Re: Autoimmunity in sudden sensorineural hearing loss. . .by Cadoni et al. (multiple letters)

Objectives-The purpose of this report is to describe an example of angiosarcoma (AS) of the submandibular salivary gland. The clinical and immunohistopathologic features of these lesions are also reviewed.Material and Methods-A 17-year-old male high school student was admitted to our clinic with a 1-year history of a slow growing, tender mass in the left submandibular region. Physical examination on initial presentation revealed a diffuse soft tissue mass 6 cm in diameter involving the left submandibular region. MRI analysis revealed a mass located superolateral to the submandibular salivary gland, measuring 4.0 x 2.0 cm(2). The mass was excised completely together with the left submandibular salivary gland. Histopathologic analysis led to a diagnosis of AS. Immunohistochemical studies were also used to determine endothelial cell differentiation. Owing to the lateralized nature of the lesion, a left functional neck dissection was performed and postoperative radiotherapy was planned.Results-AS is a malignant tumor of endothelial cell origin that may occur in any region of the body. The commonest sites include the extremities and the retroperitoneal space, with only 4% of AS tumors arising in the head and neck area. The submandibular salivary gland is an extremely rare location for this tumor. Based on a literature review, this case report represents only the second reported case of AS of the submandibular salivary gland.Conclusions-In most cases, radical surgery and postoperative radiotherapy are advocated to treat patients with AS tumors, with lymph node clearance recommended in cases of lateralized lesions. In some patients, distant metastasis may occur after many years, which necessitates long-term follow-up. The prognosis is poor in most cases of AS.