Correlations between computed tomography and positron emission tomography/computed tomography findings and pathology in 6 cases of pulmonary epithelioid angiosarcoma

Abstract

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.