Introduction: Angiosarcoma is a rare and highly aggressive soft tissue malignancy originating from vascular and lymphatic endothelial cells. Epithelioid angiosarcoma is the rarest subtype of angiosarcoma, characterized by the proliferation of large polygonal cells with an epithelioid feature. The occurrence of these tumors in the oral cavity is highly uncommon, and immunohistochemistry staining is essential to differentiate epithelioid angiosarcoma from mimicking lesions.
Aim: To present a case of intraoral angiosarcoma with an unusual clinical presentation and behavior and to report, to the best of our knowledge, a first primary appendix epithelioid angiosarcoma with metastasis foci in the oral cavity.
Objectives: To discuss the clinical, histological, and immunochemical features of an unusual case of intraoral angiosarcoma.
Case report: A 53-year-old Saudi female with an uncommon clinical presentation of intraoral angiosarcoma. The patient reported the lesion being painless, slowly growing, and of a six-month duration. The microscopic examination and immunohistochemical evaluation showed epithelioid angiosarcoma. The tumor cells were positive to ERG, FLI 1, and CD31 (focal) and negative to CK HMW, CD45, S100, HMB 45, D2-4, and CD 34.
Discussion: Due to the extremely rare occurrence and non-characteristic presentation of angiosarcoma in the oral cavity, many lesions maybe included in the differential diagnosis. Thus, making the diagnosis of intraoral angiosarcoma difficult.
Keywords: Angiosarcoma; Appendicitis; Epithelioid; Intraoral.
© 2023 The Authors. Published by Elsevier Ltd.