraoral angiosarcoma behaves aggressively with the majority of patients succumbing to their disease.

Intraoral angiosarcoma with unusual clinical presentation: A case report

“…Moreover, in this case, epithelioid angiosarcoma was entirely negative for CD34. This is not unusual, as it has been similarly reported by others previously [ 9 , 10 , [23] , [24] , [25] ].…”

“…Epithelioid angiosarcoma generally shows positivity to CD31 and 34, Fli 1, ERG and claudin-5, with the expression of immunohistochemical stains CD31 and CD34 being variable [ 9 , 10 , 23 ]. ERG, on the other hand, has the highest sensitivity (reaching 100%) and a high specificity [ [23] , [24] , [25] ].…”

“…This raises the importance of using such an endothelial marker in cases where the widely used stains are negative, weak or focally positive to detect vascular and endothelial differentiation. Additionally, tumors with epithelioid morphology may express keratins and epithelial membrane antigen (EMA) [ 9 , 10 ].…”

“…They may range from well-differentiated lesions with a vasoformative growth pattern and well-developed anastomosing vessels lined by atypical endothelial cells to a poorly differentiated malignancy of epithelioid to spindle cells arranged in solid sheets without a prominent vasoformative pattern [ 8 ]. Reaching the definitive histopathologic diagnosis of angiosarcoma is difficult, making the use of immunohistochemistry (IHC) and a panel of endothelial markers necessary [ 9 , 10 ].…”