Thyroid angiosarcoma: a rare malignancy - case report and systematic review of the literature

Thyroid angiosarcoma is a rare, highly aggressive malignancy of endothelial origin, characterized by rapid growth, invasive behavior, and a high metastatic potential. While most cases are reported from the Alpine regions of Europe, its occurrence elsewhere is exceedingly rare. The aim of our review is to report a case of thyroid angiosarcoma and provide a systematic review of the literature, focusing on clinical presentation, diagnostic strategies, and treatment outcomes.

A literature search of thyroid angiosarcoma case reports and series was performed according to PRISMA guidelines. Data on clinical features, diagnostic and treatment methods, and clinical outcomes were retrieved and analyzed. Kaplan-Meier survival curve was constructed to assess overall survival.

A systematic review of 72 cases of thyroid angiosarcoma reported in 52 articles was conducted. The median patient age was 66 years, with female predominance (4:3). Most patients presented with a rapidly enlarging thyroid mass and compressive symptoms. Diagnosis required a combination of histopathology and radiologic imaging, with Fine Needle Aspiration Cytology as the initial tool in 47% of patients. Total thyroidectomy was performed in 67% of cases, and 42% of patients received adjuvant therapies. The prognosis remains poor, with a median overall survival of 28 months and a 3-year survival rate of 40%.

This case highlights the aggressive nature of thyroid angiosarcoma, and the challenges associated with its diagnosis and management. The literature review underscores the need for standardized management protocols and further research to improve patient outcomes.