Abstract
• Thyroid angiosarcoma is a very rare malignancy that is mostly seen in elderly of Alpine regions, related to high prevalence of iodine deficient goiter. • It is a very aggressive tumor that can rapidly spread to the cervical lymph nodes, lungs, and brain or can metastasize to the duodenum, small bowel and large bowel. • Metastatic disease is associated with poor prognosis and limits the mean survival time to a few months after diagnosis and surgical treatment. • The first diagnostic challenge is to rule out an angiomatoid anaplastic carcinoma, using a large panel of antibodies. • The second challenge is to specify whether it is a primary or metastatic angiosarcoma especially when other sites are involved, based on clinical and epidemiological criteria.