대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Pulmonary Epithelioid Hemangioendothelioma

Radiologic Findings

Chest PA radiograph shows multiple nodular opacities in bilateral lung fields.
Chest CT images show multiple, well-defined, round or irregular nodules less than 1cm in both lungs with perivascular, subpleural distribution. Enhancement pattern is not conclusive because of the small size of the nodules.

Brief Review

Pulmonary epithelioid hemangioendothelioma (EHE), previously known as intravascular bronchoalveolar tumor, is a rare disease. It is more common among young women and typically presents as bilateral multiple nodules. Only rare EHE develops as a solitary lung nodule.
It can arise from many organ systems, including liver, bone, and soft tissues simultaneously or sequentially.
Chest pain, cough, and sputum are common symptoms and only few patients present with an alveolar hemorrhage at diagnosis .
The most characteristic feature of EHE on chest radiograph or CT is the presence of multiple perivascular nodules with well- or ill-defined margins in both lungs [1]. Radiologic calcification is not common, but histologic examinations often reveal calcified and ossified necrotic centers of the nodules.
Differential diagnosis includes metastatic carcinoma, chronic granulomatous disease, less commonly multiple benign tumors, vasculitis or bronchiolitis rarely. And in children, Histiocytosis X can be considered [3].
Bearing the metastatic nature of this entity in mind, the diagnosis should be followed by a search for sclerosing vascular tumors elsewhere. Extrapulmonary spread of the tumor has been mainly described in the liver. There is no clear standard for treatment, but with lesions being small and limited in number, surgical resection is recommended.

References

1. Cronin P, Arenberg D. Pulmonary Epithelioid Hemangioendothelioma : An Unusual Case and a Review of the Literature. Chest 2004; 125:789-793.
2. Gimeez A, Franquet T, Prats R, et al. Unusual Primary Lung Tumors: A Radiologic-Pathologic Overview. RadioGraphics 2002; 22:601-619.
3. Schattenberg T, Kam R, Klopp M, et al. Pulmonary Epithelioid Hemangioendothelioma. Surg Today 2008; 38:844-849.
4. Jang KY , Jin GY, Lee YC, et al. Pulmonary Epithelioid Hemangioendothelioma: A Tumor Presented as a Single Cavitary Mass. J Korean Med Sci 2003; 18: 599-602.

Keywords

Lung, Vascular, Malignant tumor,