Abstract
Primary thyroid angiosarcoma is a rare malignant tumor and characterized by its prevalence in Alpine regions of Central Europe, close relation to longstanding goiter, and aggressive clinical course with dismal prognosis. We describe the case of an 83-year-old Chinese female who lives in the coastal area of Taiwan. She came to our hospital due to a progressively enlarged mass at her anterior neck. The sonography of the thyroid revealed a well-circumscribed mass in the left lobe. She underwent left hemithyroidectomy. The diagnosis of angiosarcoma of the thyroid was made and further confirmed at a different institution. To the best of our knowledge, this is the first case of primary thyroid angiosarcoma reported from Taiwan and the sixth Asian afflicted with primary thyroid angiosarcomas in the English literatures. The literature search in the PubMed database identified 58 cases who had histologically proven primary thyroid angiosarcomas. A preliminary analysis of epidemiological presentation, clinical features, immunohistochemical characters, and prognosis of primary thyroid angiosarcoma was proposed. The prognosis of this rare neoplasm is surprisingly favorable in comparison with that of its morphological similar, the anaplastic thyroid carcinoma. The use of a comprehensive panel of immunohistochemical stains, including at least two endothelial markers (especially CD31 and ERG) and PAX-8, in combination with thorough light microscopic examination may assist in the discrimination between these two tumors.
Highlights
Primary sarcomas are rarely seen in the thyroid gland, and the reported frequency ranges from 0.01% to 1.5% [1]
Papotti et al stated that thyroglobulin messenger RNA which was detected at low levels in anaplastic carcinoma of thyroid was not found in angiosarcoma [15, 16]
Kuhn et al defined angiosarcoma and anaplastic carcinoma of the thyroid as two distinct entities based on considerable differences in genetic alterations in a recent study; the TP53 somatic mutations and TERT promoter mutations which are frequently identified in anaplastic carcinoma are not found in angiosarcoma [17]
Summary
Primary sarcomas are rarely seen in the thyroid gland, and the reported frequency ranges from 0.01% to 1.5% [1]. Primary thyroid angiosarcoma (TAS) was originally reported in patients who resided in the Alpine countries of Central Europe where TAS comprises 2-10% of all thyroid malignancies in different series [2, 3]. Sporadic cases have been described in non-Alpine areas [2]. Its predilection in these endemic goitrous regions and frequent coexistence with multinodular goiters suggest that the dietary iodine deficiency may be a potential cause [2]. To the best of our knowledge, this is the first case reported from Taiwan and the sixth Asian afflicted with TAS in the English literatures [5, 7,8,9,10]
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