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

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          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.

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          Thoracic epithelioid malignant vascular tumors: a clinicopathologic study of 52 cases with emphasis on pathologic grading and molecular studies of WWTR1-CAMTA1 fusions.

          Malignant thoracic epithelioid vascular tumors are an uncommon and heterogenous group of tumors that include low-grade to intermediate-grade epithelioid hemangioendothelioma (EHE) and high-grade epithelioid angiosarcoma (EAS). We examine the morphologic and immunohistochemical features of 52 malignant epithelioid vascular tumors (10 low-grade EHE, 29 intermediate-grade EHE, and 13 EAS) involving the thorax (lung, pleura, mediastinum, heart, great vessels) including cases with exclusively thoracic disease (35) and with multiorgan disease including the thorax (17). Intermediate-grade EHE differs from low-grade EHE by the presence of necrosis, increased mitotic activity, and increased atypia. Morphologic features such as intranuclear inclusions, intracytoplasmic vacuoles, and stromal changes (chondroid, myxoid, or hyalinized stroma) are seen more frequently in EHE, whereas blood lakes, proliferation of slit-like vessels, and prominent nucleoli favor EAS. Fluorescence in situ hybridization analysis showed CAMTA1-WWTR1 fusions in 4/7 low-grade and 23/23 intermediate-grade EHE (P<0.001). In EAS, CAMTA1 rearrangement was negative in all cases, whereas a WWTR1 complex abnormality was found in 1/5 cases (P<0.001). This offers an objective means of differentiating intermediate-grade EHE from EAS, especially on limited biopsies. All cases show expression of at least 1 vascular marker, which allows differentiation from primary thoracic epithelial malignancies, although keratin expression is a potential pitfall with 29% of EHE and 25% of EAS showing keratin expression. Survival analysis shows that higher tumor grade for all tumors (P=0.026) as well as lung and pleural tumors only (P=0.010) and the presence of pleural involvement in lung and/or pleural tumors (P=0.042) correlate with poor prognosis.
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            Multimodality imaging for characterization, classification, and staging of malignant pleural mesothelioma.

            Malignant pleural mesothelioma (MPM) is the most common primary malignancy of the pleura and is associated with asbestos exposure in approximately 80% of patients. The patient prognosis is poor, with a median survival of 9-17 months after diagnosis. However, improved survival and decreased morbidity and mortality have been demonstrated when the diagnosis is made in the early stages of disease and specific treatment strategies are implemented. A staging system that focuses on the extent of primary tumor (T), lymph node involvement (N), and metastatic disease (M) has been devised by the International Mesothelioma Interest Group and emphasizes factors related to overall survival. Radiologists should recognize the manifestations of MPM across multiple imaging modalities, translate these findings into the updated staging system, and understand the effects of appropriate staging on treatment and survival. Computed tomography (CT) remains the primary imaging modality used to evaluate MPM and efficiently demonstrates the extent of primary tumor, intrathoracic lymphadenopathy, and extrathoracic spread. However, additional imaging modalities, such as magnetic resonance (MR) imaging of the thorax and positron emission tomography (PET)/CT with fluorodeoxyglucose, have emerged in recent years and are complementary to CT for disease staging and evaluation of patients with MPM. Thoracic MR imaging is particularly useful for identifying invasion of the chest wall, mediastinum, and diaphragm, and PET/CT can accurately demonstrate intrathoracic and extrathoracic lymphadenopathy and metastatic disease.
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              Primary epithelioid angiosarcoma of the lung presenting as pulmonary hemorrhage.

              Pulmonary angiosarcomas are usually secondary tumors, and only a few primary cases have been reported. We report a unique case of epithelioid angiosarcoma presenting as a solitary mass in the right upper lobe with pulmonary hemorrhage. Because of its epithelioid histology, this tumor may resemble a carcinoma or a variety of vascular lesions with epithelioid endothelial cells. Therefore, the diagnosis of epithelioid angiosarcoma should be based on immunohistochemical staining.

                Author and article information

                Medicine (Baltimore)
                Medicine (Baltimore)
                Wolters Kluwer Health
                August 2018
                21 August 2018
                : 97
                : 35
                [a ]Department of Radiology
                [b ]Department of Pathology, the First Affiliated Hospital of Guangzhou Medical University
                [c ]Guangzhou Institute of Respiratory Health, Guangzhou, China.
                Author notes
                []Correspondence: Yubao Guan, Department of Radiology, the First Affiliated Hospital of Guangzhou Medical University, Guangzhou 510120, China (e-mail: yubaoguan@ 123456163.com ).
                MD-D-18-00233 12107
                Copyright © 2018 the Author(s). Published by Wolters Kluwer Health, Inc.

                This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc/4.0

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