Leiomyosarcoma of the Inferior Vena Cava Confirmed by Aspiration Biopsy With a Catheter During Digital Subtraction Angiography

Introduction

Inferior vena cava leiomyosarcoma (IVCLMS) is a rare, slow growing smooth muscle sarcoma arising from the media of the inferior vena cava (IVC) with intra- or extraluminal growth. ¹ Inferior vena cava leiomyosarcomas are encountered predominantly in females, and the mean age is around 60 years. ¹ The patients’ complaints are often nonspecific and may include dyspnea, malaise, nausea, vomiting, fever, abdominal pain, and lower extremity edema, and the diagnosis is often challenging and delayed. Wide surgical excision with negative margins is the only potentially curative approach; however, IVCLMS is often diagnosed at advanced stage and negative surgical margins often cannot be achieved. ^2,3 Radiologic diagnosis of IVCLMS can be achieved by various imaging techniques, such as computed tomography (CT), magnetic resonance imaging (MRI), cavography, and ultrasound (US). Definitive diagnosis of IVCLMS needs histological analysis, and obtaining of the tissue is usually done by laparotomy or percutaneous needle aspiration/biopsy. ⁴

Here we report the fourth IVCLMS case confirmed by catheter suction biopsy during digital substraction angiography (DSA) in the English literature to the best of our knowledge. ^{5 –7}

Case Report

A 65-year-old male presented to the emergency department with dyspnea and syncope. He complained of fatigue with effort for the last 2 weeks and edema in the legs 20 days ago. With the suspicion of pulmonary emboli, a pulmonary CT-angiography (CTA) was performed, and thrombi were detected in the main pulmonary arteries and in the right lung upper lobe arteries including the distal branches. Bilateral lower extremity venous Doppler examination was normal, and no deep vein thrombosis was detected. Echocardiography was also normal. Subsequent contrast-enhanced abdominal CT revealed an enhancing lesion causing total occlusion in the IVC (Figure 1A-D). In addition, contrast flow into the left renal vein by 2 paracaval and paraaortic collateral venous structures was detected. Also, 3 lumbar veins probably thrombotic or invaded by the tumor were seen.

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Figure 1.

A-D, Axial noncontrast and contrast-enhanced abdominal computed tomography (CT) images (A and B) reveal a partially enhancing lobulated mass lesion in the inferior vena cava. Coronal arterial phase image (c) reveals the enhancing mass lesion and coronal venous phase image (d) shows the extension of the thrombi.

The patient was referred to our interventional radiology service for the placement of an IVC filter to prevent further pulmonary artery emboli and to obtain a sample of the lesion during the procedure. We accessed the right axillary vein and a 6F 90-cm sheath (Cordis [NJ, USA], Johnson&Johnson Medical NV/SA [Japan]) via 0.035 M-angled-type guidewire (Radiofocus Guidewire M-angled Type 0.035 180 cm, Terumo [NJ, USA]) was placed. Through the sheath via guidewire, a 5F pigtail catheter (Cordis, Johnson&Johnson Medical NV/SA) was placed to the suprarenal portion of the IVC and venogram was performed, which showed occlusion of the IVC in the renal vein entry point as a large filling defect corresponding to the mass seen on CT. A filter (Optease Retrievable Vena Cava Filter 6F Femoral&Juguler, Cordis, Johnson&Johnson Medical NV/SA) was placed to the distal end of the lesion by DSA via 6F intraducer. We then manipulated a 5F Picard catheter (Cordis, Johnson&Johnson Medical NV/SA) into the infrarenal portion of the IVC mass through the IVC filter (Figure 2). We performed simple aspiration via the 5F Picard catheter in the lesion region. The 5F Picard catheter was then removed and a follow-up venogram was performed, which did not demonstrate any contrast extravasation into the retroperitoneal space. No complication was seen during or after the procedure. Histopathology of the aspiration material was consistent with spindle cell sarcoma, and immunohistochemical analyses revealed leiomyosarcoma. Positron emission tomography-computed tomography (PET-CT) revealed fluorodeoxyglucose (FDG) uptake (standard uptake value (SUV) max: 10.14) in the segment between the caudal end of the filter and bifurcation within the thrombus and vein wall. Hypermetabolism (SUVmax: 5.47) was detected in the lesions in the paraaortic, retrocaval, and interaortacaval space, and the largest lesion was 1 × 1.3 cm in size. These lesions were evaluated as lumbar vein thrombosis or tumor infiltration in correspondence with the previous contrast-enhanced abdominal CT.

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Figure 2.

Venogram showing the inferior vena cava (IVC) filter and the 5F Picard catheter placed into the infrarenal portion of the IVC mass through the IVC filter.

The patient was operated; intraoperative US was performed, and a hypervascular tumor from the bifurcation to the suprarenal segment obliterating the lumen was verified. Also, tumor extension to the 3 lumbar veins was detected. No lymphadenopathy was seen. Subsequently, venotomy was performed, and the mass within the vein and the formally placed filter were removed. The patient was discussed in the “tumor council” of the hospital, and treatment with chemotherapy was decided. The patient had received anticoagulation treatment starting from the initial diagnosis of the pulmonary emboli, and postoperative anticoagulation treatment was continued with Coumadin. The control thorax and abdominal CT obtained 3 months later showed postoperative changes with no residue or recurrent enhancing mass lesion in the vena cava inferior (Figure 3). Chronic thrombi in the IVC and proximal iliac veins and also left lung lower lobe pulmonary arteries were seen. Reporting of this case was approved by the local ethics committee.

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Figure 3.

Control abdominal computed tomography (CT) obtained 3 months later showing the postoperative changes with no residue or recurrent enhancing mass lesion in the vena cava inferior and chronic thrombi in the vena cava inferior and proximal iliac veins.

Discussion

Primary IVC malignancy is extremely rare. Although leiomyosarcoma represents less than 1% of all malignancies, it accounts for more than 75% of tumors arising from the large veins ^7,8 and leiomyosarcoma is the most common primary malignancy involving the IVC. This rare malignant tumor originates from the smooth muscle cells of the media with intra- or extraluminal growth. The type of the lesion is further divided into 3 levels in relation to hepatic and renal veins, respectively: Level I describes tumors below renal veins, while level II refers to lesions including renal and hepatic veins.Level III leiomyosarcomas include the entry of hepatic veins to right atrium. ⁹ This condition is frequently diagnosed only at an advanced stage partly due to the presentation with nonspecific symptoms such as dyspnea, malaise, nausea, vomiting, fever, abdominal pain, and lower extremity edema. ¹ Inferior vena cava leiomyosarcoma contains interlacing bundles of smooth muscle cells with variable uniformity and shows similar histologic features as those of leiomyosarcomas from other locations. Immunohistochemistry is needed to exclude other spindle cell malignancies. Accurate radiologic diagnosis of IVCLMS can be attained by combination of various imaging techniques; however, definitive diagnosis of IVCLMS needs histological analysis, and obtaining of the tissue is usually done by laparotomy or percutaneous needle aspiration/biopsy using US or CT guidance. ⁴ Percutaneous biopsy carries a risk of bleeding, and the method of suction biopsy during DSA should be considered in chosen appropriate cases. Radical tumor resection is associated with better 5- to 10-year survival rates (49.4% and 29.5%, respectively) compared to palliative resection or inoperable. The 5-year cumulative survival rate is 53%, and aggressive surgical management combined with adjuvant therapy offers the best treatment for patients with IVCLMS.^{4 , 10} Ten-year survival is 14% and unfortunately more than 50% of patients develop recurrent disease. ¹¹

Due to its rarity, if radiologists are not aware of the imaging features of IVCLMS, it could be misdiagnosed as other more common IVC lesions, especially thrombi and cancer thrombi. An accurate preoperative diagnosis as well as the determination of local and distant extend of the disease is allowed, especially with modern imaging modalities such as CT and MRI. Enhanced CT gives additional details of tissue heterogeneity with areas of hemorrhage or necrosis as well as adhesion or replacement of surrounding tissue. ⁵ The tumor is usually hypovascular but may show peripheral enhancement following contrast injection. On MRI, these lesions are characterized by the iso or low signal on T1-weighted image and iso or high mixed signal on T2-weighted image. Intense heterogeneous enhancement of the mass helps distinguish it from thrombus on postcontrast MRI images. Furthermore, magnetic resonance angiography (MRA) or CTA can evaluate the extent of vascular infiltration and the relationship between the tumor and the IVC branches. The differential diagnosis for primary IVCLMS include other more common IVC lesions such as thrombi and cancer thrombi, which are most commonly seen with hepatocellular carcinoma and renal cell carcinoma.

Digital subtraction angiography is a type of fluoroscopy used in interventional radiology. In the diagnosis of vascular neoplasms, besides showing the level of obstruction and bypass circulation, catheters of different calibers are available to study the status of IVC branches. Catheter aspiration via DSA carries a risk of tissue dislodgment and embolism. No complication was seen during or after the procedure in our case. There are 3 case reports on suction biopsy of IVCLMS during DSA in the English literature to the best of our knowledge. ^{5 –7} The first case was reported in 1998 by Shimoda et al; however, there was no mention of the technique used to obtain the biopsy specimens. ⁷ Abdel-Aal et al ⁶ and Wei N et al ⁵ reported their successful suction biopsy experience with the techniques in detail. Abdel-Aal AK et al accessed the right common femoral vein and they placed an 8F, 25-cm sheath. Through the sheath, they performed venogram, which showed occlusion of the entire IVC by virtue of a large filling defect. They then manipulated a 5F Berenstein catheter into the residual lumen lateral to the infrarenal portion of the IVC mass and using an Amplatz extrastiff guidewire, they advanced the 8F sheath into the residual IVC lumen. With the Amplatz wire left in place for support, they advanced a biopsy needle through the sheath and obtained 3 core biopsy samples. Wei et al inserted the catheters through right femoral vein by modified Seldinger technique. The DSA showed a large filling defect within IVC, extending from the stem of right renal vein up to the hepatic segment. To procure the tissue, a 6F guiding catheter was extended to the mass, and suction pressure was exerted by a 20-mL syringe. Also, a successful suction biopsy by using a 10F steerable embolectomy catheter—that was different from the simple DSA diagnostic catheter—has been reported in 1 case of pulmonary artery leiomyosarcoma. ¹² All of these procedures were uneventful, and the patients did not develop any complications as in our case.

In conclusion, our experience recommends catheter aspiration as a feasible, quick, and less invasive method to obtain tissue biopsy of the vascular tumors during DSA, especially in cases where percutaneous biopsy carries a risk of bleeding. It is vital to closely monitor the patients for bleeding and embolic complications during and after the procedure.