Retroperitoneal sarcomas (RPS) that arise from mesenchymal cells account for about 15% of soft tissue sarcomas [1] . Little is known about the biological behavior of the tumor, and no specific etiological associations have been identified [2] . Such tumors are usually large at initial presentation mounting no obvious clinical symptoms [3] . The management of the disease remains a challenge [4] and surgical resection of localized RPS aiming for gross or microscopically negative margins is still the best [5] . However, because of the high rate of local recurrence, the prognosis of the patients with RPS is generally poor [2] . Local recurrence accounts for 77% of RPS-related deaths without concomitant metastasis, it is, therefore, important to control the disease locally [6] . It is common for RPS to involve major arterio-venous structures [7] . Planned vascular resection is essential to achieve the oncologic standards of complete tumor resection with microscopically negative margins and the prevention of tumor spillage during surgery [8] . Still, limited knowledge is available on the extent of surgical resection and the best surgical approach [9] . Three major factors influence the need and the type of vascular replacement: the site of the lesion and especially the relation to renal veins; the extent of IVC involvement (partial or circumferential); and the presence of well-established collateral venous system [9] . RPS tumors extending to major arterio-venous structures might be rare. Due to the limited number of clinical series and the lack of multi-institutional or national registries, the short and long-term benefits following concomitant resection of major arterio-venous structures are still antagonistic [9] .

Our aim in this work was to present our institutional experience with RPS tumors, assess their vascular involvement and to analyze the outcomes (Short and long-term) of onco-vascular approach.

A retrospective review of our institutional retroperitoneal sarcoma database and patients’ clinical charts was performed. All consecutive adult patients surgically treated at our NCI from 2004 to 2010 for primary RPS directly arising from or secondarily involved blood vessels were included. This study included 14 patients; 8 males (57%) and 6 females (43%), their age ranged from 29 - 68 years with median age 51 years. The size of the tumor ranged from 5 - 15 cm with median size of 5 cm in diameter.

Surgical risks were high, and the long-term prognosis is usually poor when there is involvement of major blood vessels in tumor making a limiting factor for curative surgery [13] . Advances in surgical techniques have allowed extending the limits of surgery beyond major vascular resection, with a relatively low rate of postoperative complications [14] .

The number of patients with retroperitoneal STSs requiring major vascular resections is unknown [9] . Leiomyosarcomas, liposarcomas, and other types of sarcomas were associated with vascular involvement [15] . Most studies focus selectively on the very rare entity of primary leiomyosarcoma of the vena cava, neglecting all other sarcomas that involve blood vessels in the retroperitoneum [16] [17] [18] [19] [20] . Most of the STSs secondarily involved vascular structures either by histologically proven vascular infiltration or by vascular encasement [9] .

Retroperitoneal sarcomas are usually large at diagnosis, and the differentiation between primary or secondary blood vessel involvements is not made before the pathologist examines the specimen [10] . This implicates that both have to be treated by a common procedural algorithm and resection strategy [20] . Schwarzbach suggested a four-stage classification that describes the pattern of vascular involvement by retroperitoneal STSs [9] . In the current study, the most common type of vascular involvement was type III, venous involvement (57%). This was in agreement by a large number of reports about leiomyosarcomas of the inferior vena cava [16] [17] [18] [19] [20] . In type III tumors, surgery is performed on the vena cava or iliac veins [16] [17] [18] [19] [20] .

The present study also shows that the simultaneous involvement of retroperitoneal arteries and veins by type I sarcomas occurred with a lower frequency (29%). Dzsinich et al., [16] reported 7.7% prevalence of both arterial and venous involvement in 13 cases with leiomyosarcomas. Less common, in this study, is isolated arterial involvement by retroperitoneal sarcomas (type II) (14%). Chiche et al., [21] reported five patients with sole arterial growth pattern. Type II sarcomas usually require vascular reconstruction [18] [21] .

The decision of whether to resect blood vessels depends especially on preoperative radiologic imaging results [10] [11] . angiography or contrast-enhanced MRI can be used as diagnostic procedure [10] [22] . In patients with palpable pulses, routine angiography or MR angiography is not necessary, and venography is usually not required [22] . Intravascular ultrasound scans can be used as a complementary diagnostic tool. The value of positron emission tomography is still unclear [23] .

Arterial replacement was usually accomplished by implanting a synthetic vascular prosthesis in anatomic position. Visceral arteries must be resected for tumors extending into the mesenteric root. In such cases, reconstruction can necessitate primary anastomosis, reinsertion, or bridging by small-diameter grafts (e.g., great saphenous vein, synthetic 6-mm ePTFE, or Dacron prostheses). Good long-term patency rates (83%) were observed in this study when preferably synthetic grafts were used for arterial reconstructions. Arterial bypass grafting after sarcoma surgery has been recognized in the literature in few series [21] [24] .

Central venous repair after resection prevents lower limb edema and the clinical sequelae such as pain, swelling, tension, and skin alterations [25] . Expanded PTFE tube prostheses or ePTFE patches are preferred for reconstruction of the inferior and middle portion of the vena cava. Only in case of pre-existing venous thrombosis should caval ligation be done below the renal veins or in the iliac veins. Retroperitoneal venous reconstructions after STS resection have been previously reported [16] - [21] .

The morbidity and reoperation rates in our study were acceptable and thus favor the concept of vascular resection with consecutive repair. As bleeding was the most common complication in some series [9] ; careful hemostasis and cautious intraoperative administration of heparin have been suggested [9] .

According to our institutional surgical policy, 43% of patients received multi-visceral resection to improve quality of margins and subsequently local control [26] [27] .

One of the most common complications in venous reconstruction is graft occlusion, ranging between 7% and 28% [9] [16] [20] [28] . The most used reported graft is PTFE: it is claimed to be more resistant to abdominal viscera compression and consequently less prone to thrombosis [9] [13] [20] [28] . The venous graft is preferable for its theoretical superiority against infection. Whenever a banked homograft of adequate size is available, anatomic restoration of IVC integrity and size matching are more easily achievable than with autologous venous graft (surgical technique).

Bood vessel resection should be considered feasible whenever necessary in RSTS. This is of high importance; especially that completeness of surgical resection is the predominant prognosticator in RSTS [29] .

Some authors reported the prognosis of patients who undergo complete resection of retroperitoneal sarcoma with major vascular resection to be very poor, with no long-term survivors [26] [30] [31] . Indeed this bad outcome may be possibly related to disease extent of the patients reported in those series, as indirectly reflected by the need to perform vascular resection, rather than to the procedure itself [32] .

This study shows that complete resection with clear margins is important for long-term survival in patients with retroperitoneal soft tissue sarcomas. Primary sarcomas or local recurrences, as well as tumors with primary vascular involvement or secondary vascular involvement, require resection of diseased blood vessels necessary to improve the completeness of tumor clearance. The extent of vascular resection and the appropriate vascular repair has to be assessed in the individual patient according to the preoperative imaging, intraoperative findings, and extent of resection, and it should be carefully planned on the basis of the stage of the disease. The proposed classification and treatment algorithm can be used to plan resection and vascular replacement.

The authors declare no conflicts of interest regarding the publication of this paper.

Gad, Z., Gamal, A. and Sallam, K. (2019) Analysis of the Onco-Vascular Approach in Retroperitoneal Sarcoma with Vascular Involvement. Journal of Cancer Therapy, 10, 632-641. https://doi.org/10.4236/jct.2019.108052