Primary malignant sarcomas of the heart and great vessels in adult patients–a single-center experience. Mayer, F., Aebert, H., Rudert, M., Konigsrainer, A., Horger, M., Kanz, L., Bamberg, M., Ziemer, G., & Hartmann, J. T. The oncologist, 12(9):1134–1142, September, 2007.
doi  abstract   bibtex   
BACKGROUND: Sarcomas arising in the heart or the great vessels are rare entities. The prognosis of the patients is dismal. METHODS: Between January 1993 and September 2006, of 1,429 patients registered to the Sarcoma Center, 14 had a primary sarcoma of the heart or large vessels. RESULTS: Tumors were located in the left ventricle (n = 3), left/right atrium (n = 2/3), pulmonary artery (n = 2), and ventricular septum, aorta, pericardium, and inferior vena cava (n = 1 each). The most frequently encountered histologic subtypes were leiomyosarcoma and angiosarcoma. Six patients presented with distant metastases to the lungs (n = 5), lymph nodes (n = 2), and liver (n = 1). Eight patients had localized disease. Six of them underwent resection with curative intent. Of those, two developed local recurrence within 2 and 10 months from surgery. Eleven patients received palliative chemotherapy, seven of those as initial treatment. Eight patients attained a response to treatment, two had disease stabilization for 6 and 12 months. After a median follow-up of 14.5 months (range, 2-156), three patients were alive with no evidence of disease 11, 52, and 156 months after diagnosis. Two patients were alive with disease and nine patients had died. CONCLUSIONS: Patients with primary sarcomas of the heart and the large vessels were of a young age, and more than half of them presented with advanced disease. Given the promising response to chemotherapy, an optimized treatment approach including neoadjuvant chemo-/radiotherapy in patients with locally advanced disease should be pursued.
@article{mayer_primary_2007,
	title = {Primary malignant sarcomas of the heart and great vessels in adult patients--a single-center experience.},
	volume = {12},
	doi = {10.1634/theoncologist.12-9-1134},
	abstract = {BACKGROUND: Sarcomas arising in the heart or the great vessels are rare entities. The prognosis of the patients is dismal. METHODS: Between January 1993 and September 2006, of 1,429 patients registered to the Sarcoma Center, 14 had a primary sarcoma of the heart or large vessels. RESULTS: Tumors were located in the left ventricle (n = 3), left/right atrium (n = 2/3), pulmonary artery (n = 2), and ventricular septum, aorta, pericardium, and inferior vena cava (n = 1 each). The most frequently encountered histologic subtypes were leiomyosarcoma and angiosarcoma. Six patients presented with distant metastases to the lungs (n  = 5), lymph nodes (n = 2), and liver (n = 1). Eight patients had localized disease. Six of them underwent resection with curative intent. Of those, two developed local recurrence within 2 and 10 months from surgery. Eleven patients received palliative chemotherapy, seven of those as initial treatment. Eight patients attained a response to treatment, two had disease stabilization for 6 and 12 months. After a median follow-up of 14.5 months (range, 2-156), three patients were alive with no evidence of disease 11, 52, and 156 months after diagnosis. Two patients were alive with disease and nine patients had died. CONCLUSIONS: Patients with primary sarcomas of the heart and the large vessels were of a young age, and more than half of them presented with advanced disease.  Given the promising response to chemotherapy, an optimized treatment approach including neoadjuvant chemo-/radiotherapy in patients with locally advanced disease should be pursued.},
	language = {eng},
	number = {9},
	journal = {The oncologist},
	author = {Mayer, Frank and Aebert, Hermann and Rudert, Maximilian and Konigsrainer, Alfred and Horger, Marius and Kanz, Lothar and Bamberg, Michael and Ziemer, Gerhard and Hartmann, Jorg Thomas},
	month = sep,
	year = {2007},
	pmid = {17914083},
	keywords = {Adult, Aortic Diseases/epidemiology, Female, Follow-Up Studies, Germany/epidemiology, Heart Neoplasms/*epidemiology, Hemangiosarcoma/epidemiology, Humans, Leiomyosarcoma/epidemiology, Lung Neoplasms/secondary, Lymphatic Metastasis, Male, Middle Aged, Neoadjuvant Therapy/statistics \& numerical data, Pulmonary Artery/pathology, Remission Induction, Retrospective Studies, Sarcoma/economics/*epidemiology, Survival Rate, Vascular Neoplasms/*epidemiology, Venae Cavae/pathology},
	pages = {1134--1142},
}
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