Pulmonary Vascular Sarcomas: Clinicopathologic Analysis of 14 Cases
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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.
Abstract
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.
참고문헌 (25)
- Lee JW, "Primary intimal sarcoma originating from pulmonary valve", Korean J Thorac Cardiovasc Surg, 33, pp.823~826 (2000)
- Kimura I, "Primary osteogenic sarcoma of pulmonary artery", Jpn J Med, 29, pp.32~37 (1990)
- Yi CA, "Computed tomography in pulmonary artery sarcoma: distinguishing features from pulmonary embolic disease", J Comput Assist Tomogr, 28, pp.34~39 (2004)
- Cox JE, "Pulmonary artery sarcomas: a review of clinical and radiologic features", J Comput Assist Tomogr, 21, pp.750~755 (1997)
- Kim JG, "Surgical resection of primary sarcoma of pulmonary artery clinically suspected of carcinoid", Korean J Thorac Cardiovasc Surg, 33, pp.195~198 (2000)
- Chong S, "Unilateral usual interstitial pneumonia associated with sarcoma of the pulmonary artery", AJR Am J Roentgenol, 189, pp.W221~W223 (2007)
- Brown FM, "Problems in grading soft tissue sarcomas", Am J Clin Pathol, 114 Suppl, pp.S82~S89 (2000)
- Coindre JM, "Histopathologic grading in spindle cell soft tissue sarcomas", Cancer, 61, pp.2305~2309 (1988)
- Mandelstamm M, "Über primäre Neubildungen des Herzens", Virchows Arch Pathol Anat, 245, pp.43~54 (1923)
- Sebenik M, "Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immunohistochemical profile and review of the literature", Am J Surg Pathol, 29, pp.1184~1193 (2005)
- Choong CK, "Failure of medical therapy for pulmonary “thromboembolic” disease: beware the unsuspected primary sarcoma of the pulmonary artery", J Thorac Cardiovasc Surg, 128, pp.763~765 (2004)
- Burke AP, "Sarcomas of the great vessels: a clinicopathologic study", Cancer, 71, pp.1761~1773 (1993)
- Tavora F, "Pulmonary artery sarcoma: a histologic and follow-up study with emphasis on a subset of low-grade myofibroblastic sarcomas with a good long-term follow-up", Am J Surg Pathol, 32, pp.1751~1761 (2008)
- Johansson L, "Sarcoma of the pulmonary artery: report of four cases with electron microscopic and immunohistochemical examinations, and review of the literature", Virchows Arch, 424, pp.217~224 (1994)
- Tovar-Martin E, "Intraluminal leiomyosarcoma of the superior vena cava: a cause of superior vena cava syndrome", J Cardiovasc Surg (Torino), 38, pp.33~35 (1997)
- Kaplinsky EJ, "Primary pulmonary artery sarcoma resembling chronic thromboembolic pulmonary disease", Eur Respir J, 16, pp.1202~1204 (2000)
- Kauczor HU, "Pulmonary artery sarcoma mimicking chronic thromboembolic disease: computed tomography and magnetic resonance imaging findings", Cardiovasc Intervent Radiol, 17, pp.185~189 (1994)
- Olsson HE, "Primary and secondary pulmonary artery neoplasia mimicking acute pulmonary embolism", Radiology, 118, pp.49~53 (1976)
- Zhao J, "Combined comparative genomic hybridization and genomic microarray for detection of gene amplifications in pulmonary artery intimal sarcomas and adrenocortical tumors", Genes Chromosomes Cancer, 34, pp.48~57 (2002)
- Zhang H, "Cytogenetic and molecular cytogenetic findings of intimal sarcoma", Cancer Genet Cytogenet, 179, pp.146~149 (2007)
- Gaumann A, "Exploration of the APC", beta-catenin (WNT) pathway and a histologic classification system for pulmonary artery intimal sarcoma: a study of 18 cases, 2008, pp.453, 473~484
- Bloomberg RD, "Primary cardiac sarcoma involving the pulmonary artery and valve", Can J Cardiol, 19, pp.843~847 (2003)
- Nakahira A, "Long-term survival of a pulmonary artery sarcoma produced by aggressive surgical resection and adjuvant chemoradiotherapy", Eur J Cardiothorac Surg, 32, pp.388~390 (2007)
- Maruo A, "Surgical experience for the pulmonary artery sarcoma", Ann Thorac Surg, 82, pp.2014~2016 (2006)
- Talbot SM, "Combined heart and lung transplantation for unresectable primary cardiac sarcoma", J Thorac Cardiovasc Surg, 124, pp.1145~1148 (2002)