Abstract
BACKGROUND: Epithelioid hemangioendothelioma (EHE) is an ultrarare vascular sarcoma. At present, the epidemiological and clinical characteristics and prognostic factors are still unclear. Our study attempted to describe clinical features, investigate the prognostic indicators, and establish the nomogram prediction model based on the Surveillance, Epidemiology, and End Results (SEER) database for EHE patients. METHODS: The patients diagnosed with EHE from 1986 to 2018 were collected from the SEER database and were randomly divided into a training group and a validation group at a ratio of 7 : 3. The Cox proportional hazard models were used to determine the independent factors affecting prognosis and establish a nomogram prognostic model to predict the survival rates for patients with EHE. The accuracy and discriminative ability of the model were measured using the concordance index, receiver operating characteristic curves, and calibration curves. The clinical applicability and application value of the model were evaluated by decision curve analysis. RESULTS: The overall age-adjusted incidence of EHE was 0.31 patients per 1,000,000 individuals, with a statistically significant difference per year. Overall survival at 1, 5, and 10 years for all patients was 76.5%, 57.4%, and 48.2%, respectively. Multivariate Cox regression analysis identified age, tumour stage, degree of tissue differentiation, surgical treatment, chemotherapy, and radiotherapy as independent factors affecting prognosis (P < 0.05). The C-index values for our nomogram model of training group and validation group were 0.752 and 0.753, respectively. The calibration curve was in good agreement with the actual observation results, suggesting that the prediction model has good accuracy. The decision curve analysis indicated a relatively large net benefit. CONCLUSIONS: The nomogram model may play an important role in predicting the survival rate for EHE patients, with good concordance and accuracy, and can be applied in clinical practice.