Abstract
We describe surgical and adjuvant therapeutic management of a right ventricular (RV) sarcoma and pulmonary artery occlusion. Echocardiographic evaluation of a 39-year-old man with exertional dyspnea revealed a tumor mass in the right ventricle, pulmonary trunk, and bilateral pulmonary arteries. The tumor was resected with concomitant pulmonary valvotomy, but the resection was incomplete. The RV outflow was reconstructed with an allograft patch, and a stentless valve was implanted for pulmonary valvular function. The pulmonary trunk and arteries were enlarged with allograft patches. The tumor was undifferentiated sarcoma and caused postoperative pulmonary artery restenosis. Radiotherapy improved pulmonary perfusion (reduction of RV pressure), but the patient died of brain metastasis. Undifferentiated cardiac sarcomas associated with pulmonary hypoperfusion should be resected even if incompletely, and radiation therapy could alleviate reduced pulmonary perfusion.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Gadducci A, Cosio S, Romanini A, Genazzani AR. The management of patients with uterine sarcoma: a debated clinical challenge. Crit Rev Oncol Hematol 2008;65:129–142.
Orbach D, Rey A, Oberlin O, Sanchez de Toledo J, Terrier-Lacombe MJ, van Unnik A, et al. Soft tissue sarcoma or malignant mesenchymal tumors in the first year of life: experience of the International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumor Committee. J Clin Oncol 2005;23:4363–4371.
Mayer F, Aebert H, Rudert M, Königsrainer A, Horger M, Kanz L, et al. Primary malignant sarcomas of the heart and great vessels in adult patients: a single-center experience. Oncologist 2007;12:1134–1142.
Silverman NA. Primary cardiac tumor. Ann Surg 1980;191:127–138.
Burke AP, Cowan D, Virmani R. Primary sarcoma of the heart. Cancer 1992;69:387–395.
Head HD, Flam MS, John MJ, Lipnik SS, Slater DL, Stewart RD. Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy. Ann Thorac Surg 1992;53:332–334.
Mazzucco A, Luciani GB, Bertolini P, Faggian G, Morando G, Ghimenton C. Primary leiomyosarcoma of the pulmonary artery: diagnostic and surgical implications. Ann Thorac Surg 1994;57:222–225.
Anderson MB, Kriett JM, Kapelanski DP, Tarazi R, Jamieson SW. Primary pulmonary artery sarcoma: a report of six cases. Ann Thorac Surg 1995;59:1487–1490.
Kruger I, Borowski A, Horst M, de Vivie ER, Theissen P, Gross-Fengels W. Symptoms, diagnosis, and therapy of primary sarcomas of the pulmonary artery. Thorac Cardiovasc Surg 1990;38:91–95.
Eng J, Murday AJ. Leiomyosarcoma of the pulmonary artery. Ann Thorac Surg 1992;53:905–906.
Tobe S, Nohara H, Yoshida K, Tanimura N, Kurokawa R, Kawata M. Right ventricular outflow tract reconstruction for primary artery sarcoma. Jpn J Thorac Cardiovasc Surg 2004;52:139–142.
Ishikawa K, Takanashi S, Mihara W, Fukui T, Hosoda Y. Surgical treatment for primary cardiac leiomyosarcoma causing right ventricular outflow obstruction. Circ J 2005;69:121–123.
Long H, Qin Q, Xie C. Response of pulmonary artery intimal sarcoma to surgery, radiotherapy and chemotherapy: a case report. J Med Case Rep 2008;2:217.
Nakahira A, Ogino H, Sasaki H, Katakami N. Long-term survival of a pulmonary artery sarcoma produced by aggressive surgery and adjuvant chemoradiotherapy. Eur J Cardiothorac Surg 2007;32:388–390.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Yamamoto, H., Yamamoto, F., Ishibashi, K. et al. Primary sarcoma of the right ventricle: surgical and adjuvant therapy. Gen Thorac Cardiovasc Surg 57, 421–425 (2009). https://doi.org/10.1007/s11748-009-0403-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11748-009-0403-7