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 cm, but most are less than 1 cm in diameter and are usually found in relation to small and medium-sized vessels and bronchi. Radiologic calcifications in PEH are rare, but histological examinations often show nodules with calcified and ossified necrotic centers on microscopy. In most cases, patients develop calcifications 10–20 years after diagnosis, the majority of which tend to be punctate calcifications. This presentation of multiple bilateral pulmonary nodules can easily be misinterpreted as hematogenous pulmonary metastases or fungal granulomas. However, because of the indolent nature of the disease, little or no growth is observed on serial examinations. Tumor calcifications or cavitation is rare. The reticulonodular pattern is characterized by multiple reticulonodular opacities associated with interlobular septal thickening and ground-glass opacities, mimicking hematolymphangitic metastases or diffuse interstitial lung disease. The parenchymal tumor with pleural invasion is less common and is associated with the worst prognosis. This may be seen as a solitary lung mass or as multiple nodules with one or more dominant nodules that invade the adjacent pleura. The diffuse pleural thickening pattern is uncommon and is seen as smooth or nodular thickening of the pleura, which can extend into the fissures and is often associated with pleural effusion. This pattern is most commonly confused with pleural mesothelioma or pleural metastases

References

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