Pulmonary Vascular Sarcomas: Clinicopathologic Analysis of 14 Cases

Na Rae Kim, Jhingook Kim, Seung Yeon Ha, Joungho Han
2011 The Korean Journal of Pathology  
Primary neoplasms of the great vessels (aorta, pulmonary vessels, and vena cava) are rare. The pathogenesis of these tumors is still unclear, and it has been suggested that these tumors arise from the mesenchymal cells of the muscle anlage in the bulbus cordis in cases of pulmonary artery sarcoma. 1 Most primary sarcomas of the elastic vessels such as the aorta and the pulmonary artery or vein are undifferentiated sarcomas, while leiomyosarcomas predominate in the muscular arteries and great
more » ... ns such as the inferior vena cava. 2,3 Pulmonary vessel sarcomas are rare and show no characteristic symptoms, even though the thromboembolic events are the most severe events, and a subsequently delayed diagnosis may contribute to a fulminant prognosis. Recent advances in imaging modalities and accumulated clinical experience have made it possible to preoperatively diagnose these tumors. Most pulmonary vessel sarcomas have been reported as single case reports and there have only been a few radiologic review articles. 3, 4 Here, we review pulmonary artery and vein sarcomas with their clinicopathologic features. MATERIALS AND METHODS The data were retrospectively collected on all patients who were pathologically confirmed as having pulmonary vessel sarcomas between 1994 and 2010. The included cases were unequivocal cases in which there was evidence of a primary tumor arising within the lumen of the pulmonary vessels radiologically and/or grossly at the time of surgical specimen examination. Clinical history and follow-up data were obtained from the Korea National Statistics Office and the referring physicians. This study was approved by the Institutional Review Board of Samsung Medical Center (IRB No. 2010-09-110). The resected specimens were fixed in 10% buffered formalin and embedded in paraffin. Each case was routinely stained with hematoxylin and eosin. In order to confirm the specific differentiation of the tumors, immunohistochemistry was done using avidin-biotin-peroxidase complex methods.
doi:10.4132/koreanjpathol.2011.45.2.132 fatcat:2u6plxicdjesjbhrjbygtz7vpu