Definition
Angiosarcoma (AS) is a primitive malignant sarcomatous neoplasm of the thyroid with vascular differentiation (Wick et al. 2017).
Clinical Features
Incidence
It is an exceedingly rare tumor; the reported ratio of 2.3% of all malignant neoplasms (Maiorana et al. 1996) is likely to be overrated. Originally reported in patients living in Alpine areas in Switzerland and Italy, where the nodular goiter was endemic, it has been described also in seaside countries, in Eastern countries, and in the USA (Chan et al. 1986; Eusebi et al. 1990; Lamovec et al. 1994; Goh et al. 2003; Ryška et al. 2004; Rotellini et al. 2015). Moreover, the association with a chronic goiter has been challenged.
Age
The mean age is the sixth-seventh decade of life (Wick et al. 2017; Collini et al. 2016; Kuhn et al. 2019).
Sex
The male to female ratio is 4.5:1 in older series (Maiorana et al. 1996), but no sex prevalence is reported in more recent...
References and Further Reading
Chan, Y. F., Ma, L., Boey, J. H., & Yeung, H. Y. (1986). Angiosarcoma of the thyroid. An immunohistochemical and ultrastructural study of a case in a Chinese patient. Cancer, 57, 2381–2388.
Collini, P., Barisella, M., Renne, S. L., Pizzi, N., Mattavelli, D., Stacchiotti, S., & Mattavelli, F. (2016). Virchows Archiv, 469, 223–232.
Eusebi, V., Carcangiu, M. L., Dina, R., & Rosai, J. (1990). Keratin-positive epithelioid angiosarcoma of thyroid. A report of four cases. The American Journal of Surgical Pathology, 14, 737–747.
Goh, S. G., Chuah, K. L., Goh, H. K., & Chen, Y. Y. (2003). Two cases of epithelioid angiosarcoma involving the thyroid and a brief review of non-Alpine epithelioid angiosarcoma of the thyroid. Archives of Pathology & Laboratory Medicine, 127, E70–E73.
Kuhn, E., Ragazzi, M., Ciarrocchi, A., Torricelli, F., de Biase, D., Zanetti, E., Bisagni, A., Corrado, S., Uccella, S., La Rosa, S., Bongiovanni, M., Losito, S., & Piana, S. (2019). Angiosarcoma and anaplastic carcinoma of the thyroid are two distinct entities: A morphologic, immunohistochemical, and genetic study. Modern Pathology, 32, 787–798.
Lamovec, J., Zidar, A., & Zidanik, B. (1994). Epithelioid angiosarcoma of the thyroid gland. Report of two cases. Archives of Pathology & Laboratory Medicine, 118, 642–646.
Maiorana, A., Collina, G., Cesinaro, A. M., Fano, R. A., & Eusebi, V. (1996). Epithelioid angiosarcoma of the thyroid. Clinicopathological analysis of seven cases from non-Alpine areas. Virchows Archiv, 429, 131–137.
Mills, S. E., Stallings, R. G., & Austin, M. B. (1986). Angiomatoid carcinoma of the thyroid gland. Anaplastic carcinoma with follicular and medullary features mimicking angiosarcoma. American Journal of Clinical Pathology, 86, 674–678.
Mills, S. E., Gaffey, M. J., Watts, J. C., Swanson, P. E., Wick, M. R., LiVolsi, V. A., Nappi, O., & Weiss, L. M. (1994). Angiomatoid carcinoma and ‘angiosarcoma’ of the thyroid gland. A spectrum of endothelial differentiation. American Journal of Clinical Pathology, 102, 322–330.
Papotti, M., Volante, M., Negro, F., Eusebi, V., & Bussolati, G. (2000). Thyroglobulin mRNA expression helps to distinguish anaplastic carcinoma from angiosarcoma of the thyroid. Virchows Archiv, 437, 635–642.
Rotellini, M., Vezzosi, V., & Bianchi, S. (2015). Epithelioid angiosarcoma of the thyroid: Report of a case from an Italian non-alpine area and review of the literature. Endocrine Pathology, 26, 152–156.
Ryška, A., Ludviková, M., Szépe, P., & Böör, A. (2004). Epithelioid haemangiosarcoma of the thyroid gland. Report of six cases from a non-Alpine region. Histopathology, 44, 40–46.
Wick, M. R., Eusebi, V., Lamovec, J., & Riška, A. (2017). Angiosarcoma. In R. V. Lloyd, R. Y. Osamura, G. Klöppel, & J. Rosai (Eds.), WHO Classification of tumors of endocrine organs (pp. 129–132). Lyon: IARC.
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Piana, S. (2020). Angiosarcoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5260-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5260-1
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