Synonyms
Hemangiosarcoma; Malignant angioendothelioma; Malignant hemangioendothelioma; Lymphangiosarcoma; Lymphoedema-associated angiosarcoma
Definition
Angiosarcoma is a malignant tumor that recapitulates the morphological and functional features of endothelium to a variable degree.
Clinical Features
Incidence
Less than 1% of mediastinal malignancies represent angiosarcoma (AS); those examples are more common in young patients (6), unlike the population that typically develops cutaneous angiosarcoma.
Age
In average, the patients with AS present with an age range from 20 to 70 years.
Sex
There is no gender predilection.
Site
AS arises most often in the anterior mediastinum.
Treatment
The surgical approach may facilitate complete surgical resection in anterior mediastinal ASs and avoid local tumor recurrence. Chemotherapy or radiotherapy may be effective.
Outcome
The prognosis is generally poor with a median survival of 42 months and a 5-year overall survival of 43%.
Macroscopy
The...
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References and Further Reading
Contreras, A. l., Lunar, M., & Tamblyn, P. (2010). Mediastinal germ cell tumors with angiosarcomatous component: A report of 12 cases. Human Pathology, 41, 832–837.
Deyrup, A. T., Miettinen, M., North, P. E., Khoury, J. D., Tighiouart, M., Sputi, S. L., Parham, D., Weiss, S. W., & Shehata, B. M. (2009). Angiosarcoma arising in the viscera and soft tissue of children and young adults: A clinicopathologic study of 15 cases. American Journal of Surgical Pathology, 33, 264–269.
Suster, S., Moran, C. A., & Koss, M. N. (1994). Epithelioid hemangioendothelioma of the anterior mediastinum: A clinicopathologic and ultrastructural analysis of 12 cases. American Journal of Surgical Pathology, 18, 871–881.
Weiss, S. W., Antonescu, C. R., & Deyrup, A. T. (2013). Angiosarcoma of soft tissue. In C. D. M. Fletcher, J. A. Bridge, P. C. W. Hegendorn, & F. Mertens (Eds.), WHO classification of tumours of soft tissue and bone (4th ed., pp. 156–158). Lyon: International Agency for Research on Cancer.
Weissferd, T. A., Kalhor, M., Suster, S., & Moran, C. A. (2010). Primary angiosarcoma of the anterior mediastinum: A clinicopathologic and immunohistochemical study of 9 cases. Human Pathology, 12, 1711–1717.
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Fukunaga, M. (2018). Mediastinal Angiosarcoma. In: Allen, T.C., Suster, S. (eds) Pathology of the Pleura and Mediastinum. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-66796-6_4
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DOI: https://doi.org/10.1007/978-3-319-66796-6_4
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