Liver resection for recurrent sarcoma with allogenic aortic patch as partial inferior vena cava replacement – case report and review of literature

Summary

In this case report, we discuss the topic of hepatic sarcomas, their occurrence and treatment options. We present a case of successful radical resection in a young man with recurrent sarcoma of the liver with inferior vena cava infiltration. We describe the surgical procedure in steps and point out the severity of the disease with frequent recurrences. We also review evidence behind the topic aimed to support our treatment approach.

Key words:
hepatectomy – sarcoma – inferior vena cava – blood vessel prosthesis – allograft

INTRODUCTION

Soft tissue sarcomas are a heterogeneous group of malignant tumours derived from mesenchymal cells. Normally they differentiate into muscle, adipose tissue, ligament, bone or cartilage [1]. It is a very heterogeneous group of malignant tumours [2]. There are more than 40 histological subtypes [1,2]. Rhabdomyosarcoma is the most common type in childhood. Typical adult types include non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) – malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma, etc. [1]. Primary hepatic sarcomas (PHS) of the liver are extremely rare in adults and only slightly more common in children [3]. They are mostly chemoresistant. Prediction of biological behaviour is difficult and range of effective cytostatic drugs is limited [1]. Optimal therapeutic approaches remain unclear [3]. Treatment of choice seems to be surgical resection. Surgical radicality of the procedure (R0 resection) is crucial for the patient’s survival [3,4,6]. We would like to present a case of a patient with recurrent sarcoma of the liver with infiltration of the inferior vena cava (IVC), which was treated by liver resection, IVC resection and its replacement with aortic homograft. Using a brief overview of the literature, we tried to determine the number of similar cases.

CASE REPORT

Male patient, age 31, blood group B+, no medical history until 2012. In 2012, the patient was diagnosed with a 25cm tumour of the right kidney, histologically primitive neuroectodermal tumour (PNET). The patient underwent a right nephrectomy and adrenalectomy followed by chemotherapy (CHT). In 2013, a resection of two liver metastases – PNET, was performed. Thereafter, the patient was diagnosed with kidney failure on the basis of CHT (topotecan+cyclophosphamide) and referred for hemodialysis. He was then referred to our department with a recurrent liver mass for a second opinion regarding possible resection; surgeons in the primary hospital refused liver resection because of suspicious vena cava infiltration (Figs. 1,2).

The surgical procedure performed was a right hemihepatectomy with resection of segment I, diaphragm resection, resection of the inferior vena cava and replacement with allogenic aortic patch, radical lymphadenectomy (Fig. 3).

The surgical procedure step by step: patient in the supine position, right subcostal incision, liver dissection and intraoperative ultrasound evaluation. The ultrasound confirmed the tumour of the right hepatic lobe 7x6x8 cm in size, IVC was partly involved in the tumour above the right renal vein, about one third to half of the vena cava diameter. There was no thrombus in the IVC on the scan.

Right sided portal vein and right hepatic artery were dissected, ligated and transsected. The liver resection margin was marked with diathermy. Liver resection was performed using Cusa, clips and stitches. At the end of the resection, the tumour was only attached to the IVC. Satinski vascular clamps were placed above the tumour but below the hepatic veins and below the tumour above the renal veins. The tumour with two thirds of the perimeter of the IVC 9cm in length was resected (Figs. 4,5). A 5x10cm area of the diaphragm was also resected because of ingrowth of the tumour. The patient was classified as an urgent candidate for vascular graft transplantation and placed on the waiting list. He received a graft from a different blood type. Fresh aortic allograft, blood type 0, was prepared for use (Fig. 6). All the graft arterial lumbar branches were closed with 6/0 monofilament non-absorbable stitches. Patch plasty of the resected IVC was performed with a running suture (Fig. 7).

The surgery was uneventful, the operation took 9 hours and 30 minutes and blood loss was 2500 ml. After the surgical procedure, the patient was placed at the general resuscitation and intensive care unit, where he spent 10 days. After this time the patient was transferred to the surgical intensive care unit, after 9 days to the surgical ward. The patient was discharged from the hospital 23 days after surgery. At the time of discharge the patient was fully independent, on a normal diet, without drains and no skin staplers in place. Postoperatively, we continued anticoagulation with low molecular weight heparin (LMWH) and we also started immunosuppressive therapy (rapamycine, Rapamune). We maintained the LMWH until an effective level of immunosuppression was achieved. We decided to use immunosuppression as there is a probable risk of developing thrombus on the graft. The evidence behind this suspicion is weak. The first outpatient clinic visit was planned 5 days after discharge. At this time the patient was in good condition. Histologically, the specimen was diagnosed as metastasis of Ewing sarcoma/PNET with invasion into the IVC wall (Figs. 8,9). The patient is now 8 months after surgery, feeling well, without signs of recurrence, followed up at the oncology department with no need for adjuvant therapy.

DISCUSSION

Since PHS is a relatively rare malignancy, only few series and case reports have been discussed about the management and outcome of PHS in the literature [4]. They represent less than 1% of all hepatic malignancies [4,12]. Primary tumours of the liver were mainly primary hepatic angiosarcoma and leiomyosarcoma [4,12]. They are rare tumours with poor prognosis [7]. There are no distinct clinical characteristics that allow the diagnosis of hepatic sarcoma to be made solely on the basis of histology, physical examination and imaging studies [3]. Symptoms are nonspecific, weight loss [4], right upper abdominal pain [4, 7] and fever [7] are the most common symptoms. Regarding image studies, the most favorable modalities are multiphase computed tomography (CT) and magnetic resonance imaging (MRI) [4].Some 10% of patients are diagnosed at the stage of primary generalized disease [1]. Unless there isn´t an absolute contraindication, liver resection should be performed. Involvement of the vascular structures is not a contraindication [11]. Hepatic resection for sarcoma or sarcoma metastases is a reasonable management strategy if a complete resection (R0) may be achieved [3,4,6].

For example Lin et al. [4] describe the case of 13 patients who underwent surgery for PHS. Nine of them underwent liver resection, two liver transplantations (LTx) and the others received non-operative treatments. The size of the tumour was sized from 0.6 cm to 16 cm. The most frequent procedure was a right lobectomy – 6 patients, left lobectomy – 2 patients and resection of segment IV – 1 patient. R0 resection was performed in nine cases, in two it was an R1 resection. The 2-year survival rate of patients with R0 resection was 77.8% but the two patients with R1 resection died within 1 year. Median survival was 31.4 months. One-year survival rate was 72.7% and 5-year survival rate was 36.4%. Poggio et al. [3] described 20 patients who underwent liver resection (19 patients) and LTx (1 patient) for PHS. The 5 –year survival rate for all patients was 37%. With complete resection, patients with high-grade tumours had a 5-year survival rate of 18% compared with 80% for patients with low-grade tumours. He also described that better adjuvant chemotherapy is necessary especially for high-grade malignancies. Liver transplant for primary or secondary hepatic sarcomas have poor outcomes and was abandoned except for hepatic hemangioendothelioma [3,4].

Sarcomas metastatic to the liver need to be distinguished from PHS [3]. The value of hepatic resection for metastatic sarcoma is unknown [5]. Metastases originated mostly from gastrointestinal stromal tumour (GIST), or leiomyosarcoma [5,6]. The terapeutic options for patients with liver metastases from sarcoma are chemotherapy, ablation, liver transplantation and partial hepatectomy. Complete resection of liver metastases from sarcoma in selected patients is associated with prolonged survival [5]. Traditionally, chemotherapy has been used for patients with metastatic sarcoma, although chemotherapy generally does not provide a survival benefit in sarcoma [5].

The use of neoadjuvant chemotherapy isn´t currently a standard in treatment of operable sarcomas [1]. In selected cases of intrahepatic tumour recurrence, even repeated liver resection might be worthwhile [6].

The records of 26 patients who between 1982 and 1996 underwent a total of 34 liver resections for hepatic metastases from leiomyosarcoma were reviewed by Lang et al. [6]. There were 23 first, 9 second, and 2 third liver resections.  Five-year survival for all of the patients was 13%, 20% after R0 resection. After repeat liver resection the median survival was 31 months, there was no 5-year survivor in the overall group after repeat liver resection.

DeMatteo et al. [5] mentioned 56 cases of patients who underwent hepatic resection for sarcoma metastases to the liver. Most of them had GIST. One-year survival after operation was 88%, 5-year survival 30%. But the 5-year survival rate in patients who didn´t undergo complete resection was only 4%. Liver resections were categorized into four types: wedge resection, segmentectomies, lobectomies and extended resections. Recurrence after complete hepatic resection occurred in 84%. The median time to recurrence was 16 months.

Whilst searching for evidence, we found some thirteen papers discussing resection of the IVC with need for replacement [8−11,1−21]. Sarcomas were most common. Usually, when the IVC needs to be replaced, reinforced polytetrafluoroethylene (PTFE) prosthetic grafts of various sizes are commonly used [3, 6]. However, the limits of this well-established graft are mainly the risk of prosthetic graft occlusion and/or infection [8,10]. The replacement was done with aortic homograft in three cases [16,20,21], with fresh inferior vena cava allograft in five cases [8−11,19]. Svab et al. [13] described leiomyosarcoma of the IVC treated by excision of the front wall with tumour and its replacement by Goretex patch.

Guerrero et al. [9] reported a case of a patient with primary leiomyosarcoma of the IVC after its resection and replacement with fresh IVC allograft without the need of immunosuppression or long-term anticoagulation.

Currently, venous and arterial allografts are considered tissue inducing an immune response, which is similar to rejection in patients after parenchymal organ transplantation. Their immunogenecity is conditional to expression of HLA class I and II in the vessel wall. Nevertheless, the use of immunosuppression after transplant of vascular allografts isn´t common in the world. Approach in individual centres varies from its absolute rejection to use of triple combination immunosuppressants as after transplantation of solid organs [22].

In our case, we achieved R0 resection. We chose immunosuppression with rapamycine because of its anti-tumour effect [23]. We used anticoagulation for only two weeks. After this time, the effective level of immunosuppression was achieved. The patient is now 9 months after surgery, with no signs of recurrence. Adjuvant chemotherapy was not needed, because, in accordance with the literature, it doesn´t prolong survival [5].

CONCLUSION

Primary and secondary sarcomas of the liver are very rare malignant tumours. They are mostly resistant to chemotherapy and the prognosis is poor in most cases. Therefore, surgical treatment (R0 resection) is the only effective therapy to improve the prognosis and quality of the patient´s life. We present a case of a successful R0 resection of recurrent liver sarcoma and replacement of the inferior vena cava with an aortic homograft.

LIST OF ABBREVIATIONS

CHT – chemotherapy

CT – computed tomography

GIST – gastrointestinal stromal tumour

HLA – human leukocyte antigen

IVC – inferior vena cava

LMWH – low molecular weight heparin

LTx – liver transplantation

MRI – magnetic resonance imaging

NRSTS – non-rhabdomyosarcoma soft tissue sarcoma

PHS – primary hepatic sarcoma

PNET – primitive neuroectodermal tumour

PTFE - polytetrafluoroethylene

Conflict of Interests

The authors declare that they have not conflict of interest in connection with the emergence of and that the article was not published in any other journal.

Veronika Lovasova, M.D.

Department of Transplant Surgery

Institute for Clinical and Experimental Medicine (IKEM)

Videnska 1958/9

140 21 Prague 4

e-mail: veronika.lovasova@ikem.cz