O Kyu Noh1, Soon Sun Kim2, Min Jae Yang3, Sun Gyo Lim3, Jae Chul Hwang3, Hyo Jung Cho3, Jae Youn Cheong3, Sung Won Cho3. 1. Department of Radiation Oncology, Ajou University School of Medicine, Suwon, Korea; Department of Biomedical Informatics, Ajou University School of Medicine, Suwon, Korea; Office of Biostatistics, Ajou Research Institute for Innovative Medicine, Ajou University Medical Center, Suwon, Korea. 2. Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea. Electronic address: soonsunkim@aumc.ac.kr. 3. Department of Gastroenterology, Ajou University School of Medicine, Suwon, Korea.
Abstract
BACKGROUND: Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor of vascular origin with an unknown etiology, a low incidence, and a variable natural course. We evaluated the management and prognosis of HEH from the Surveillance, Epidemiology and End Results (SEER) program and changes in treatment modalities of HEH over 30 years. METHODS: From 1973 to 2014 in the SEER database, we selected patients diagnosed with HEH. We analyzed the clinical characteristics, patterns of management, and clinical outcomes of patients with HEH. RESULTS: We identified 79 patients with HEH (median age: 54.0 years; male to female ratio: 1:2.6). The initial extent of disease was local in 22 (27.8%) patients, regional metastasis in 22 (27.8%), distant metastasis in 31 (39.2%) and unknown in 4 (5.1%). The median size of primary tumor was 3.85 cm (interquartile range, 2.50-7.93 cm). Among 74 patients with available management data, the most common management was no treatment (29/74, 39.2%), followed by chemotherapy only (22/74, 29.7%), liver resection-based (13/74, 17.6%), and transplantation-based therapy (6/74, 8.1%). The 5-year cancer-specific survival rate was 57.8%. Patients who underwent surgical treatment had significantly higher survival than those who underwent non-surgical treatment (5-year survival; 88% vs. 49%, P = 0.019). Multivariate analysis revealed that surgical therapy was the only independent prognostic factor for survival (hazard ratio: 0.20, P = 0.040). CONCLUSIONS: Resection or liver transplantation is worth considering for treatment of patients with HEH.
BACKGROUND:Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor of vascular origin with an unknown etiology, a low incidence, and a variable natural course. We evaluated the management and prognosis of HEH from the Surveillance, Epidemiology and End Results (SEER) program and changes in treatment modalities of HEH over 30 years. METHODS: From 1973 to 2014 in the SEER database, we selected patients diagnosed with HEH. We analyzed the clinical characteristics, patterns of management, and clinical outcomes of patients with HEH. RESULTS: We identified 79 patients with HEH (median age: 54.0 years; male to female ratio: 1:2.6). The initial extent of disease was local in 22 (27.8%) patients, regional metastasis in 22 (27.8%), distant metastasis in 31 (39.2%) and unknown in 4 (5.1%). The median size of primary tumor was 3.85 cm (interquartile range, 2.50-7.93 cm). Among 74 patients with available management data, the most common management was no treatment (29/74, 39.2%), followed by chemotherapy only (22/74, 29.7%), liver resection-based (13/74, 17.6%), and transplantation-based therapy (6/74, 8.1%). The 5-year cancer-specific survival rate was 57.8%. Patients who underwent surgical treatment had significantly higher survival than those who underwent non-surgical treatment (5-year survival; 88% vs. 49%, P = 0.019). Multivariate analysis revealed that surgical therapy was the only independent prognostic factor for survival (hazard ratio: 0.20, P = 0.040). CONCLUSIONS: Resection or liver transplantation is worth considering for treatment of patients with HEH.
Authors: Anna M Frezza; Vinod Ravi; Salvatore Lo Vullo; Bruno Vincenzi; Francesco Tolomeo; Tom Wei-Wu Chen; Pawel Teterycz; Giacomo G Baldi; Antoine Italiano; Nicolas Penel; Antonella Brunello; Florance Duffaud; Nadia Hindi; Shintaro Iwata; Alannah Smrke; Alexander Fedenko; Hans Gelderblom; Winette Van Der Graaf; Aurore Vozy; Elizabeth Connolly; Massimiliano Grassi; Robert S Benjamin; Javier-Martin Broto; Giovanni Grignani; Robin L Jones; Akira Kawai; Andrzej Tysarowski; Luigi Mariani; Paolo G Casali; Silvia Stacchiotti Journal: Cancer Med Date: 2021-03-13 Impact factor: 4.452