Hemangiosarcoma; Malignant angioendothelioma; Malignant hemangioendothelioma; Lymphangiosarcoma; Lymphoedema-associated angiosarcoma
Angiosarcoma is a malignant tumor that recapitulates the morphological and functional features of endothelium to a variable degree.
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.
In average, the patients with AS present with an age range from 20 to 70 years.
There is no gender predilection.
AS arises most often in the anterior mediastinum.
The surgical approach may facilitate complete surgical resection in anterior mediastinal ASs and avoid local tumor recurrence. Chemotherapy or radiotherapy may be effective.
The prognosis is generally poor with a median survival of 42 months and a 5-year overall survival of 43%.
References and Further Reading
- 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.PubMedCrossRefGoogle Scholar
- 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.Google Scholar