Prognostic Factors and Surgical Indications of Pulmonary Epithelioid Hemangioendothelioma: A Review of the Literature

Background Pulmonary epithelioid hemangioendothelioma is a rare vascular tumor of low-grade malignancy, the prognosis of which remains unpredictable. The purpose of this analysis based on 80 patients was to determine prognostic factors and to evaluate results of surgery. Methods We recorded data of 75 patients from reports published in the English and French literature using the terms “intravascular bronchoalveolar tumor” or “pulmonary epithelioid hemangioendothelioma” or a combination of both. We added to this database 5 more cases of pulmonary epithelioid hemangioendothelioma operated on in our thoracic surgery department from 1989 to 2005. Univariate and multivariate analyses of prognostic factors were performed using the log rank test and the Cox model. The factors we tested were age, sex, clinical symptoms, biologic and radiologic findings, and surgical treatment. Results There were 49 women and 31 men with a mean age of 39.7 years (range, 7 to 72 years). The 5-year survival probability was 60% (range, 47% to 71%). Univariate analysis showed that loss of weight, anemia, pulmonary symptoms, and more particularly pleural hemorrhagic effusions were significant factors of poor prognosis, with a median survival of less than 1 year. Multivariate analysis showed a statistically worse survival in patients with hemorrhagic symptoms (hemoptysis, p < 0.0001; pleural effusion, p < 0.0001). Conclusions Pulmonary epithelioid hemangioendothelioma typically occurs among young patients. Surgery can be proposed in cases of unilateral single or multiple nodules. There is no single effective treatment in cases of bilateral multiple nodules. Lung transplantation should be evaluated in patients with vascular aggressivity with pleural hemorrhagic effusion and anemia.