Cardiac Sarcoma Treatment & Management

Updated: Sep 21, 2023
  • Author: John H Raaf, MD, PhD; Chief Editor: Eric H Yang, MD  more...
  • Print

Approach Considerations

Complete surgical excision remains the mainstay of therapy for primary cardiac sarcoma, followed by radiotherapy with or without sequential chemotherapy. Chemotherapy regimens containing anthracyclines, ifosfamide, or taxanes have been reported. [22]

Neoadjuvant chemotherapy may also be considered. In one case report, preoperative chemotherapy reduced the size of an unresectable undifferentiated cardiac sarcoma, allowing complete resection of residual tumor. [23]  In a case series of 44 patients with primary right-sided sarcomas, the addition of neoadjuvant chemotherapy increased survival from 9.5 months to 20 months. [24]

Multimodality therapy has proved most effective. In a retrospective chart review, patients who received multimodality treatment (any combination of surgery, radiation therapy, and chemotherapy) had an estimated median survival of 36.5 months compared with 14.1 months for patients treated with surgery, radiation therapy, or chemotherapy only (P=0.05). [15]


Surgical Care

Cardiac sarcoma is rarely cured, but prolonged survival or significant palliation is possible with surgical resection. [25, 26] Exploration with biopsy yields tissue for histologic diagnosis and assessment of the gross extent of the tumor.

The role of orthotopic heart transplantation for malignant cardiac tumors continues to be debated. [27, 28, 29, 30] In a study of 46 patients who underwent orthotopic heart transplantation for unresectable primary cardiac sarcoma, median survival in those with angiosarcoma (the most common histologic type) was 9 months, versus 36 months in the patients with other histologic types (P = 0.002). Additionally, in patients with angiosarcoma, median survival in patients who received palliative care only was similar to that in patients who underwent transplantation (8 vs 9 months; P = 0.768). [31]

Bench surgery (explantation and autotransplantation of the heart) may aid in achieving more complete tumor resection. [32, 33, 34]

Complete or partial excision of primary or metastatic cardiac sarcoma can provide hemodynamic improvement and relief from congestive heart failure. Alternatively, a pericardial window or pericardiectomy may ameliorate symptoms.

Postsurgical radiation and chemotherapy have not proven consistently beneficial. However, adjuvant radiation or chemotherapy can be beneficial in ameliorating symptoms and improving quality of life.



Long-Term Monitoring

No specific guidelines for follow-up care have been established; however, because of the low postoperative survival rate (median survival, 6 mo), pay careful attention postoperatively to the patient's cardiopulmonary status and overall physical state.