Primary malignant sarcomas of the heart and great vessels in adult patients--a single-center experience.
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.
Mayer
Frank
F
Department of Medical Oncology/Hematology/Immunology/Rheumatology/Pulmonology, UKT-Medical Center, South West German Cancer Center, Eberhard-Karls-University Tuebingen, Otfried-Mueller-Str. 10, 72076 Tuebingen, Germany.
Aebert
Hermann
H
Rudert
Maximilian
M
Königsrainer
Alfred
A
Horger
Marius
M
Kanz
Lothar
L
Bamberg
Michael
M
Ziemer
Gerhard
G
Hartmann
Jörg Thomas
JT
D000328:Adult; D001018:Aortic Diseases / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D005858:Germany / Q000453:epidemiology; D006338:Heart Neoplasms / Q000453:epidemiology*; D006394:Hemangiosarcoma / Q000453:epidemiology; D006801:Humans; D007890:Leiomyosarcoma / Q000453:epidemiology; D008175:Lung Neoplasms / Q000556:secondary; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D020360:Neoadjuvant Therapy / Q000706:statistics & numerical data; D011651:Pulmonary Artery / Q000473:pathology; D012074:Remission Induction; D012189:Retrospective Studies; D012509:Sarcoma / Q000191:economics / Q000453:epidemiology*; D015996:Survival Rate; D019043:Vascular Neoplasms / Q000453:epidemiology*; D014684:Venae Cavae / Q000473:pathology