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Updated: May 18, 2026
Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
Case of high-risk epithelioid hemangioendothelioma of the neck
Shingo Matsuzawa1, Takeharu Kanazawa, Takehiko Yamaguchi
1Department of Otolaryngology/Head and Neck Surgery, Jichi Medical University School of Medicine, Shimotsuke, Japan.
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.
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