Leiomyosarcoma of the retrohepatic portion of the inferior vena cava: Clinical presentation and surgical management in five patients

Abstract

Leiomyosarcoma (LMS) is a rare primary soft tissue sarcoma arising from the inferior vena cava (IVC). For LMS involving the retrohepatic portion of IVC there are limited published data about tumor features, surgical strategies, and IVC replacement. Clinical data, surgical procedures, and pathologic features of five consecutive patients referred for IVC-LMS, in 5 years, were reviewed. A complete surgical resection of the tumor was performed in each patient and IVC replacement used expanded polytetrafluoroethylene grafts. Abdominal pain (n = 4) and palpable flank mass (n = 3) were the most frequent signs. To assure a complete tumoral exeresis, adjacent organ resection included hepatectomy (n = 4), extended right nephrectomy (n = 3), and right adrenalectomy (n = 1). Prosthetic IVC reconstruction was performed in four patients, three times associated with arteriovenous fistula. Median postoperative stay was 18 days. No prosthetic-related complication was observed, venous insufficiency sequela did not occur. Tumoral clearance was achieved in all patients, and direct tumoral involvement of the liver was less frequent than for kidney. Three patients died at a median followup of 34 months, two are alive and disease-free at 34 and 44 months. LMS of the IVC is characterized by locally advanced status at the time of diagnosis. A radical tumoral resection associated with liberal use of venous prosthetic replacement may offer a chance for cure and good quality of life in palliative situations.