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Angiosarcoma

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Evidence-Based Management of Head and Neck Vascular Anomalies

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Abstract

Angiosarcoma (AS) is a rare but aggressive mesenchymal tumor arising from vascular or lymphatic endothelial cells. It accounts for <2% of all soft tissue sarcomas and approximately 10% of sarcomas occurring in the head and neck (Rouhani et al, Cancer 113(3):616–627, 2008; Peng et al, Otolaryngol Head Neck Surg 151(4):627–633, 2014). Primary cutaneous angiosarcomas that arise sporadically represent the majority of these tumors, but they can also occur in the setting of previous radiation, chronic lymphedema, or in the deep soft tissues or parenchymal organs. The incidence has been gradually increasing since the 1970s, which is likely due in part to the greater use of radiation treatment for breast and other cancers. It carries a poor prognosis due to delays in diagnosis, extensive treatment modalities, and a high rate of locoregional recurrence and distant metastasis. Because of its rarity and poor prognosis, most of the available literature is composed of single-institution case series and small clinical trials. The focus of this chapter will be on diagnosis and treatment of head and neck angiosarcomas.

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Authors and Affiliations

  1. Department of Otorhinolaryngology, Mayo Clinic, Rochester, MN, USA

    Katherine A. Lees MD, Christoph M. Prummer MD & Jeffrey R. Janus MD

Authors
  1. Katherine A. Lees MD
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  2. Christoph M. Prummer MD
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  3. Jeffrey R. Janus MD
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Corresponding author

Correspondence to Jeffrey R. Janus MD .

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Editors and Affiliations

  1. Otolaryngology-Head and Neck Surgery, University of Washington, Seattle Children’s Hospital, Seattle, WA, USA

    Jonathan A. Perkins

  2. Otorhinolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, MN, USA

    Karthik Balakrishnan

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Lees, K.A., Prummer, C.M., Janus, J.R. (2018). Angiosarcoma. In: Perkins, J., Balakrishnan, K. (eds) Evidence-Based Management of Head and Neck Vascular Anomalies. Springer, Cham. https://doi.org/10.1007/978-3-319-92306-2_13

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  • DOI: https://doi.org/10.1007/978-3-319-92306-2_13

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-92305-5

  • Online ISBN: 978-3-319-92306-2

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