Surgery for angiosarcoma and other cardiac sarcomas: A single-institution experience

Abstract

Background: Cardiac sarcomas are rare malignancies with a poor prognosis. Although angiosarcoma is the most common histological subtype, its features are poorly characterized. This study aimed to compare the clinical characteristics of the various cardiac sarcomas and the surgical techniques used and to identify factors influencing the prognosis.

Methods: Forty patients who underwent surgery for cardiac sarcomas were included; 60% of them had angiosarcoma. Clinical characteristics, tumor location, surgical techniques used, and the prognosis were compared between patients with angiosarcoma and patients with other subtypes. Kaplan-Meier curves and multivariable Cox regression were used to identify predictors of postoperative survival.

Results: Angiosarcomas were more likely than the other subtypes to present as pericardial effusion (85% vs 50%, P = .014). Early surgery was performed (median 24.0 days) regardless of histological subtype. The surgical technique varied according to histological subtype. Mean postoperative survival was 10 months. A positive margin (P = .13), high Ki-67 index (P = .19), younger age (P = .86), and angiosarcoma (P = .87) were identified to be potentially poor prognostic factors in univariate analyses. Cox regression identified R0 resection to be the only significant independent predictor of the prognosis after surgery (hazard ratio, 0.423, P = .039).

Conclusions: Angiosarcoma differs from other subtypes of cardiac sarcoma in terms of clinical symptoms, tumor location, surgical techniques used, and prognosis. Early surgery is needed regardless of subtype. R0 resection is the only independent predictor of postoperative survival, and complete resection is usually achievable. The prognosis may be poorer in patients with a positive margin, high Ki-67 index, younger age, and angiosarcoma.

Keywords: angiosarcoma; cardiac neoplasm; cardiac sarcoma; surgery.

Graphical abstract

Each subtype of cardiac sarcoma had a predilection for anatomic location and extent.

Figure 1

Schematic illustrations showing that each subtype of cardiac sarcoma had a predilection for anatomic location and extent. Angiosarcomas (red) were most likely to involve the right atrium, right atrioventricular groove, superior and inferior vena cavae, atrial septum, and left atrial roof. Malignant fibrous cytomas (blue) usually involved the left atrium, pulmonary veins, left atrioventricular groove, and posterior wall of the pulmonary artery. Synovial sarcomas (green) were more likely to involve the right ventricular outflow tract and the anterior wall of the pulmonary artery.

Figure 2

Contrast-enhanced computed tomography scans showing the typical features of the different subtypes of cardiac sarcoma (red arrows). A, Angiosarcoma, which usually involved the right atrium, right atrioventricular groove, right coronary artery, and right ventricular inflow tract. B, Synovial sarcoma, usually seen as a cystic mass compressing the right ventricular outflow tract. C, Malignant fibrous cytoma, which often involved the left atrium and pulmonary veins.

Figure 3

An intraoperative photograph demonstrating the complexity of reconstructive surgery for cardiac sarcoma. This patient had angiosarcoma and required reconstruction of the aortic root, ascending aorta, right atrium, tricuspid valve annulus, right ventricular inflow and outflow tracts, and bypass grafting for right coronary artery. Ao, Aorta; RA, right atrium; RVOT, right ventricular outflow tract; RVIFT, right ventricular inflow tract.

Figure 4

An intraoperative photograph obtained during orthotopic autologous heart transplantation. The explanted heart was preserved in a solution consisting of iced saline and histidine-tryptophan-ketoglutarate solution. The posterior annulus of the mitral valve and the posterior wall of the left atrium were reconstructed using bovine pericardium.

Figure 5

Kaplan–Meier curves showing that R0 resection (P = .013) and a Ki-67 index less than 50 (P = .019) were significantly associated with better survival. Patients aged less than 40 years (P = .086) and those with angiosarcoma (P = .087) tended to have a poorer prognosis. There was no significant association of postoperative survival with metastasis at the time of diagnosis (P = .185) or male sex (P = .287). CI, Confidence interval.

Figure 6

Cox regression analysis identified 4 potentially significant prognostic variables (R0 resection, a higher Ki-67 index, younger age, and angiosarcoma). However, R0 resection was the only independent predictor of postoperative survival (hazard ratio, 0.423, 95% confidence interval, 0.187-0.958, P = .039).

Figure 7

Angiosarcoma differs from other subtypes of cardiac sarcoma in terms of clinical symptoms, tumor location, surgical techniques used, and prognosis. Early surgery is needed regardless of subtype. R0 resection is the only independent predictor of postoperative survival, and complete resection is usually achievable. The prognosis may be poorer in patients with a positive margin, high Ki-67 index, younger age, and angiosarcoma. CI, Confidence interval.