Mutational status of K-ras and TP53 genes in primary sarcomas of the heart.

We investigated three patients with cardiac angiosarcomas and two with cardiac rhabdomyosarcomas, all for mutations at exons 5, 6, 7 and 8 of the p53 gene and at exon 1 of K-ras. No point mutations were observed in the p53 gene in any of the five cases; however, at exon 1 of K-ras, three patients (60%) presented the same mutation at the first base of codon 13 (G to A transition). Interestingly, this mutation was detected in both rhabdomyosarcoma and angiosarcoma histologic sarcoma types.

[Right ventricular arrhythmogenic dysplasia. The role of heart transplantation in its management].

Arrhythmogenic right ventricular dysplasia is a rare disease that usually presents with ventricular arrhythmias and sometimes with heart failure. Rarely symptoms become severe and refractory to conventional therapy. We present the case of a 36-year-old man with this disease who had sustained ventricular arrhythmias and severe right heart failure.

Heart transplantation for cardiac angiosarcoma: should its indication be questioned?

Two men, aged 31 and 32 years, respectively, underwent orthotopic heart transplantation, in both cases to treat primary cardiac angiosarcoma of the right atrium. Total removal of the tumor was performed, and no evidence was found of distant dissemination at the time of surgery. Their postoperative progress was good; however, the patients died 8 and 9 months after transplantation, respectively, of multiple brain metastases.