Epithelioid hemangioendothelioma is a rare vascular neoplasm that represents a low-to-intermediate grade sarcoma, with clinical behavior intermediate between hemangioma and angiosarcoma. This neoplasm typically arises from the liver, lung, skin, or bone, and may be multicentric in origin. Patients with pulmonary epithelioid hemangioendothelioma may be detected asymptomatically at imaging performed for incidental reasons, with presenting symptoms commonly including chest pain, cough, dyspnea, and fatigue; hemoptysis is less commonly reported and is associated with a worse prognosis. Thoracic imaging findings in pulmonary epithelioid hemangioendothelioma are relatively nonspecific, with chest radiography commonly showing multiple, bilateral, poorly defined nodular opacities and thoracic computed tomography showing small, circumscribed nodules, multifocal reticular, and nodular opacities associated with interlobular septal thickening and ground-glass opacities (resembling diffuse lung disease), and diffuse, infiltrative pleural thickening mimicking malignant pleural mesothelioma. FDG-PET imaging may show increased tracer utilization within nodules in patients with pulmonary epithelioid hemangioendothelioma, but some nodules may not show increased tracer utilization. The pattern of organ involvement in patients with epithelioid hemangioendothelioma affects the prognosis of the lesion, and, while this neoplasm can be aggressive, indolent behavior, with long-term survival, has been reported. No specific therapy for epithelioid hemangioendothelioma is available, with surgical resection favored for relatively localized disease and various chemotherapy regimens, hormonal therapy, interferon-2α treatment, and advocated for multicentric disease.
- epithelioid hemangioendothelioma
- thoracic CT
- vascular neoplasm
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
- Critical Care and Intensive Care Medicine