대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Pulmonary Epithelioid Hemangioendothelioma, probably metastasis from the liver

Radiologic Findings

Figure 1. Chest PA shows reticular or reticulonodular opacities in both mid to lower lung zone and moderate amount of pleural effusion in left hemithorax. PCD insertion state. Multiple calcific nodular lesions in liver.
Figure 2. Lung window setting of the chest CT scan shows diffuse interlobular septal line thickening mixed with GGOs and nodular fissural thickening in both side lung.
Figure 3-4. Pre and Post images of the chest CT scan show localized parietal pleural thickening and effusion in left hemithorax. Several enlarged LNs in 4L, 7, 11R area. There are heterogeneous enhancing lesions with nodular calcifications in both lobes of liver.
Figure 5. PET-CT shows multifocal hypermetabolic masses in liver and both lungs. It shows increased uptake of glucose in left pleural effusion, multiple lymph node in intrathoracic & upper abdominal area and multiple bones including spines, both ribs, both pelvic bones, both scapulae, left humerus & left femur.

Brief Review

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare neoplasm of vascular origin. It is a low-to-intermediate grade malignant tumor, of borderline malignancy and a clinical course usually between hemangioma and angiosarcoma
The most common presentations were liver alone (21%), liver plus lung (18%), lung alone (12%), and bone alone (14%). Clinical manifestation is chest pain, dyspnea cough, sputum
Radiographically, bilateral multiple nodular nodules are the most common presentation [27, 49]. The presence of multiple discrete pulmonary perivascular nodules with wellor 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 mediumsized vessels and bronchi. This form of presentation may appear in many lung diseases and is easily mistaken for metastatic carcinoma, which is usually the initial radiologic interpretation. However, little growth is shown on serial chest CT examinations. When this pattern is found the differential diagnosis list generally includes the following: pulmonary metastases,miliary granulomatous infection, sarcoidosis, silicosis, primary lungmalignancy, and lymphangitic carcinomatosis
The prognosis is very variable, with survival ranging from less than one year up to 30 years. Slow progression or growth for long periods and spontaneous regression may occur, especially in asymptomatic patients.

Please refer to

Case 639, Case 691, Case 762, Case 772, Case 932, Case 1088, Case 1094, Case 1195,

References

1. Hindaw vol. 2017, 5972940

Keywords

lung, liver, Epithelioid hemangioendothelioma,