대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Epithelioid hemangioendothelioma

Radiologic Findings

Chest radiograph demonstrates a large amount of right pleural effusion. Chest CT demonstrates multiple pleural masses in right hemithorax. PET/CT depicts increased FDG uptake in lung and pleural lesions.

Brief Review

Epithelioid hemangioendothelioma (EH) is a rare vascular tumor, originating from vascular endothelial or pre-endothelial cells. This low- to intermediate-grade sarcoma may involve the liver alone (21%), liver and lung (18%), lung alone (12%), or bone alone (14%), and can be aggressive, even resulting in systemic metastasis. Pulmonary EH is four times more common in women than in men and is often detected incidentally.
EH in the thorax involves not only the lungs but also the pleurae and the mediastinum. In the lungs, EHE usually presents as multiple lung nodules. When the disease presents as an isolated pleural lesion, it typically exhibits pleural effusion and pleural nodules or thickening and simulates malignant pleural mesothelioma. Extrathoracic organ involvement includes the liver, long bones, spine, and soft tissues in the body.
The prognosis is very variable, with survival ranging from less than one year up to 30 years. There is no established standard treatment of choice; Surgery and/or chemoradiation therapy could be considered, depending on intrathoracic tumor spread and systemic metastases.

References

1. Angela Sardaro, Lilia Bardoscia, Maria Fonte Petruzzelli, Maurizio Portaluri : Epithelioid hemangioendothelioma : an overview and update on a rare vascular tumor. Oncology Review 2014; 8:82-91
2. Eun Young Kim, Tae Sung Kim, Joungho Han, Joon Young Choi, O Jung Kwon and Jhingook Kim : Thoracic epithelioid hemangioendothelioma. Acta Radiologica 2011; 52: 161-166
3. Kyung Soo Lee, Joungho Han, Man Pyo Chung, Yeon Joo Jeong. Radiology Illustrated: Chest Radiology, Verlag Berlin Heidelberg: Springer, 2014:190
4. Jung Han Han, Tae Jung Kim, Kyung Soo Lee, Tae Sung Kim, Byung-Tae Kim : Epithelioid hemangioendothelioma in the thorax: Clinicopathologic, CT, PET, and prognostic features. Medicine 2016; 95: 30

Keywords

Epithelioid hemangioendothelioma ,