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Head Neck. 2013 Oct;35(10):E317-20. doi: 10.1002/hed.23160. Epub 2012 Sep 28.

Case of high-risk epithelioid hemangioendothelioma of the neck.

Author information

1
Department of Otolaryngology/Head and Neck Surgery, Jichi Medical University School of Medicine, Shimotsuke, Japan.

Abstract

BACKGROUND:

Epithelioid hemangioendotheliomas (EHEs) of the head and neck region are uncommon malignant neoplasms that exhibit various biologic behaviors characteristic of both low- and high-grade malignancy. A subgroup of EHEs identified as "high-risk" EHEs because of their size and mitotic activity is associated with an unfavorable clinical course and poor prognosis.

MATERIALS AND RESULTS:

We describe the treatment of the first and, in terms of size, largest case of high-risk EHE arising from the neck. Despite wide excision, recurrence occurred 9 months after surgery, as had been expected. However, the tumor was found to express both vascular endothelial growth factor (VEGF) and VEGF receptor 2, indicating the potential of anti-VEGF therapy in the treatment of such cases.

CONCLUSION:

The finding that a high-risk EHE arising from the head and neck region is characterized by expression of VEGF and its receptor provides further support for the development of targeted molecular therapies.

KEYWORDS:

angiosarcoma; epithelioid hemangioendothelioma; neck; vascular endothelial growth factor

PMID:
23019156
DOI:
10.1002/hed.23160
[Indexed for MEDLINE]

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