Cardiac Sarcoma Treatment & Management

Updated: Dec 23, 2019
  • Author: John H Raaf, MD, PhD; Chief Editor: Eric H Yang, MD  more...
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Approach Considerations

For treatment of cardiac sarcoma, complete surgical excision remains the therapeutic mainstay, followed by radiotherapy with or without sequential chemotherapy. Chemotherapy regimens containing anthracyclines, ifosfamide or taxanes have been reported. [15]

In one case report, preoperative chemotherapy reduced the size of an unresectable undifferentiated cardiac sarcoma, so that residual tumor could then be completely resected. [16]  In a case series of 44 patients with primary right-sided sarcomas, survival was doubled (20 months vs 9.5 months) with the addition of neoadjuvant chemotherapy. [17]

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). [9]


Surgical Care

Cardiac sarcoma is rarely cured, but prolonged survival or significant palliation is possible with surgical resection. [18, 19] 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. [20, 21, 22, 23]  The results of a study analyzing the outcomes of 46 patients who underwent orthotopic heart transplantation for unresectable primary cardiac sarcomas found that median survival time after heart transplantation for patients with angiosarcoma was much less than that of other histologic types (9 vs 36 months; P = 0.002). Additionally, it was similar to the median survival of 8 months for patients with angiosarcoma receiving palliative care (P = 0.768). [24]

Bench surgery (explantation and autotransplantation of the heart) may aid in achieving more complete tumor resection. [25, 26, 27]

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 adjuvant 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.