Primary cardiac sarcomas are rare and carry a poor prognosis. The standard of care is complete resection. Outcomes for patients without complete resection are dismal, and the benefit of adjuvant therapy is uncertain. A 9-year-old girl presented with a large right-sided cardiac mass. After biopsy, the tumor was classified as an undifferentiated sarcoma. Resection was not feasible due to apparent invasion of the right ventricle and atrioventricular groove. Treatment with oral etoposide resulted in a 97% reduction in tumor volume and allowed for complete resection of residual tumor. She is alive with no evidence of disease 25 months from diagnosis.
*Department of Pediatrics, Division of Hematology-Oncology-BMT
†Department of Pediatrics, Division of Cardiology
‡Department of Surgery, Division of Pediatric Surgery
§Department of Pathology
∥Department of Surgery, Division of Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, WI
The authors declare no conflicts of interest.
Reprints: Chelsea L. Collins, MD, Department of Pediatrics, Division of Hematology-Oncology-BMT, Medical College of Wisconsin, MFRC 3018, 8701W Watertown Plank Road, Milwaukee, WI 53226 (e-mail: firstname.lastname@example.org).
Received November 20, 2012
Accepted February 27, 2013