Fig. 1
Admission imaging work-up of a 54-year-old woman. The computed tomography scan confirmed the anatomo-pathologic finding of a biopsy-proven primary leiomyosarcoma of the retro-hepatic vena cava (Panel A). Magnetic resonance imaging allowed a detailed definition of oncologic involvement of the heart right atrium (Panel B)
MRI allowed detailed definition of the margin of the intracardiac involvement (Fig. 1, Panel B). She was well compensated because her IVC was completely thrombosed above the renal veins, and she had established collaterals.
After a biopsy-proven diagnosis of primary leiomyosarcoma of the retro-hepatic vena cava due to multiorgan involvement, and the location at the junction of the IVC and right atrium, it was determined that cardiopulmonary bypass and possible circulatory arrest would be necessary to resect the tumor.
A cardiac surgeon was consulted to plan a combined procedure, and the patient was admitted to the hospital for a cardiac catheterization, which was negative for severe pulmonary hypertension.
It was agreed to proceed, and in August 2014 the patient was taken to the operating room of UPMC and put on full cardiopulmonary bypass.
The incision was a long midline from the sternal notch to below the umbilicus. The vena cava was removed with the liver attached from just above the renal veins up to and including the inferior portion of the right atrium (Fig. 2).
Fig. 2
The right atrium is open and to the left. The inferior portion of the right atrium with sarcoma and IVC to the right (arrow). The image shows a cardiac sump pump and one of the bypass cannulas
The tumor in continuity with the inferior vena cava was resected from the liver on the back table. During this phase, the liver was flushed with 4 l of histidine-tryptophan-ketoglutarate solution (Fig. 3).
Fig. 3
Explanted liver showing the leiomyosarcoma occluding the retro-hepatic IVC
During the operation, the cardiac surgeons harvested the patient’s pericardium, treated it with glutaraldehyde solution, combined it with a small amount of bovine pericardium, and then constructed a tube graft from that.
They then sewed the tube graft to the inferior part of the right atrium so that there was a smaller orifice to sew the liver to. Once the cardiac surgeons had attached the pericardial tube graft, the patient was converted from cardiopulmonary bypass to standard VVB (Fig. 4). The liver was reimplanted using the confluence of the three hepatic veins to the pericardial graft. The artery and portal vein were reconstructed end to end, and the bile duct was reconstructed with a Roux-en-Y.
Fig. 4
This image shows the reconstructed inferior vena cava graft reattached to the right atrium (the heart to the left). The graft was constructed from the patient’s pericardium (blue arrows) plus a small portion of bovine pericardium (black arrow)
An intraoperative ultrasound was done to confirm valid flows to and from the left liver. Operating time was 767 min, and 14 units of blood were used during the procedure.
The histology report of the excised surgical specimen confirmed the diagnosis of leiomyosarcoma of the IVC, with a tumor mass of 115 g and 8.8 × 5 × 4.7 cm. The serially parallel sectioning to the long axis revealed a mottled, tan, trabecular cut surface with focal hemorrhage and yellow peripheral necrosis, 1 × 0.8 × 0.6 cm. An irregular rim of the atrium was present on the upper polar side, measuring 0.8 cm in length and 0.2 cm in thickness (Fig. 5).
Fig. 5
Surgical specimen after radical tumor excision. Worth noting is the pathologic adhesion between the leiomyosarcoma (blue arrows) and the IVC (black arrow)
The patient did well postoperatively, with no complications. She was extubated on the second postoperative day. She was discharged from the hospital on the tenth postoperative day, has had no complications nor required readmission, and is alive and well at the time of this writing.
Uneventful postoperative CT scan to confirm the absence of vascular anatomic complications was done 2 months after surgery, with no complaints (Fig. 6).
Fig. 6
Follow-up computed tomography 2 months after surgery for IVC tumor, showing resolution of the preoperative pathologic condition
Conclusion
Technical skills in advanced hepatobiliary surgery, patient hemodynamics and resuscitation, diagnostic evaluation, operative indications by grade of IVC tumor involvement, selection criteria for operative management, and criteria for the choice of operation are mandatory for indicating formal tumor excision and liver resection as initial or delayed management of patients with involvement of the IVC by hepatic tumors or those with large centrally located lesions or lesions in close proximity to the confluence of the IVC and hepatic veins.
Cross-References
References
Azoulay D, Andreani P, Maggi U et al (2006) Combined liver resection and reconstruction of the supra-renal vena cava. The Paul Brousse experience. Ann Surg 244(1):80–88CrossRefPubMedPubMedCentral
Azoulay D, Maggi U, Lim C et al (2014) Liver resection using total vascular exclusion of the liver preserving the caval flow, in situ hypothermic portal perfusion and temporary porta-caval shunt: a new technique for central tumors. Hepatobiliary Surg Nutr 3(3):149–153PubMedPubMedCentral
Fortner JG, Shiu MH, Kinne DW et al (1974) Major hepatic resection using vascular isolation and hypothermic perfusion. Ann Surg 180:644–652CrossRefPubMedPubMedCentral
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