대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Pulmonary epithelioid hemangioendothelioma

Radiologic Findings

Fig 1. Chest PA shows localized nodular opacity in the right middle lung zone, abutting mediastinal shadow.
Fig 2-4. Axial, coronal, and sagittal CT images with mediastinal and lung window setting reveal a well-defined poorly enhancing nodule with calcified foci in RML and multiple peribronchial distributed irregular nodular opacities with calcification in both lungs.
Fig 5. Axial PET/CT image show mild hypermetabolic soft tissue lesion in RML of the lung (SUVmax 2.7).

The patient underwent endobronchial ultrasound-guided biopsy and the pathologic examination confirmed pulmonary epithelioid hemangioendothelioma in the RML nodule.

Brief Review

Epithelioid hemangioendothelioma (EHE) is a locally aggressive vascular neoplasm originating from vascular endothelial or preendothelial cells. It can arise anywhere in the body and can metastasize to the viscera, bones, and soft tissue. The most common sites of involvement are the liver (21% of patients), bone (14%), and lung (12%). There is still no characteristic clinical or biological marker for EHE. EHE typically occurs among young patients and is more common in women than in men and many patients are asymptomatic at presentation, so it is often an incidental finding on imaging studies.

Pulmonary epithelioid hemangioendothelioma (PEH) has been divided into four imaging patterns: a multinodular pattern, a reticulonodular pattern, a parenchymal tumor with pleural invasion, and diffuse pleural thickening. The multinodular pattern is the most common pattern and is associated with the best prognosis. The presence of multiple discrete pulmonary perivascular nodules with well- or ill-defined margins in both lungs on chest radiographs or CT is the characteristic finding. The nodules can range in size up to 3

References

1. Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M. Epithelioid hemangioendothelioma: an over-view and update on a rare vascular tumor. Oncol Rev 2014; 8:259
2. Lau K, Massad M, Pollak C, et al. Clinical pat-terns and outcome in epithelioid hemangioendo-thelioma with or without pulmonary involvement: insights from an internet registry in the study of a rare cancer. Chest 2011; 140:1312

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