Angiosarcoma of the thyroid. An immunohistochemical and ultrastructural study of a case in a Chinese patient

Abstract

A case of angiosarcoma (malignant hemangioendothelioma) developing in a chronic goitrous thyroid gland of an elderly Chinese woman is described. Histologically it showed the same classical appearance of angiosarcoma occurring in the skin and soft tissue. The endothelial origin of this tumor was confirmed by demonstrating Factor VIII-related antigen in the neoplastic cells with the immunoperoxidase technique and Weibel-Palade bodies by electron microscopic study. Because of its extreme rarity outside the European Alpine regions, many authorities are reluctant to accept it as a distinct entity and merely consider it as a variant of an undifferentiated carcinoma. Our report not only provides additional evidence that angiosarcoma of the thyroid gland is a specific condition of endothelial origin but also documents the first case among Chinese.

A Case of Hemangiosarcoma in Thyroid with Severe Anemia due to Bone Marrow Metastasis

“…The highest prevalance is in Switzerland and the Alpine regions [1]. This is the first case reported from Turkey according to MEDLİNE search.…”

“…Aggressive tumors can rapidly spread to the lungs, lymph nodes and the brain [1,[6][7][8]. Tumors can also metastasize to the duodenum, small and large bowel and can cause severe bleeding [6].…”

“…Metastatic disease is associated with poor prognosis and limits the mean survival time to a few months after diagnosis and surgical treatment [1,6,8,10,11]. Recently, better survival rates have been reported in the literature [7,12].…”

“…The incidence is highest in European Alpine regions and it constitutes 2-10% of all malignant thyroid tumors in Switzerland, Austria and northern Italy [1,2]. In other parts of the world it is extremely rare.…”

Angiosarcoma of the Thyroid and Regional Lymph Node Metastasis

“…The incidence is highest in European Alpine regions and it constitutes 2-10 % of all malignant thyroid tumors in Switzerland, Austria and northern Italy. In other parts of the world it is extremely rare [2,3]. In an analysis of 7023 thyroidectomy specimens reported from Singapore, only two angiosarcomas have been reported [4].…”