21 August 2018
Previous studies on primary pulmonary epithelioid angiosarcoma (PEA) have been mostly clinical or pathological case reports. We here summarize findings from computed tomography (CT) and positron emission tomography/computed tomography (PET/CT) analyses of PEA to improve the diagnosis and differentiation of this rare tumor.
We conducted a retrospective analysis of the clinical findings, radiological imaging, and pathological findings of 6 cases of primary PEA confirmed by surgery, biopsy, and pathology. All cases were evaluated by CT and x-ray prior to surgery, and 2 cases were further examined by PET/CT.
CT images indicated maximum tumor diameters of 2.4 to 9.8 cm and inhomogeneous density, with 1 case exhibiting nodular calcification. Contrast-enhanced CT revealed inhomogeneous enhancement with visible necrosis in all 6 cases, while 3 cases had hilar and mediastinal lymph node metastasis. Five cases displayed extensive tumor involvement with extension into the chest wall, mild-to-moderate levels of pleural effusion, and varying degrees of volume loss in the corresponding hemithorax. One case had limited pleural thickening and invasion. Preoperative PET/CT of 1 case revealed abnormal fluorine-18 fluorodeoxyglucose ( 18F-FDG) uptake by the tumor and multiple enlarged right hilar and mediastinal lymph nodes, right diffuse pleural thickening, and systemic multiple bone metastasis. In the other case, PET/CT scan at 7 months after surgery revealed pleural thickening and mediastinal lymph nodes with increased 18F-FDG uptake on the surgical side. Immunohistochemistry analyses determined that all 6 tumors were positive for CD34, CD31, ERG, and vimentin.
CT and PET/CT findings reveal that malignant characteristics, including extensive pleural thickening, invasion and metastasis, and pleural effusion, are common in PEA. Imaging data are only supportive; therefore, the final diagnosis should be based on pathology and immunohistochemistry analyses.