Pazopanib for pulmonary epithelioid hemangioendothelioma: 1 Pazopanib for metastatic pulmonary epithelioid hemangioendothelioma - a suitable 1 treatment option: Case report and review of anti-angiogenic treatment options

26 Background. Epithelioid hemangioendothelioma is a rare vascular tumor of borderline or 27 low-grade malignancy. The lungs and liver are the two common primary organs affected. 28 Metastatic disease was reported in more than 100 cases in the literature. However, no firm 29 conclusions can be determined for recommended treatment options. 30 Case presentation. The current case presents a patient with metastatic pulmonary epithelioid 31 hemangioendothelioma to the cervical and mediastinal lymph nodes, lungs and liver that has 32 been treated with pazopanib for more than two years with partial response and long-lasting 33 stable disease. Target therapies that block VEGFR have a logical base in this rare malignancy. 34 Conclusion. The current case is the first to report objective, long-lasting response to 35 pazopanib. 36 37