CT and clinicopathological features of primary pulmonary artery sarcoma
CHEN Li1, LIN Yizi2,ZHANG Peigui3, XIA Ruike2, CHENG Weipeng4, LI Jun5, WANG Jianshu6, GAO Yuantong2.
1.Department of Anesthesiology, the Third Affiliated Hospital of Wenzhou Medical University, Wenzhou 325200, China;2.Department of Radiology, the Third Affiliated Hospital of Wenzhou Medical University, Wenzhou 325200,China; 3.Department of Radiology, Yueqing People’s Hospital, Wenzhou 325600, China; 4.Department of Neurosurgery, the First People’s Hospital of Suzhou, Suzhou 234000, China; 5.Department of Radiology, the Affiliated Hospital of Jiangxi University of Chinese Medicine, Nanchang 330006, China; 6.Department of Bone and Soft Tissue Oncology, Tumor Hospital of Gansu Province, Lanzhou 730050, China
CHEN Li,LIN Yizi,ZHANG Peigui, et al. CT and clinicopathological features of primary pulmonary artery sarcoma[J]. JOURNAL OF WEZHOU MEDICAL UNIVERSITY, 2024, 54(6): 487-491.
Abstract：Objective: To analyze the CT and pathological features of primary pulmonary artery sarcoma (PPAS). Methods: Retrospective analysis was conducted on CT images and pathological data from 13 cases of PPAS. CT scans focused on observing the location, extent, shape, density, invasion and metastasis of surrounding tissues. Pathological manifestations of the tumor were also analyzed to identify potential characteristics. Results:In 12 cases, the main body of the tumor was located in the main pulmonary artery pulmonary artery trunk, while in 1 case it was found in the pulmonary artery trunk. Tumor involvement was observed in the pulmonary lobar branches of the pulmonary artery in 9 cases. In 13 cases, PPAS indicated significant localized thickening of the pulmonary arteries, with complete or partial filling defects in corresponding areas of the main pulmonary artery and proximal pulmonary artery. These defects presented as cast, polypoid, or nodular changes. The inner wall boundary of the diseased segment of the pulmonary artery appeared unclear. There were 5 cases that showed lesion invasion outside the arterial wall, penetrating the vascular wall and forming a mass within the mediastinal.One case revealed invasion of the main left pulmonary vein, forming a polypoid mass shadow. Furthermore, one case exhibited secondary solitary nodular metastasis in the left upper lobe of the lung. On plain scanning, the tumor appeared uneven or slightly lower in density, with uneven enhancement observed in the arterial phase,continuous enhancement in the venous phase, and reduced enhancement in the delayed phase after enhancement.Multiple small and patchy exudative solid lesions were observed in 4 cases, including 1 case with cavity. Mild pericardial effusion was detected in 2 cases, along with mild pleural effusion. Microscope examination revealed tumor cells were predominantly irregular vascular cavities and spindle cells. Some areas exhibited mucinous changes, necrosis, and large nuclei atypia. Immunophenotype: tumor cells showed diffused positivity for Vimentin,and positivity for CD34 and CD31. Conclusion: PPAS mostly invades the main pulmonary artery, as well as the left and right pulmonary artery trunks, with involvement of more than 2 sites commonly observed. The lesions are mostly cast-like, polypoid, or nodular, exhibiting uneven density on plain scans and uneven enhancement on enhanced scans. The tumor exhibits strong invasiveness. The tumor cells showed diffused positive for Vimentin,while CD34 and CD31 are positive. These findings can aid in the recognition and diagnosis of PPAS.
