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.

Questions

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Media Gallery

Angiosarcoma of the heart, with pericardial encasement by hemorrhagic tumor. Image courtesy of Dr. K. Marchant.

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Author

John H Raaf, MD, PhD (Retired) Professor, Department of Surgery, Case Western Reserve University School of Medicine

John H Raaf, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American College of Surgeons, Central Surgical Association, Society of Surgical Oncology, American Association of Endocrine Surgeons, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

Anastasios K Konstantakos, MD Clinical Associate Surgeon, Department of Cardiovascular Surgery, Billings Clinic

Disclosure: Nothing to disclose.

Heather N Raaf, MD (Retired) Chief Deputy Coroner, Cuyahoga County Coroner's Office

Heather N Raaf, MD is a member of the following medical societies: National Association of Medical Examiners, American Academy of Forensic Sciences

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Eric H Yang, MD Associate Professor of Medicine, Director of Cardiac Catherization Laboratory and Interventional Cardiology, Mayo Clinic ArizonA

Eric H Yang, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Additional Contributors

Robert C Shepard, MD, FACP Associate Professor of Medicine in Hematology and Oncology at University of North Carolina at Chapel Hill; Vice President of Scientific Affairs, Therapeutic Expertise, Oncology, at PRA International

Robert C Shepard, MD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for Physician Leadership, European Society for Medical Oncology, Association of Clinical Research Professionals, American Federation for Clinical Research, Eastern Cooperative Oncology Group, Society for Immunotherapy of Cancer, American Medical Informatics Association, American College of Physicians, American Federation for Medical Research, American Medical Association, American Society of Hematology, Massachusetts Medical Society

Disclosure: Nothing to disclose.