Resection of Sarcoma Involving the Intrahepatic Vena Cava: Report of 2 Cases From a Specialized Center

Background

Primary leiomyosarcoma (LMS) of the hepatic vena cava is a rare malignancy that has only been published in case reports. Only a few cases with successful R0 resection have been reported in the literature.

Methods

We report 2 similar cases of extended primary LMS of the intrahepatic inferior vena cava (IVC). Both patients previously underwent operations in nonspecialized centers that resulted in inadequate tumor resection. After admission to a high-volume center focusing on the treatment of patients with sarcoma, R0 resection was feasible with a multimodal therapeutic treatment approach.

Results

Radical complete tumor resection was achieved by means of extended right-sided hemihepatectomy (segments V–VIII and I), en bloc resection, and prosthetic replacement of the IVC and nephrectomy in 1 patient. Both patients are currently tumor-free and healthy 6 months postoperatively.

Conclusions

Patients with such complex tumors should be referred to centers with specialized surgeons who can preoperatively estimate whether complete resection may be possible and who are capable of performing such delicate interventions.

Introduction

Primary leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a malignant tumor of mesenchymal origin that is rare but is the most common primary malignancy of the IVC.¹ LMS is a subtype of soft tissue sarcoma (STS). STSs represent <1% of all malignant tumors in adults. Among retroperitoneal or abdominal STSs, LMSs and liposarcomas are the most common histologic subtypes.² Vascular LMSs account for 2% of all LMSs, and 50% of these affect the IVC.³ The prognosis of these tumors is unfavorable because of their slow growth pattern and their location, which allows them to reach quite advanced stages before becoming symptomatic.1, 4 Complete surgical resection within a multimodal therapeutic concept is the most important strategy. R0 resection, however, is often difficult to achieve because of the retroperitoneal location of the tumor and its proximity to vital organs.1, 3, 5, 6 Local disease control has been shown to be crucial during treatment, given the fact that the feasibility of radical surgery decreases with further tumor recurrence.6, 7, 8 Patients with recurrent disease eventually develop widespread multifocal abdominal disease, which is incurable even by modern multimodal treatment concepts and is the leading cause of disease-related deaths in these patients.⁷

To date, only a few cases of successful R0 resection in patients with primary LMS of the hepatic IVC have been reported.⁹ We report 2 patients with extended primary venous LMS of the IVC and discuss their cases with reference to the published literature. In particular, they show that complex sarcoma surgery should be performed at specialized centers and treated within a multimodal therapeutic concept.

A 34-year-old woman with a grade 3 primary LMS of the intrahepatic vena cava underwent an operation in a community hospital (partial resection of the infrahepatic vena cava with patch plastic and right nephrectomy; Table I). A postoperative positron emission tomographic/computed tomographic (PET/CT) scan confirmed a 4 cm × 2.9 cm × 1.8 cm residual tumor around the IVC in agreement with a histologically confirmed R2 resection (Fig. 1A). Tumor resection was followed by a total of 4 cycles of a chemotherapy regimen (etoposide 125 mg/m² on days 1 and 4, ifosfamide 1500 mg/m² on days 1 to 4, and doxorubicin 50 mg/m² on day 1 [EIA]) given every 3 weeks. The chemotherapy was combined with regional hyperthermia using electromagnetic waves aiming for tumor temperatures of 42°C for 60 minutes on days 1 and 4 of each EIA cycle, which has been published previously by Lindner et al.⁶ A PET/CT scan after the hyperthermic chemotherapy revealed a good metabolic response of the residual tumor. The time schedule of the neoadjuvant chemotherapy and diagnostics was as follows: week 0, PET/CT scan; weeks 1 to 13, chemotherapy (4 cycles EIA with regional hyperthermia [RHT]); and week 16, PET/CT scan.

Thereafter, a tumor resection en bloc with an extended right hemihepatectomy (segments V–VIII and I) and complete resection of the intra- and partial infrahepatic IVC, including para-aortal and paracaval lymph nodes and the lumbar portion of the diaphragm was performed at the Ludwig-Maximilians University surgical department. For IVC reconstruction, a GoreTex radially supported prosthetic vascular graft (22 mm in diameter; W.L. Gore and Associates, Newark, DE) was implanted, and the left renal vein was reinserted in the prosthesis (Fig. 1B, C). A Histologic examination revealed microscopically complete remission. The early postoperative course was complicated by infection of the alloplastic graft on postoperative day 7 because of a leak from a perforation of the duodenum. The cause of the perforation is not known, and differential causes are speculative. However, the most likely reasons may be (1) a thermic lesion, caused by the monopolar electric tissue dissector; (2) mechanical erosion by an intra-abdominal drainage, or (3) mechanical erosion by ring gussets of the polytetrafluoroethylene graft, which intraoperatively revealed slight motion caused by the transmitted aortic pulsation. This severe complication was controlled by suturing of the duodenum and replacement of the alloplastic graft in the vena cava position by a homograft of the thoracic aorta in a second operation. Because a histologic examination of the tumor initially revealed a pleomorph LMS (grade 3), the patient underwent 4 more cycles of adjuvant EIA chemotherapy and RHT. Six months thereafter, the patient is in good condition without signs of recurrence.

The second patient is a 59-year-old woman with a LMS of the right upper abdomen infiltrating the IVC and the liver. Initially, the tumor was diagnosed in a community hospital via a CT-guided punch biopsy specimen that revealed a grade 1 LMS (Table I). The first attempt at surgical resection in that hospital was interrupted because of dramatic intraoperative bleeding, which could only be controlled by chemoembolization of the right lobe of the liver. Four months later, the patient was admitted to the sarcoma center of the University Hospital of Ludwig-Maximilians University Grosshadern for reevaluation regarding surgical resection of the tumor. After the patient was discussed in the local interdisciplinary tumor board, primary resection of the tumor was indicated. Before the operation, the patient underwent another CT scan, which revealed a 7.7 cm × 8.4 cm × 11.9 cm large intrahepatic soft tissue tumor with central necrosis conically invading the liver (Fig. 2A, B). A complete en bloc resection of the tumor was performed via right-sided hemihepatectomy (segments V–VIII and I and the right hepatic vein), resection of the IVC, and a right-sided nephrectomy. The IVC was replaced by a GoreTex radially supported prosthetic vascular graft into which the left renal vein was reinserted. Preoperative CT scan–based volumetric examination indicated that the remaining left liver lobe represented 40% of the liver tissue. This was considered enough remnant liver and confirmed that resection of segments V to VIII and I was clinically possible. A histologic examination of the resected tissue revealed an intravenously grown LMS (grade 1) that was 12 cm in length at its greatest diameter. It was adherent to the venous wall without infiltration into the parenchyma of the liver and was completely resected with tumor-free margins (Fig. 2C). After 14 days in the hospital and a complication-free postoperative course, the patient was discharged, tumor-free and in good health. Because of confirmed grade 1 differentiation of the tumor, no postoperative chemotherapy was necessary, and the patient has been tumor-free for 6 months.