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The Korean Journal of Pathology 2011;45(2): 132-138.
doi: https://doi.org/10.4132/KoreanJPathol.2011.45.2.132
Pulmonary Vascular Sarcomas: Clinicopathologic Analysis of 14 Cases.
Na Rae Kim, Jhingook Kim, Seung Yeon Ha, Joungho Han
1Department of Pathology, Gachon University Gil Hospital, Incheon, Korea.
2Department of Thoracic Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
3Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. hanjho@skku.edu
BACKGROUND: Pulmonary vessel sarcomas are rare, and their pathogenesis is still unclear. METHODS: We focus on the pathologic findings of fourteen pulmonary artery and/or vein sarcomas along with clinical prognosis. RESULTS: Nine patients were male and five were female, and they ranged in age from 26 to 72 years (mean, 47 years). There were ten cases of pulmonary artery sarcoma, three cases of pulmonary artery and vein sarcoma, and one case of pure pulmonary vein sarcoma. Ten out of the fourteen cases were associated with pulmonary thromboembolism. Microscopically, all the tumors showed an undifferentiated sarcomatous portion. There were leiomyosarcoma portions in 8 cases, malignant fibrous histiocytomatous portions in 7 cases, angiosarcomatous differentiation in 3 cases, and osteosarcomatous portion in 1 case. All but two patients died during the follow up period (range, 1 to 78 months). The mean survival time of the patients who died was 14 months and the longest survival time was 78 months after surgical resection. CONCLUSIONS: The current study is one of the largest single institutional reviews of pulmonary artery and/or vein sarcoma. Regardless of the histological components and macroscopic growth patterns, these rare tumors have a grave prognosis.
Key Words: Sarcoma; Pulmonary artery; Pulmonary veins