Introduction:Thyroid angiosarcoma is a malignant neoplasm, which usually shows local aggressive behavior and associated with a high recurrence rate. It was originally described in the Alpine region and extremely rare in other parts of the world.Case Presentation:An asymptomatic 61-year-old woman presented with a right lobe thyroid nodule with fine needle aspiration cytology indicating “suspicious for malignancy” was reported in our study. Histological examination revealed a vascular 35mm neoplasm with areas of necrosis. Immunohistochemistry staining had negative results for MNF, CAM5.2, CD34, thyroglobulin, and positive for CD31. The findings described were compatible with angiosarcoma diagnosis. Besides, a 4mm papillary microcarcinoma was found in the left lobe. Computerized tomography (CT) scan performed about two months after the operation, showed a right neck nodular lesion, conditioning tracheal deviation. At our institution, the study performed was consistent with local recurrence (angiosarcoma). Tumor excision was performed and invasion to larynx, trachea and esophagus was detected intraoperatively. Histopathologic examination confirmed tumor recurrence and the patient was submitted to radiotherapy (60 Gy), completed four years ago. There is not, so far, any evidence of tumor recurrence.Conclusions:We described a rare case of a usually aggressive thyroid angiosarcoma in a patient living in a non-Alpine region, with an unusual favorable outcome after the operation and radiotherapy.

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