A case of rapidly progressing primary thyroid angiosarcoma in a Japanese man

Abstract

Primary thyroid angiosarcoma is a very rare and aggressive disease, originally reported in patients from the Swiss Alpine region, but is rare in other parts of the world. We describe a Japanese case of primary angiosarcoma of the thyroid in a 64-year-old man, who presented with a rapidly enlarging painful neck mass. Fine-needle aspiration biopsy was performed, and the results were interpreted as malignant. FDG-PET-CT demonstrated a necrotic lesion on the right side of the neck with high FDG uptake. The patient underwent a total thyroidectomy. Pathological findings revealed a proliferation of atypical cells lined vascular spaces. Immunohistochemistry staining (IHCS) was positive for factor VIII, and CD31, but not CD34, and negative for CK 5, CK 7, and CK 20. From these findings, it was diagnosed as an epithelioid angiosarcoma. Two weeks after the operation, bilateral bloody pleural effusion was observed, and he died 3 months after the surgery.