Leiomyosarcoma of the Inferior Vena Cava - Radical Resection, Vascular Reconstruction and Challenges: A Case Report and Review of Relevant Literature

Abstract

Leiomyosarcomas of the inferior Vena Cava (IVC) are rare soft tissue sarcomas accounting for only 0.5% of all soft tissue sarcomas in adults with fewer than 300 cases reported. Extraluminal tumor growth along the adventitia of the IVC seems to be the common presentation. Intraluminal tumor growth is rare. The origin of the tumor is divided into three levels in relation to the hepatic and renal veins. The presentations and surgical modalities vary accordingly. Retroperitoneal tumors are often not diagnosed until the disease is at an advanced stage with large tumor growth and involvement of surrounding structures. This is partly because of the nonspecific clinical presentation as well as absence of early symptoms. Most patients present with abdominal or flank pain. Symptoms vary according to the dimensions of the tumor, growth pattern and localization of the tumor. Radical en bloc resection of the affected venous segment remains the only therapeutic option associated with prolonged survival. The goals of surgical management of these tumors include the achievement of local tumor control, maintenance of caval flow, and the prevention of recurrence. The involvement of renal or hepatic veins determines the strategy for vascular reconstruction. Reconstruction of the IVC is not always required, because gradual occlusion of the IVC allows the development of venous collaterals. However, when pararenal leiomyosarcoma of the IVC is present, reconstruction of the IVC and the renal vein is necessary to prevent transient or permanent renal dysfunction. Recent study has shown that radical surgery combined with adjuvant multimodal therapy has improved the cumulative survival rate. We report a case of IVC leiomyosarcoma in a young healthy woman along with details of its diagnostic workup and discussion of the surgical options and reconstruction of caval continuity.

Challenging Inferior Vena Cava Leiomyosarcoma: A Case Report and Literature Review of Management

“…Consistent with other published reports [1], this report demonstrates that surgical resection can be safely performed and is associated with improved survival.…”

“…The three most common presenting symptoms are abdominal pain (52%), distention (20%), and deep venous thrombosis (12%) [1]. Other symptoms include Budd-Chiari syndrome, weight loss, fever, weakness, anorexia, vomiting, night sweats, and dyspnea.…”

“…Leiomyosarcomas tend to enlarge via intraluminal or extraluminal growth rather than in filtration [1].Imaging usually demonstrates extraluminal tumor in the retroperitoneum. If they involve adjacent organs, these tumors are often mistaken for primary LMS arising from retroperitoneum rather than the IVC.…”

“…Being that rare, available evidence that is used to guide management and follow up is mostly in the form of case reports and case series [1].…”

Leiomyosarcoma of Intrahepatic IVC - Treatment with Radical Resection and IVC Replacement with PTFE Graft: A Case Report and Review of Literature

“…Submit your Manuscript | www.austinpublishinggroup.com presentation with IVC occlusion or Budd-Chiari syndrome [20].…”