Angiosarcoma of the nasal septum mimicking reticulohistiocytoma

Angiosarcomas are rare, aggressive tumors of endothelial cells with a high degree of invasiveness and poor survival. Although they arise in the face and scalp of elderly people, the nose represents a rare location with few reports in the literature. Nasal angiosarcoma resembling benign lesion morphologically has been described, but there is no report of angiosarcoma mimicking benign lesion histologically.Here, we report a case of nasal septum angiosarcoma in which the initial misdiagnosis submitted by the referring pathologist was reticulohistiocytoma. Nevertheless, the nasal septum and anterior nasal spine invasion by the tumor led us to suggest extensive surgical treatment: resection of the caudal septum, the anterior nasal spine, the columella, and the philtrum. Thereafter, an L-strut rib cartilage graft reconstructed the septum defect and was lined with a free radial forearm flap, resulting in a satisfactory functional and aesthetic outcome. Histology showed complete resection of a malignant neoplasm of mesenchymal origin, and immunohistochemistry established the diagnosis of epithelial angiosarcoma. The literature regarding this rare presentation of angiosarcoma was reviewed.