Cardiac Transplantation for Giant Sarcoma of the Left Ventricle
An 8-year-old boy with a growing left ventricular mass underwent cardiac surgery for tumor resection. Radical resection seemed impossible, and thus, only a large biopsy sample was taken with a concomitant patch closure of the left ventricular defect. Histological examination revealed a rare liposarcoma. The patient was discharged without further therapy.
Five months later, the patient was transferred to our hospital because his clinical condition had worsened to New York Heart Association class IV, and a recurrent left ventricular tumor became evident. MRI (Figures 1, 2, and 3⇓⇓) and echocardiography (Figure 4) showed an inoperable tumor almost totally obstructing the left ventricular cavum, with excessive infiltration of the myocardium. According to T1- and T2-weighted images with fat saturation, there were only small streaky areas of fatty tissue within the tumor. Left ventricular function was significantly impaired (Movie I and Movie II).
Whole-body positron emission tomography (PET) with 2-[18F]fluoro-2-deoxy-d-glucose (FDG) in combination with thoracic and abdominal computed tomography with an integrated PET-CT scanner (Figure 5) demonstrated an inhomogeneously increased glucose metabolism in the tumor of the left ventricle and excluded lymph node and distant metastases. We decided to schedule the patient for urgent heart transplantation, which was performed after 34 days. Figure 6 impressively demonstrates the left ventricular obstruction caused by the tumor mass. Histopathological examination revealed a sarcoma with areas of lipomatous differentiation. Today, 6 months after orthotopic heart transplant, the young patient is alive and in excellent clinical condition (New York Heart Association class I), without evidence of tumor recurrence.
The online-only Data Supplement, which contains Movies I and II, is available at http://circ.ahajournals.org/cgi/content/full/112/14/e247/DC1.