Epithelioid hemangioendothelioma in the thorax

Abstract

Little is known about prognostic factors in epithelioid hemangioendothelioma (EHE). We aimed to identify prognostic factors among various clinicopathologic and imaging features of thoracic EHEs.Forty-two patients (male:female = 20:22; median age, 49 years) of EHEs with (n = 19) and without (n = 23) thoracic involvement were included. We reviewed electronic medical records for clinical information and computed tomography (CT) features for thoracic involvement. Differences in demographics and survival outcomes of patients with and without thoracic involvement were assessed. We also estimated overall survival.The most common pattern of thoracic involvement was multiple pulmonary nodules (n = 10), followed by parenchymal tumor with pleural invasion (n = 4), reticulonodular opacities (n = 3), and diffuse pleural thickening (n = 2). No significant difference in survival was found between the thoracic EHE group and nonthoracic EHE group (P = 0.68). Among 4 different thoracic involvement types, the lung multinodular pattern tended to demonstrate longer median survival (8.5 months) than other patterns, whereas the shortest median survival (1 month) was observed for the nodule/mass with pleural involvement pattern (P = 0.038).CT manifestations of thoracic EHEs are classified into 4 patterns, of which lung multinodular pattern is associated with longer survival. Survival is not different between patients with and without thoracic involvement.

Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts

“…The only prognostic factors are the extent of disease at presentation (tumor size or the evidence of metastatic disease) and the presence of systemic signs and symptoms. 2 , 5 , 14 , 16 , 17 , 18 , 19 , 20 , 21 In patients with serosal involvement with/without effusion, the expected survival ranges from 1 to 11 months. The prognostic impact of locoregional metastases versus systemic metastases is still controversial.…”

Immunoglobulin G4-Related Disease Unilaterally Involving the Pulmonary Interstitium and Pleura: A Case Report

“…The current case is the first one of IgG4-RLD with pleural effusion, pleural nodules, and bronchovascular bundle thickening seen unilaterally on the CT scan. The differential diagnosis of patients with unilateral pleural effusion and perilymphatic interstitial thickening should include malignant pleural effusion with lymphangitic metastasis, mesothelioma, and epithelioid hemangioendothelioma (15,16). In our patient, the disease course was subacute rather than acute or rapidly progressive.…”

Multiple spindle cell hemangiomas in both lungs: a rare case report and review of the literature

“…PEH was a rare vascular neoplasm that usually occurs in the lung and liver and often presented as bilateral multiple lung nodules. PEH often showed multifocal areas of reticulonodular patterns [6, 7]. Histologically, they were different and spindle cell hemangioma showed vascular tumor with spindle cell stroma without epithelioid appearance shown in PEH.…”