帕唑帕尼治疗转移性肺上皮样血管内皮瘤——一种合适的治疗选择：病例报告及抗血管生成治疗选择综述
Pazopanib for metastatic pulmonary epithelioid hemangioendothelioma-a suitable treatment option: case report and review of anti-angiogenic treatment options.
作者信息
Semenisty Valeriya, Naroditsky Inna, Keidar Zohar, Bar-Sela Gil
机构信息
Integrated Oncology and Palliative Care Unit, Rambam Health Care Campus and Technion-Israel Institute of Technology, POB 9602, Haifa, 31096, Israel.
Institute of Pathology, Rambam Health Care Campus and Technion-Israel Institute of Technology, Haifa, Israel.
出版信息
BMC Cancer. 2015 May 13;15:402. doi: 10.1186/s12885-015-1395-6.
BACKGROUND
Epithelioid hemangioendothelioma is a rare vascular tumor of borderline or low-grade malignancy. The lungs and liver are the two common primary organs affected. Metastatic disease was reported in more than 100 cases in the literature. However, no firm conclusions can be determined for recommended treatment options.
CASE PRESENTATION
The current case presents a patient with metastatic pulmonary epithelioid hemangioendothelioma to the cervical and mediastinal lymph nodes, lungs and liver that has been treated with pazopanib for more than two years with PET avid complete metabolic response in the mediastinum and lungs, and long-lasting stable disease. Target therapies that block VEGFR have a logical base in this rare malignancy.
CONCLUSIONS
The current case is the first to report objective, long-lasting response to pazopanib.
背景
上皮样血管内皮瘤是一种罕见的具有交界性或低级别恶性的血管肿瘤。肺和肝是两个常见的受影响的原发器官。文献报道了100多例转移性疾病。然而，对于推荐的治疗方案尚无明确结论。
病例报告
本病例为一名患有转移性肺上皮样血管内皮瘤的患者，肿瘤转移至颈部和纵隔淋巴结、肺和肝，接受帕唑帕尼治疗超过两年，纵隔和肺部的PET显示完全代谢缓解，且疾病长期稳定。在这种罕见的恶性肿瘤中，阻断血管内皮生长因子受体（VEGFR）的靶向治疗具有合理依据。
结论
本病例首次报道了对帕唑帕尼的客观、持久反应。
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