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

Case Report

Austin J Clin Case Rep. 2017; 4(2): 1118.

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

Chaudhary RJ¹, Rastogi A¹, Pahari H¹, Thiagarajan S¹, Bhangui P¹, Goja S¹, Gautam D², Piplani T³ and Soin AS¹

¹Medanta Institute of Liver Transplantation and Regenerative Medicine, India

²Department of Pathology, Medanta -the Medicity, India

³Department of Radiology, Medanta -the Medicity, India

*Corresponding author: Rohan Jagat Chaudhary, Medanta Institute of Liver Transplantation and Regenerative Medicine, Gurugram, India

Received: March 23, 2017; Accepted: June 15, 2017; Published: June 22, 2017


Leiomyosarcomas of the Inferior Vena Cava (IVC) are rare tumors accounting for only 0.5% of all soft tissue sarcomas in adults with fewer than 300 cases reported. Intraluminal leiomyosarcoma of IVC is rare. The involvement of renal or hepatic veins determines the strategy for vascular reconstruction. Recent studies have shown that radical surgery with adjuvant multimodal therapy improves the cumulative survival rate. We report a case of IVC leiomyosarcoma in a young healthy woman along with diagnostic workup followed by resection of tumor and reconstruction of IVC by Polytetrafluoroethylene (PTFE) graft. She reports no recurrence/complications after 6 cycles of chemotherapy and 40 months of follow-up. The prognosis depends on grade of the tumor, segment of the IVC involved (level 2-better prognosis), size of tumor (size<9 cm better prognosis) and adjuvant therapy. However, overall leiomyosarcoma runs a poor prognosis; over half of the patients with surgical excision develop recurrence. The 5-year survival rate for these patients ranges between 31 and 62%.

Keywords: Inferior vena cava; Leiomyosarcoma; PTFE (Polytetrafluoroethylene ); Vascular reconstruction


Primary vascular leiomyosarcoma is a rare tumor of mesenchymal origin and arises from the smooth muscle cells of the tunica media predominantly within the inferior vena cava [1,2]. Extraluminal tumor growth along the adventitia of the Inferior Vena Cava (IVC) seems to be the common presentation [1,3]. Intraluminal tumor growth is rarely found. The origin of the tumor is described in relation to the hepatic and renal veins. The IVC is divided into three levels [1,4-6]: Level 1 extends from the entry of the hepatic veins up to the right atrium; Level 2 comprises the area between the confluences of the renal and hepatic veins; Level 3 includes the area below the renal veins.

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 [1,4].

Radical en bloc resection of the affected venous segment remains the only therapeutic option associated with prolonged survival [1,4,5]. Ito, et al. in a recent study with leiomyosarcoma of the IVC concluded that radical surgery combined with adjuvant multimodal therapy had a 5-year cumulative survival rate of 62% [6].

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 [1,5,7].

We report a case of leiomyosarcoma of intrahepatic IVC with discussion of the surgical procedure and reconstruction of caval continuity.

Case Presentation

A 40 year old previously healthy female presented with abdominal pain and generalised discomfort over a span of 3 months. She was advised a routine USG abdomen which showed a retroperitoneal mass in right hypochochondrium, rest was normal. Her Complete Blood Count (CBC) and basic metabolic panel were within normal limits. Tumor markers [Beta Human Chorionic Gonadotropin (HCG), Ca 125, Alfa Fetoprotein (AFP), Ca 19.9, Carcinoembryonic antigen (CEA)] were normal.

Radiology workup

Contrast Enhanced Computed Tomography (CECT) Abdomen revealed well-defined homogenous, hypodense mass measuring 9.9 cm (CC) X 5.5 cm (TR) X 6 cm (AP) in upper retro peritoneum on right side, in close relation to IVC in its intrahepatic course. The mass showed moderate post-contrast enhancement and no necrosis/fat density/calcification. The mass was abutting posterior wall of IVC with significant compression of its lumen in the intrahepatic course. Right adrenal gland appeared adherent to the mass. Hepatic venous confluence into IVC was normal. Right renal vein was not visualized and right kidney was cystic - dysplastic, non functional. Left renal vein was normal. A differential diagnosis of either level 2 IVC tumor (leiomyoma or a low grade leiomyosarcoma), exophytic right adrenal mass or mesenchymal tumour of retroperitoneum was made.

A Positron Emission Tomography (PET) scan was performed from skull base to mid thigh with concurrently acquired triphasic CT for attenuation correction and anatomical localisation. It showed that the mass was non-FDG avid and there was no evidence of extrahepatic disease (Figure 1).