Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival

Abstract

Although cardiac sarcomas are rare in comparison to their soft tissue counterparts, they are the second most common type of primary cardiac neoplasm. Of the few hundred cases reported, most has been based on autopsy series. A series of 27 cardiac sarcomas removed at surgery for curative and diagnostic intent were reviewed for clinicopathologic features with correlation to available postoperative follow-up data in 17 patients. There were 6 angiosarcomas, 6 myxofibrosarcomas, 3 malignant peripheral nerve sheath tumors, 3 leiomyosarcomas, 2 synovial sarcomas, 1 epithelioid hemangioendothelioma, 1 chondrosarcoma, 1 osteosarcoma, and 4 poorly differentiated sarcomas. There was a wide age and size range with slight female predilection. There were 20 cases that arose in the atria/pulmonary vessels, 4 in the ventricles, 1 in mitral valve, and 2 in epi/pericardium. There was a slight left predilection. The histologic grade was low in 4, moderate in 3, and high in 20 cases. Six high-grade and 1 low-grade tumors were also treated with adjuvant chemotherapy and/or radiation. In 17 patients with follow-up data, 6 of 12 patients with high-grade tumor died (4 within 5 days of the initial surgery, 1 in 21 months, and 1 in 131 months), and 1 patient with moderate-grade tumor and all 4 patients with low-grade tumor were alive without evidence of disease at the end of follow-up. Tumor grade appeared to be prognostically important in cardiac sarcoma. Long survival was achieved in patients who survived the initial surgery well.

Fig. 1

Primary cardiac soft tissue sarcomas. A, Monophasic synovial sarcoma with uniform spindle cell morphology. B, MPNST with S100 immunostaining (insert). C, EHE with polypoid growth and bland epithelioid tumor cells with vacuolated cytoplasms in cords or nests in hyaline myxoid stroma (insert). D, High-grade angiosarcoma with poorly formed vascular spaces (A, B, and D: hematoxylin-eosin [H&E] ×200; C: H&E ×25; B inset, anti-S100 antibody, immunoperoxidase ×200; C inset, H&E, ×400).

Fig. 2

Primary cardiac bone sarcomas. A and B, Cardiac osteosarcoma (case 6, Table 2): multilobulated polypoid mass grossly invaded to the resected left atrial wall (A) and osteoid formation and chondroblastic differentiation were seen microscopically (B). C and D, Cardiac chondrosarcoma (case 9; Table 3) with well-differentiated chondroblastic element and nonchondroblastic spindle cell area (A: gross image; B: H&E ×100; C: H&E ×200; D: H&E ×400).

Fig. 3

High-grade myxofibrosarcoma (case 4; Table 1). A, Interface of the high-grade and low-grade areas to show difference in cellularity and nuclear pleomorphism. B, Hypocellular area with curvilinear vasculature and myxoid stroma. C and D, High-grade areas with necrosis (C) and higher cellularity and marked nuclear pleomorphism (D) (A, B, and C: H&E ×100; D: H&E ×200).

Fig. 4

Cardiac well-differentiated angiosarcoma (case 1; Table 1). A and B, Complex anastomosing vascular channels. C, The bland and flat endothelial cell lining. D, Tufted endothelial proliferation (A: H&E ×50; B: H&E ×100; C and D: H&E ×200).

Fig. 5

Kaplan-Meier plots for overall survival (top) and disease-free survival (bottom) of all cardiac sarcoma cases. Months of clinical follow-up are plotted on the x-axes and percentage of surviving cases plotted on the y-axes.

Fig. 6

Kaplan-Meier plots for overall survival (top) and disease-free survival (bottom) of high-grade (triangles) and low-grade (squares) cardiac sarcoma cases. Months of clinical follow-up are plotted on the x-axes and percentage of surviving cases plotted on the y-axes.