Pathology of the Pleura and Mediastinum

2018 Edition
| Editors: Timothy Craig Allen, Saul Suster

Mediastinal Angiosarcoma

  • Masaharu FukunagaEmail author
Reference work entry


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.

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%.



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References and Further Reading

  1. 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.PubMedPubMedCentralCrossRefGoogle Scholar
  2. 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
  3. 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.PubMedCrossRefGoogle Scholar
  4. 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
  5. 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.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of PathologyThe Jikei University School of MedicineTokyoJapan