VEGF-VEGFR pathway seems to be the best target in hepatic epithelioid hemangioendothelioma: A case series with review of the literature |
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| Institution: | 1. Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, Turkey;2. Division of Medical Oncology, Department of Internal Medicine, Medeniyet University School of Medicine, Istanbul, Turkey;3. Department of Nucleer Medicine, Marmara University School of Medicine, Istanbul, Turkey;4. Department of Pathology, Medeniyet University School of Medicine, Istanbul, Turkey;1. Department of Radiology, Neuroradiology Unit, Fondazione Poliambulanza Hospital, Brescia, Italy;2. Department of Pathology, Fondazione Poliambulanza Hospital, Brescia, Italy;3. Department of Neurosurgery, University of Brescia, Brescia, Italy;4. Department of Oncology, Fondazione Poliambulanza Hospital, Brescia, Italy;1. Clinical Nutrition and Dietetics Unit, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy;2. Department of Oncology, San Bortolo General Hospital, Vicenza, Italy;3. Italian Federation of Volunteer-based Cancer Organizations, Rome, Italy;4. Medical Oncology Unit, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy;5. Department of Internal Medicine and Medical Therapy, University of Pavia, Pavia, Italy;1. Department of Neurosciences, College of Medicine and Philippine General Hospital, University of the Philippines, Manila, Manila, Philippines;2. Division of Medical Oncology, Department of Medicine, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Philippines;3. Department of Radiology, College of Medicine and Philippine General Hospital, University of the Philippines, Manila, Manila, Philippines;1. Marmara University School of Medicine, Department of Internal Medicine, Division of Medical Oncology, Istanbul, Turkey;2. Marmara University School of Medicine, Department of Pulmonary Medicine, Istanbul, Turkey;3. Marmara University School of Medicine, Department of Cardiology, Istanbul, Turkey |
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| Abstract: | Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor originating from endothelial cells. Clinical 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 patients 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 combination 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 for 18 months with stable disease, and is still being followed without any treatment. Although targeting VEGF-VEGFR pathway seems to be the best approach in HEHE, randomized studies are urgently needed to support these findings. |
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