ARTICLE IN PRESS JID: YMCN [mUS1Ga;March 19, 2020;11:0] Current Problems in Cancer xxx (xxxx) xxx Contents lists available at ScienceDirect Current Problems in Cancer journal homepage: www.elsevier.com/locate/cpcancer VEGF-VEGFR pathway seems to be the best target in hepatic epithelioid hemangioendothelioma: A case series with review of the literature Tugba Akin Telli a,∗ , Ilker Nihat Okten b , Tu ˘ gba Basoglu Tuylu a , Nazim Can Demircan a , Rukiye Arikan a , Ozkan Alan a , Ozlem Ercelep a , Tunc Ones c , Aysenur Toksoz Yildirim d , Faysal Dane a , Perran Fulden Yumuk a a Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, Turkey b Division of Medical Oncology, Department of Internal Medicine, Medeniyet University School of Medicine, Istanbul, Turkey c Department of Nucleer Medicine, Marmara University School of Medicine, Istanbul, Turkey d Department of Pathology, Medeniyet University School of Medicine, Istanbul, Turkey a b s t r a c t Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor originating from endothelial cells. Clini- cal aspect of the disease covers a wide spectrum from a low-grade tumor to a fatal cancer. Most common sites of EHE are reported as lung, liver and bone. Hepatic EHE (HEHE) is a clinical form with an incidence of less than 1 person in a million. Due to rarity of the disease, there is no standard therapy established. Surgery and liver transplantation still seem to be the best approach if possible. However, most of the pa- tients present with unresectable or metastatic disease. Many conventional chemotherapeutic agents and antiangiogenic drugs have been reported previously in the literature with inconsistent outcomes. Here we report 4 cases of HEHE, who benefit distinctly from anti-VEGF treatments in different settings. While com- bination of paclitaxel and bevacizumab resulted in partial response in 3 patients, one of them also achieved long-term disease stabilization with bevacizumab maintenance with no adverse event. Two of the patients had clear benefit from pazopanib during the course of disease. One patient was treated with thalidomide ✩ Funding: The author(s) received no financial support for the research, authorship, and/or publication of this article. ✩✩ Conflicts of interest: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. ∗ Correspondence to: Tugba Akin Telli, Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, Turkey. https://doi.org/10.1016/j.currproblcancer.2020.100568 0147-0272/© 2020 Elsevier Inc. All rights reserved. Please cite this article as: T.A. Telli, I.N. Okten and T.B. Tuylu et al., VEGF-VEGFR pathway seems to be the best target in hepatic epithelioid hemangioendothelioma: A case series with review of the literature, Current Problems in Cancer, https://doi.org/10.1016/j.currproblcancer.2020.100568