Department of Otolaryngology/Head and Neck Surgery, Jichi Medical University School of Medicine, Shimotsuke, Japan.
Head Neck. 2013 Oct;35(10):E317-20. doi: 10.1002/hed.23160. Epub 2012 Sep 28.
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.
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.
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.
头颈部上皮样血管内皮细胞瘤（EHE）是一种罕见的恶性肿瘤，具有低级别和高级别恶性肿瘤的多种生物学行为特征。一组被称为“高危”EHE 的肿瘤因其大小和有丝分裂活性而被识别，与不良的临床病程和预后不良相关。
我们描述了首例也是迄今为止颈部最大的一例高危 EHE 的治疗情况。尽管进行了广泛的切除，但正如预期的那样，在手术后 9 个月出现了复发。然而，肿瘤表达血管内皮生长因子（VEGF）及其受体 2，表明抗 VEGF 治疗在这类病例中的应用潜力。
头颈部高危 EHE 表达 VEGF 及其受体的发现为靶向分子治疗的发展提供了进一步的支持。
