Epithelioid Hemangioendothelioma: Incidence, Mortality, Prognostic Factors, and Survival Analysis Using the Surveillance, Epidemiology, and End Results Database

Abstract

Background. Although epithelioid hemangioendothelioma (EHE) is a rare and aggressive vascular tumor, its demographic characteristics remain unclear. We used the surveillance, epidemiology, and end results (SEER) database to determine the clinical features, incidence, and prognostic factors associated with overall survival in patients with EHE. Methods. The demographic and clinical data of patients with EHE were extracted from the SEER database to calculate the incidence of EHE and survival rate in these patients. The Cox proportional hazards model and Kaplan-Meier method were used to analyze the prognostic factors of overall survival in these patients. A nomogram and time-dependent receiver operating characteristic (ROC) curve were employed to predict the 3-and 5-year survival rate. Results. The overall incidence rate (IR) of EHE was 0.230 (95%confidence interval ½CI = 0:201 -0:263) per 1,000,000 person-years. According to the age-stratified IR, the highest age-adjusted IR was observed in patients aged 60-79 years (0.524 per 1,000,000 person-years, 95%CI = 0:406 -0:665). The majority (30.8%) of the tumors were located in the soft tissue and skin, followed by lesions in the abdomen (28%), respiratory system (19%), bone and joint (8.6%), and others. The 5-year overall survival rate was 55.6% (95%CI = 32:8 -73:5%).

Incidence, demographics, and survival of malignant hemangioendothelioma in the United States

“…To our knowledge this is the largest cohort of malignant hemangioendothelioma/EHE patients reported to date. The US incidence rate in this series (120 new cases diagnosed in the US per year) is similar to that recently reported in France and approximately double the previously reported US incidence 1,2 . Prior differences likely reflected ascertainment bias, with earlier US data under‐representing the true incidence of EHE.…”

“…The US incidence rate in this series (120 new cases diagnosed in the US per year) is similar to that recently reported in France and approximately double the previously reported US incidence. 1,2 Prior differences likely reflected ascertainment bias, with earlier US data underrepresenting the true incidence of EHE. We also found equal numbers of cases in men and women, whereas prior reports have suggested a female preponderance.…”

“…Malignant hemangioendotheliomas are ultra‐rare vascular sarcomas with variable clinical behavior that can be either indolent or aggressive; the majority of malignant hemangioendotheliomas are epithelioid (EHE). The overall incidence has previously been reported as 0.38 per 1,000,000 person‐years in a large European database, 1 but has been reported to be only 0.23 per 1,000,000 person‐years in the United States (US) 2 . Most (83%) are molecularly characterized by the presence of a WWTR1‐CAMTA1 gene fusion and have a 5‐year overall survival (OS) of 59% in case series 3–5 .…”

“…The overall incidence has previously been reported as 0.38 per 1,000,000 person-years in a large European database, 1 but has been reported to be only 0.23 per 1,000,000 personyears in the United States (US). 2 Most (83%) are molecularly characterized by the presence of a WWTR1-CAMTA1 gene fusion and have a 5-year overall survival (OS) of 59% in case series. [3][4][5] A smaller subset has a YAP1-TFE3 gene fusion, associated with better 5-year survival of 86%.…”

Anteroposterior Combined Surgery of a Rare Massive Epithelioid Hemangioendothelioma at the Cervicothoracic Junction

“…Epithelioid hemangioendothelioma, a rare borderline tumor consisting of epithelioid, endothelial, and dendritic cells, was first reported by Weiss-Enzinger in 1982 [ 1 ]. Its estimated prevalence is 1:1,000,000 in the general population [ 2 ]. Non-soft tissue epithelioid hemangioendothelioma can also be seen in different organs.…”