Low-grade endometrial stromal sarcoma with inferior vena cava tumor thrombus and intracardiac extension: radical resection may improve recurrence free survival.

BACKGROUND: Endometrial stromal sarcoma (ESS) represents 0.2% of all uterine malignancies. Based on the mitotic activity, a distinction is made between low and high-grade ESS. Although the overall five-year survival rate for low-grade ESS exceeds 80%, about 50% of the patients show tumor recurrence, mostly after a long latency period. Tumor invasion of the great vessels is extremely rare. We describe a patient with advanced low-grade ESS with tumor invasion of the infrarenal aorta and the inferior vena cava. The patient presented with a large tumor thrombus extending from the inferior vena cava into the right atrium. METHODS: Review of literature and identification of 19 patients, including our own case report, with advanced low-grade ESS with invasion of the great vessels and formation of an inferior vena cava tumor thrombus. RESULTS: All 19 patients presented with an abdominal tumor mass and a tumor thrombus protruding into the inferior vena cava. The tumor thrombus extended into the right heart cavities in nine patients reaching the right atrium in four, the right ventricle in three and the pulmonary artery in two patients. There were 5 patients with an advanced primary tumor and 14 patients with an advanced recurrent tumor. Seven patients presented with synchronous metastatic disease and six patients with a pelvic tumor infiltrating the bladder, the rectosigmoid colon or the infrarenal aorta. Mean age at surgery was 45.9+/-12.3 years (median 47, range 25-65 years). Tumor thrombectomy was accomplished by cavatomy or by right atriotomy after installation of a cardiopulmonary bypass. There was no peri-operative mortality and a very low morbidity. Radical tumor resections were achieved in 10 patients. The follow-up for these 10 patients was 2+/-1.3 years (median 2, range 0.3-4.5 years). Nine patients remained recurrence free whereas one patient suffered an asymptomatic local recurrence. CONCLUSIONS: Low-grade ESS is a rare angioinvasive tumor with a high recurrence rate. Resection of an inferior vena cava tumor thrombus, even with extension into the right heart cavities, can be performed safely. Extensive radical surgery is therefore justified in the treatment of advanced tumor manifestations of a low-grade ESS potentially improving recurrence free survival.

Failing Systemic Right Ventricles With Persistent Pulmonary Hypertension: Candidates for Ventricular Assist Devices as Destination Therapy?

Dysfunction of the systemic right ventricle is common after the atrial switch procedure for transposition of the great arteries. Cardiac transplantation remains the only long-term solution in terminal systemic right ventricular (RV) failure, but concomitant pulmonary hypertension (PHT) may preclude it. The increasing number of such patients, together with the concerns related to combined heart-lung transplantation (HLTx), urge us to consider other therapeutic options.

Ten-year comparison of pericardial tissue valves versus mechanical prostheses for aortic valve replacement in patients younger than 60 years of age.

OBJECTIVE: Aortic valve replacement using a tissue valve is controversial for patients younger than 60 years old. The long-term survival in this age group, the expected event rates during long-term follow-up, and valve-related complications are not clearly determined. METHODS: From January 2000 to December 2009, overall survival, valve-related events, and echocardiographic outcomes were analyzed in all patients younger than 60 years of age, who underwent biologic aortic valve replacement. Patients who received a Perimount Carpentier-Edwards pericardial tissue valve (n = 103) were selected and compared with a propensity matched group of 103 patients who received aortic valve replacement using a mechanical bileaflet valve. The mean follow-up was 33 ± 24 months (range, 2-120), and the mean age at implantation was 50.6 ± 8.8 years (bioprosthesis, 55 ± 8.9 years; mechanical valve, 50 ± 8.6 years; P = .03). RESULTS: Survival was significantly reduced in patients after biologic aortic valve replacement (90.3% vs 98%; P = .038). Freedom from all valve-related complications (bioprosthesis, 54.5%; mechanical valve, 51.6%; P = NS) and freedom from reoperation (bioprostheses, 100%; mechanical valve, 98%; P = NS) were comparable in both groups. The average transvalvular mean (11.2 ± 4.2 mm Hg vs 10.5 ± 6.0 mm Hg, P = .05) and peak (19.9 ± 6.7 mm Hg vs 16.7 ± 8.0 mm Hg, P = .03) gradients were greater after biologic aortic valve replacement. Regression of the left ventricular mass index was more pronounced after mechanical valve replacement (118.5 ± 24.9 g/m(2) vs 126.5 ± 38.5 g/m(2); P = NS). The echocardiographic patient-prosthesis mismatch was greater at follow-up after biological aortic valve replacement (0.876 ± 0.2 cm(2)/m(2) vs 1.11 ± 0.4 cm(2)/m(2); P = .01). Oral anticoagulation was a protective factor for survival among the bioprosthetic valve patients (P = .024). CONCLUSIONS: In the present limited cohort of patients younger than 60 years old, biologic aortic valve replacement was associated with reduced mid-term survival compared with survival after mechanical aortic valve replacement. Despite similar valve-related event rates in both groups, the better hemodynamic performance of the mechanical valves and/or protective effect of oral anticoagulation seemed to improve the outcome. The transcatheter valve-in-valve intervention as potential treatment of tissue valve degeneration should not be considered the sole bailout strategy for younger patients because no evidence is available that this would improve the outcome.