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.

Treatment with IL2/vaccinia recombinant virus leads to serologic, histologic and phenotypic normalization of autoimmune MRL/lpr-lpr mice.

We have analyzed the effect of IL2 administered in vivo on both the lymphoproliferation and autoimmune disease progression of MRL/lpr mice. Human IL2 was delivered by infecting MRL/lpr mice with vaccinia virus recombinants at different stages of lpr disease. The results reported here showed that treatment of lpr mice with IL2 mediated: (1) restored normal thymic differentiation illustrated by an expansion of the double positive population accompanied by increased numbers of mature thymocytes; (2) depletion of the peripheral CD3+ CD4- CD8- (DN) T-cell population; (3) normalization in the pattern of TcRV beta gene expression displayed by mature T cells; (4) decreased urine-protein levels and immune complex deposition in the kidney, with a resultant absence of glomerulonephritis; and (5) an increased longevity (from 195 to more than 400 days).

Schwannomas of the falx.

A case of multiple schwannomas arising from the falx in a 17-year-old girl, previously operated on for an intracerebral schwannoma, is presented. The appearance of multiple schwannomas has to be considered in the followup of the rare cases of intracranial schwannomas not related to cranial nerves.

Subependymoma of the cervical spinal cord.

A case of subependymoma of the spinal cord in the cervical region is presented. Our experience with this patient and the seven cases of spinal cord subependymoma previously reported show that this tumor predominates in the cervical region and in males (3:1). Although radical removal is considered the rational goal of treatment, larger experience is necessary to know both the rate of growth and prognosis of subependymomas when these tumors arise within the spinal cord.