Biatrial primary synovial sarcoma of the heart.

Synovial sarcomas that primarily arise from the heart and pericardium are extremely rare, especially the ones involving both sides of the heart. To date, few cases have been reviewed in the literature. Our patient was a 36-year-old man who presented with primary biatrial synovial sarcoma that also involved the tricuspid annulus, heart valves, and interatrial septum. The tumor was debulked to relieve the atrioventricular obstruction. Molecular analysis confirmed the diagnosis of synovial sarcoma with positive t (X;18) SYT-SSX gene fusion. The patient is currently receiving chemotherapy. From our review of the 20 cases thus far reported in the English-language medical literature, the tumor more frequently affects young male patients, and carries a poor prognosis. Early detection is difficult because of the aggressive nature of the tumor. Like other sarcomas, wide surgical resection remains the mainstay of therapy. Adjuvant radiation therapy for local recurrence and chemotherapy for control of systemic disease may have some beneficial effect on overall survival, but the benefit is likely limited.

Outcomes of lung transplantation in patients with scleroderma.

Patients with pulmonary insufficiency due to scleroderma have long been considered suboptimal candidates for lung transplantation. This has been supported by small single-center experiences that did not reflect the entire U.S. experience. We sought to evaluate the outcome of patients with scleroderma who underwent lung transplantation. We conducted a retrospective review of 47 patients with scleroderma who underwent lung transplantation at 23 U.S. centers between 1987 and 2004 and were reported to the United Network for Organ Sharing. Women constituted 57% of the patients. The mean age was 46 years. Twenty-seven patients received single lung transplants (57%), and the remaining received double lung transplants. The mean cold ischemia time was 4.1 hours. There were 7 early deaths (< or =30 days) and 17 late deaths (> 30 days). The causes of early death were primary graft failure and a cardiac event in two patients each and bacterial infection and stroke in one patient each. Late mortality was due to infection in seven patients, respiratory failure in three, malignancy in two, and multisystem organ failure, rejection, pulmonary hypertension, and a cardiac event in one patient each. The causes of early and late death were not recorded for two patients. One patient received a second transplant owing to graft failure of the first. Twenty-three patients (49%) were alive at a mean follow-up of 24 months. The Kaplan-Meier 1- and 3-year survival rates were 67.6% and 45.9% respectively, which are not significantly different from those of 10,070 patients given transplants for other lung conditions during the same period (75.5% and 58.8% respectively, P = 0.25). Donor gender, recipient's age, and type of transplant did not affect survival. In carefully selected patients with scleroderma who have end-stage lung disease, lung transplantation is a valid life-saving therapeutic option. Available data suggest acceptable short-term morbidity and mortality and a long-term survival similar to that of patients given transplants for other lung conditions.