Treatment strategies and survival outcomes in hepatic epithelioid hemangioendothelioma: A systematic review.

Abstract

e16239 Background: The hepatic epithelioid hemangioendothelioma, or HEHE, is a very rare type of blood vessel cancer that shows different symptoms and does not have a standard treatment plan. Emerging non-surgical therapy and prognostic indicators are still not well described, although liver transplantation (LT) and liver resection (LR) are frequently pursued. This systematic review looks at the results and survival rates of different groups of HEHE patients from published studies to help doctors make better treatment choices based on evidence. Methods: The purpose of this study was to conduct a systematic review of 18 studies that included 1,105 patients who had histologically confirmed HEHE. These studies covered both surgical and non-surgical treatment modalities. Data were collected on demographics, treatment approaches, overall survival (OS), disease-free survival (DFS), recurrence rates, and the duration of patient follow-up. Only studies that included at least five patients and data that could be extracted from the outcomes were considered for inclusion. Results: 18 studies, which included 1105 patients diagnosed with HEHE, were analyzed. The median age was 46 years, and approximately 60–65% of the patients were female. The treatments included liver resection (LR, LT) and non-surgical methods like TACE and sirolimus. Surgical interventions were associated with improved survival rates. The OS rates for liver resection were 96%, 86%, and 88% after one, three, and five years, respectively. Conversely, liver transplantation exhibited OS rates of 91%, 73–80%, and 73–75% at the same intervals. Non-surgical management, in contrast, presented more heterogeneous outcomes, with five-year OS rates fluctuating between 37% and 63%. 10 years after treatment, the OS rates were low for all groups, with liver transplantation showing a 35% survival rate, liver resection at 20%, and non-surgical treatments at 23%. 5-year disease-free survival ranged from 37.4% to 60.1%. Worse survival rates were linked to having bone metastases, a larger tumor size, older age, and lung involvement. It is important to note that patients receiving surgical interventions exhibited significantly improved long-term outcomes when contrasted with those managed non-surgically (5-year OS: 88% versus 49%). The median follow-up duration was 39 months across the studies, ranging from 8 to 76.6 months. Conclusions: Surgical intervention improves HEHE survival, especially without bone metastases. Liver resection had high OS rates, whereas LT was still a possibility for some patients. Nonsurgical therapy had mixed results, suggesting that patients require customized treatment. Bone metastases, age ≥60, and symptom load were negative prognostic factors. This rare malignancy requires prospective registries and global data sharing to improve therapeutic decision-making.