Abstract
Background/Aim: Retroperitoneal sarcomas represent very aggressive malignancies with high capacity of invading the surrounding vital structures. Case Report: We present the case of a 46-year-old patient who had been initially diagnosed with a large retroperitoneal mass 18 months ago. At that moment the mass was resected en bloc with the inferior cava vein, which was reconstructed using a cadaveric graft, the histopathological studies demonstrating the presence of a leiomyosarcoma. One year later she was diagnosed with recurrent disease invading the abdominal aorta and a liver metastasis. This time the recurrence was resected en bloc with the abdominal aorta, which was reconstructed by placing a cadaveric graft; atypical liver resection was also performed. The postoperative course was uneventful. Conclusion: Extended vascular resections and cadaveric graft reconstructions might be needed in order to achieve a good local control of the disease in patients with retroperitoneal sarcomas.
Due to the fact that they remain asymptomatic for a long period of time, retroperitoneal tumors are frequently diagnosed in advanced stages of the disease when local invasion of vital structures has already developed (1, 2). In such cases, multiple resections might be needed in order to provide complete excision as well as negative resection margins, a sine-qua-non condition for long-term survival (3-6). Although it has been considered for a long period of time that local invasion of vital structures such as abdominal aorta or inferior cava vein should be considered as formal contra-indication for resection, recent improvement in vascular surgery techniques, as well as the development of various types of grafts ranging from polytetrafluoroethylene grafts to cadaveric ones, allowed surgeons to perform such resections and to safely re-establish the continuity of the major vascular structures by using these types of prosthesis (6-10). However, data reported so far remain scarce due to the extreme complexity of these procedures even in cases in which a single reconstruction (abdominal aorta or cava vein) is needed. The aim of the current paper was to report the case of a female patient who initially benefitted from cava vein resection and reconstruction, followed, one year later, by aortic resection and reconstruction (9-11).
Case Report
The 46-year-old patient with no significant medical history was initially investigated for diffuse abdominal and lumbar pain in association with discrete edema at the level of the both inferior limbs.
The computed tomography, performed at that moment, demonstrated the presence of a large retroperitoneal mass invading the inferior cava vein; the endoscopic ultrasound confirmed the presence of this mass as well as the possibility to biopsy the mass; the histopathological studies raised the suspicion of a retroperitoneal leiomyosarcoma. In the meantime, the imagistic studies confirmed the absence of distant metastases (including pulmonary or liver metastases) so the patient was submitted to surgery, and at that moment, the tumor was resected en bloc with the inferior cava vein. The length of the resected inferior cava vein was 5 cm, and the venous continuity was re-established by using a cadaveric graft. During the early postoperative period, heparin sodium treatment was initiated, the patient being discharged in the seventh postoperative day. However, low molecular weight heparin treatment was administrated for the next three months. The histopathological and immunohistochemical studies confirmed the presence of a moderately differentiated retroperitoneal leiomyosarcoma with negative resection margins. Postoperatively, the patient was submitted to adjuvant chemotherapy consisting of six cycles of doxorubicin and ifosfamide. However, at one-year follow-up, computed tomography was performed and demonstrated the presence of a retroperitoneal recurrent tumor invading the abdominal aorta as well as the caval graft, which was thrombosed. The venous flow was re-established through a rich network of collateral vessels. In the meantime, a liver metastasis located at the level of the fourth segment was also encountered. After discussing the case in a multidisciplinary team, the second line chemotherapy consisting of trabectedin was initialized; after four cycles of trabectedin a computed tomography was performed, which demonstrated the partial regression of the retroperitoneal recurrence as well as partial regression of the liver metastasis. In this context, the patient was considered to have a partial response to treatment [according to Response Evaluation Criteria in Solid Tumors - RECIST criteria (12)] so she was resubmitted to surgery. This time, the tumor was resected en bloc with the invaded abdominal aorta, invaded cava vein, right nephrectomy and atypical hepatectomy. Caval reconstruction was no longer needed due to the presence of patent collateral circulation, while the abdominal aorta was reconstructed by placing a cadaveric graft (Figures 1 and 2). The postoperative course was uneventful, the Doppler ultrasounds performed at 24 and 48 h, as well as the one performed in the seventh postoperative day, demonstrated the presence of normal flow at the level of the reconstructed segment. The histopathological studies confirmed the presence of a moderately differentiated leiomyosarcoma. Postoperatively chronic antiplatelet therapy was administrated.
Discussion
Complete resection of the retroperitoneal tumors en bloc with the adherent or invaded surrounding viscera represents the standard of care in treating such lesions. This approach is nowadays endorsed in guidelines and consensus articles published on this theme (13-15). As for the necessity of resection of one or both major abdominal vessels during this type of surgery, it has been demonstrated that it is needed in up to 15% of cases diagnosed with retroperitoneal tumors (16-18). In cases presenting inferior cava vein invasion, two different therapeutic strategies have been proposed so far with similar results: resection followed by reconstruction or ligation only. The latter method seems to be feasible if adequate collateral venous circulation had already developed due to the presence of chronic venous obstruction. An interesting study, which compared the outcomes of the two methods, has just been published in 2019 by a study group conducted by Ferraris (18); the study included 67 patients, 24 cases being submitted to iliac vein resection, 39 cases to inferior cava vein resection and 4 cases to inferior cava vein and iliac vein resection, the most commonly encountered histopathological subtype being represented by retroperitoneal sarcomas. Among the 43 cases who necessitated inferior cava vein resection, there were 38 cases who necessitated circumferential venous resection; among these cases, 22 cases were submitted to reconstruction by using a banked venous homograft, 10 cases were submitted to polytetrafluoroethylene prosthesis reconstruction, while the remaining six cases were treated by ligation, only due to the presence of a patent collateral venous network. Interestingly, cases treated with ligation only did not develop any related complications such as limb edema (18).
Major vascular resections as part of debulking surgery for locally advanced or relapsed retroperitoneal sarcoma might predispose to the development of serious complications during the postoperative period especially due to the fact that most often these cases carry a heavy history of chemotherapy and even surgery. Therefore, resection is most often a demanding procedure, which usually involves other surrounding viscera in order to provide negative margins and to offer a chance for cure for these patients, who otherwise would have almost no chance to benefit from a long-term survival (1, 5, 6, 10, 11). Studies conducted so far on this issue have demonstrated that in such cases the per cent of cases who will need the association of other visceral resections in order to maximize the debulking effort might range between 44% and 93% (11, 19-21). The most commonly encountered early postoperative complications after major reconstructions are related to graft thrombosis or infection, while during the long-term follow-up the most fearful and frequent complication is represented by graft narrowing, especially in cases submitted to polytetrafluoroethylene graft reconstruction (22-24).
As for the issue of aortic resection and reconstruction, cases necessitating this therapeutic strategy are less frequently encountered when compared to cases necessitating cava vein resection. In the study conducted by Bertrand et al. on 126 patients submitted to oncovascular surgery for retroperitoneal sarcomas the authors reported the necessity of aortic resection in three cases (25); however, only two cases were finally submitted to aortic resection followed by reconstruction, the third case presenting a massive aortic involvement including the celiac axis and the superior mesenteric artery. As expected, other multiple visceral resections were also needed in order to achieve negative resection margins, the most commonly reported being represented by nephrectomy (in 48.4% of cases) small bowel (in 16.1% of cases), pancreas (in 13% of cases) and duodenum (in 9.7% of patients) (25). Another study, which investigated the feasibility of major vessels resections followed by reconstruction was conducted by Schwarzbach et al. and was published in 2006 (26). Among the 141 patients submitted to surgery for retroperitoneal sarcomas, 36% of them also necessitated arterial resection. However, none of these cases were submitted to cadaveric graft aortic reconstruction, the options of choice in this study being represented by Dacron or polytetrafluoroethylene prosthesis. As for the postoperative outcomes, the authors reported a single case of arterial and vein reconstruction. Moreover, after a median follow-up of 19.3 months the arterial patency rate was 88.9%, while the venous patency rate was 93.8%; as for the long-term survival, the authors demonstrated an overall survival of 21 months for patients with complete resection and 8 months for cases with incomplete resection, demonstrating in this way, once again, the necessity of achieving complete tumoral resection and negative margins (26).
The final aspect after resection and aortic reconstruction using a cadaveric graft.
Concomitant atypical hepatectomy and right nephrectomy were performed. The inferior cava vein was no longer reconstructed due to the presence of patent collateral circulation.
As for the aortic reconstruction using a cadaveric graft, scarce data have been published so far; therefore, in the study conducted by Poultsides et al. in 2015 which included 50 patients, aortic reconstruction was needed in 12 cases and was performed by using a cryopreserved allograft in only three cases, the other modalities of reconstruction being represented by the interposition of Dacron or polytetrafluoroethylene prosthesis (in three and respectively five cases) (27). Although it is less commonly performed due to the low number of available cadaveric grafts, the method is safer and more efficient in terms of postoperative risks of developing postoperative complications such as occlusion or infection. As for the long-term outcomes, it seems that this method is more efficient in order to prevent the development of aneurysmal dilatation, or aortic blowout (28).
As for the indication of re-resection in our case, it was represented by the partial response to chemotherapy according to RECIST criteria. As mentioned in the revised RECIST guideline which was published in 2010, partial response to therapy is represented by a >30% decrease in the sum of the longest diameters of the target lesions when compared to baseline (12). Due to the fact that this criterium was respected by both the retroperitoneal lesion and the liver metastases, the multidisciplinary team decided for re-resection; however, this decision was also influenced by the good clinical status and by the young age of the patient.
An interesting technique for avoiding the risk of developing further ischemic complications in cases in which the collateral circulation network is not patent has been proposed by Nishinari et al. (29). The authors proposed performing a temporary arterial and venous bypass using a Dacron prosthesis as the first step procedure in such cases, followed by resection of the tumor and the involved vascular structures during the second step procedure. The method proved to be efficient especially in cases in which an adequate collateral network is not developed. In our case, the presence of a well-developed such network allowed us to perform per primam resection. Moreover, during the second surgical procedure, inferior cava vein was no longer reconstructed due to the presence of this adequate network (29).
Conclusion
Major vascular resections have been commonly reported as part of debulking surgery for retroperitoneal sarcomas, inferior cava vein being significantly more frequently reported when compared to aortic resection. As for the type of reconstruction, patients presenting an adequate collateral circulation and cava vein invasion might be submitted to cava vein ligation only and no reconstruction. Furthermore, when it comes to the aortic reconstruction, it can be performed by placing a synthetic prosthesis or a cadaveric graft. Although data regarding cadaveric graft reconstruction are rare, it seems that this method is associated with significantly improved short-term and long-term outcomes.
Acknowledgements
This work was supported by the project entitled „Multidisciplinary Consortium for Supporting the Research Skills in Diagnosing, Treating and Identifying Predictive Factors of Malignant Gynecologic Disorders”, project number PN-III-P1-1.2-PCCDI2017-0833.
Footnotes
Authors' Contributions
IB, SP, VB, EC, SA, MV performed the surgical procedure; IB, NB reviewed literature data and prepared the draft of the manuscript; IB reviewed the final version of the manuscript. All Authors read and approved the final version of the manuscript.
Conflicts of Interest
The Authors have no conflicts of interest to declare regarding this study.
- Received December 16, 2019.
- Revision received December 21, 2019.
- Accepted December 30, 2019.
- Copyright© 2020, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved
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