Treatment and Prognosis of Hepatic Epithelioid Hemangioendothelioma: A SEER Database Analysis

Abstract

Background Hepatic epithelioid hemangioendothelioma (HEH) is a rare tumor that can affect multiple organs. Little is known about the pathophysiology, clinical course and management of this disease. The aim of this study is to determine survival rates and elucidate the role of various prognostic factors and therapeutic modalities as compared to surgery on patients with HEH. Methods A retrospective analysis on patients diagnosed with HEH between 2004 and 2016 was performed utilizing the SEER database. Kaplan–Meier curves were constructed to determine overall and cancer‐specific survival, and the log‐rank test was used to compare between groups. To explore prognostic factors and treatment outcomes, univariable and multivariable Cox proportional hazard models were developed. Results A total of 353 patients with HEH (median age: 50.4 years) were identified. The most common surgery performed was liver resection (90.8%). One‐year OS in the surgical group and non‐surgical group was 86.6% and 61.0%, respectively, while 5‐year OS was 75.2% and 37.4%, respectively. On multivariable analysis, surgery emerged as a favorable prognostic factor [HR (95%CI): 0.404 (0.215–0.758) p value = 0.005]. Age > 65 years [HR (95%CI): 2.548 (1.442–4.506) p value = 0.001] and tumor size > 10 cm [HR (95%CI): 2.401 (1.319–4.37) p value = 0.004] were shown to be poor survival prognostic factors. Conclusion HEH is a rare disease that is poorly understood. Surgical intervention is associated with improved survival rates. Multicenter prospective collaborations are needed to improve our limited knowledge about this neoplasm and determine the optimal treatment strategy.

The challenging surgical management of hepatic epithelioid hemangioendothelioma: a narrative review

“…In the first study (N=56, period 1973-2014), 1-year overall survival rates were 57% vs. 67% vs. 80% in patients undergoing resection, no surgical approaches, and LT, respectively (31). The more recent analysis (N=79, period 2004-2016) reported 1-and 5-year overall survival of 87% vs. 75% and 61% vs. 37% in the surgical vs. the nonsurgical group, respectively (62).…”

Sirolimus combined with interferon-alpha 2b therapy for giant hepatic epithelioid hemangioendothelioma: a case report

“…Although, spontaneously tumor regression of HEH was reported, we found that the tumor progressed slowly for most patients ( 3 , 4 , 17 ). Surgical resection has been reported with good long-term results ( 1 , 18 ). However, due to the multiple intrahepatic lesions and extrahepatic metastasis, radical surgery was impossible for most HEH patients.…”

“…Hepatic epithelioid hemangioendothelioma (HEH) is a very rare tumor originated from vascular endothelial cells, with unpredictable malignancy ( 1 , 2 ). The rarity of the disease limits the implementation of clinical trials and no standard treatment paradigm has been established yet.…”

Case report: Successful treatment with the combined therapy of interferon-alpha 2b and anlotinib in a patient with advanced hepatic epithelioid hemangioendothelioma

“…While considering the risk of post-transplantation recurrence and the potential indolence of HEH, the value of this procedure has also been doubted recently (7). Surgical resection has also been reported with good long-term results (1,23). However, according to our previous study, surgical resection was impossible for most patients with HEH due to the multiple intrahepatic lesions (5).…”

“…Hepatic epithelioid hemangioendothelioma (HEH) is an extremely rare tumor that originates from vascular endothelial cells and has unpredictable malignancy (1,2). Due to the rarity of the disease, clinical trials are very difficult to conduct.…”