Primary leiomyosarcoma of the inferior vena cava: Reports of infrarenal and suprahepatic caval involvement

Abstract

We report two cases of primary inferior vena cava (IVC) leiomyosarcoma. The first patient was a 60-year-old female who presented with abdominal pain. The patient was initially diagnosed with a retroperitoneal sarcoma that may have involved the right renal vessels and the IVC. The Vascular Surgery Service was consulted intra-operatively when it became evident that the IVC was primarily involved. The patient was treated with total en-bloc excision of the infrarenal IVC tumor with concomitant interposition polytetrafluoroethylene (PTFE) graft caval replacement. The second patient was a 58-year-old female who presented with general malaise and anemia. This tumor began in the distal infrarenal IVC and extended cephalad to the suprahepatic IVC, ending approximately 2 cm below the right atrium. Intrapericardial IVC clamping was required for cephalad control in this patient, who was then treated with excision of the tumor and concomitant interposition PTFE graft caval replacement with reimplantation of the right renal vein. A review of this rare tumor is presented.

Diagnosis and management of inferior vena cava leiomyosarcoma: review of the literature with a case presentation

“…Mingoli et al [3] described the following three regions within the IVC for tumor involvement: (i) the lower segment (infrarenal) (37%), (ii) the middle segment (from the hepatic veins to the renal veins) (44%), and (iii) the upper segment (from the right atrium to the hepatic veins) (20%). [3,9,15] Similarly, according to Kulaylat, [5] primary tumors of the IVC occur in three different segments: segment I (below the inflow of the renal veins), segment II (from the inflow of the renal veins to the inflow of the hepatic veins, excluded), and segment III (from the inflow of the liver veins up to the right atrium). The authors found that segment II was more often involved (46%) than segment I (38%) or segment III (16%).…”

“…The use of cavography is also important in cases of IVC occlusion in order to demonstrate collateral flow and plan the appropriate surgical strategy. [2,10] Confirmation of the diagnosis is done via a histopathological examination after intravenous, fluoroscopic CT, a duplex-guided biopsy, or usually after surgical resection, [9] as was performed in our case.…”

“…Moreover, dilated veins on the abdominal wall, palpable mass, abdominal pain, deep venous thrombosis (DVT), and lower extremity edema can be seen at the infrarenal level(segment I-lower) involvement. [2,9] Additional nonspecific symptoms may include fever, weakness, anorexia, nausea, vomiting, night sweat, and dyspnea, some of which may be a result of metastasis. [2,10] These symptoms usually develop at a late stage due to the tumor growth which delays the diagnosis and worsens the prognosis.…”

“…[4] Clinical staging has been suggested as being either occlusive (presenting with a mass, edema, or thrombophlebitis), nonocclusive (asymptomatic or an incidental finding), or terminal (distant metastasis). [9] Prognosis Inferior vena cava leiomyosarcoma has a poor prognosis with surgical resection playing a critical role in patient survival. The risk of death is higher for proximal segment disease and is the lowest for middle segmental involvement.…”

“…[13] Therefore, it is recommended that an incompletely occluded IVC with insufficient collateralization should be replaced with a prosthesis, whereas a completely occluded IVC that does not involve renal veins inflows and which is richly collateralized can be ligated without functional impairment. [7] Performing surgery to create an arteriovenous fistula (AVF) either between the aorta and IVC or iliac vein or between the femoral vessels [9] has also been recommended in order to boost flow velocity, which would then improve the patency of the IVC graft. Proponents of this procedure state that it would make long-term anticoagulation therapy unnecessary.…”