Primary thyroid angiosarcoma in a non-endemic region – a rare case

Abstract

Thyroid angiosarcoma (AS) is a specific type of vascular tumor that arises from the endothelial cells, being highly aggressive, with increased recurrence rates and metastasis. It is characterized by positive endothelial markers and co-positive markers for cytokeratins and epithelial membrane antigen. We are describing the case of a 76-year-old patient who presented to the hospital for dyspnea and asthenia. The clinical and paraclinical investigations confirmed the presence of a right large thyroid nodule, which turned out positive at immunostaining for AS. Even if thyroid AS is a rare type of tumor, mainly described in the Alps, one has to take into account that it can evolve in any other regions and should be considered as a differential diagnosis.

Neck Surgery for Non-Well Differentiated Thyroid Malignancies: Variations in Strategy According to Histopathology

“…They usually occur in the context of long-standing multinodular goiters [81,82], mainly in the alpine region although they have been described elsewhere [83,84]. While some authors have considered them as an anaplastic or undifferentiated carcinoma with angiomatoid characteristics [85][86][87], there is now broad consensus that angiosarcomas arise from endothelial cells, characterized by positivity for the CD31 marker and erythroblast transformation-specific related gene (ERG) [77,85,[88][89][90]. Clinical and radiological features are nonspecific.…”