Indian Journal of Vascular and Endovascular Surgery

CASE REPORT
Year
: 2019  |  Volume : 6  |  Issue : 3  |  Page : 208--210

Leiomyosarcoma of inferior vena cava – An institutional experience


Harishankar Ramachandran Nair, Shivanesan Pitchai, Tom Thomas Kattoor, Sreekumar Ramachandran 
 Department of CVTS, Division of Vascular Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India

Correspondence Address:
Dr. Sreekumar Ramachandran
Department of CVTS, Division of Vascular Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala
India

Abstract

Primary inferior vena cava (IVC) tumors are rare with the incidence of about 0.05% overall with leiomyosarcoma (LMS) being malignant and the most common. They are late to present and slow growing, seen predominantly in females in their fifth or sixth decades and have a poor prognosis with a 5-year survival rate touching about 35% even with complete surgical resection. We present our experience with two patients diagnosed to have IVC LMS and underwent complete surgical resection of the tumor with reconstruction of IVC using coated polyester graft. In one patient, only infrarenal IVC was reconstructed, whereas in second patient, bilateral renal veins were also reconstructed along with suprarenal IVC. There is no consensus regarding the optimal treatment of this aggressive tumor, but complete excision of the tumor with wide margins with or without IVC reconstruction have been widely accepted as primary modality of treatment. Neoadjuvant or adjuvant chemoradiotherapy have limited role in the management of this rare tumor.



How to cite this article:
Nair HR, Pitchai S, Kattoor TT, Ramachandran S. Leiomyosarcoma of inferior vena cava – An institutional experience.Indian J Vasc Endovasc Surg 2019;6:208-210


How to cite this URL:
Nair HR, Pitchai S, Kattoor TT, Ramachandran S. Leiomyosarcoma of inferior vena cava – An institutional experience. Indian J Vasc Endovasc Surg [serial online] 2019 [cited 2019 Oct 21 ];6:208-210
Available from: http://www.indjvascsurg.org/text.asp?2019/6/3/208/265782


Full Text



 Introduction



Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare and aggressive tumor and arises from the smooth muscle cell layer of the IVC wall. It is late to present and the patient might have vague complaints of abdominal and back pain or fullness of abdomen. It has poor prognosis with high recurrence rate and complete surgical excision is warranted whenever possible.

 Methods



Patient-1

A 38-year-old female, with no known comorbidities, presented with the complaints of worsening right upper quadrant pain radiating to back for 6 months. She was evaluated elsewhere with magnetic resonance imaging which showed retroperitoneal tumor with IVC infiltration and was sent to our center for further management. Computed tomography (CT) angiography [Figure 1]a and [Figure 1]b showed a retroperitoneal tumor arising from the IVC at the level of renal veins extending up to the retrohepatic segment with a size of 6.5 cm × 5 cm. There was no evidence of local invasion or hepatic and pulmonary metastases. She underwent excision of the tumor.{Figure 1}

Intraoperatively, there was a firm-to-hard tumor arising from the IVC [Figure 2]a and [Figure 2]b from the level of renal veins extending till about 2 cm below the confluence of hepatic veins. Liver was mobilized medially by dividing the coronary and triangular ligaments and the tumor delineated completely, separately looping the renal veins which were free of tumor. IVC with the tumor was resected en bloc and reconstruction done with 14-mm coated polyester interposition graft (MAQUET INTERGARD®) [Figure 3]. Proximal clamp on IVC was 2 cm below the hepatic veins and distally just below the joining of renal veins after which tumor and IVC were resected. The tumor had proliferative appearance and was arising from the IVC wall filling the lumen [Figure 4]a and [Figure 4]b. There was no infiltration of adjacent structures. Proximal anastomosis was done first in end-to-end fashion after which right renal vein was mobilized and reimplanted onto the graft. Separate bypass was given to the left renal vein using 8-mm polyester graft which was found to be adequate for the vein size. Renal protection was provided by packing ice over the kidneys. Proximal IVC clamp was released and both renal veins were allowed to drain first after which the clamp was shifted distal to the renal veins. Distal graft anastomosis was performed in an end-to-end fashion with IVC (infrarenal) stump. There was good flow in the graft post declamping.{Figure 2}{Figure 3}{Figure 4}

She was started on therapeutic anticoagulation in the postoperative period and was discharged on the 7th postoperative day. Biopsy report showed vimentin and S-100 positivity with surgical margins free of tumor. She was not given adjuvant therapy after discussion with oncologist. At 6 months follow-up, she was symptom-free with patent graft on oral anticoagulation. There was no evidence of recurrence.

Patient-2

A 58-year-old male presented with lower back pain and abdominal pain for 1 year and was on irregular treatment for back pain with no relief. CT angiography revealed 7 cm × 5 cm tumor arising from the infrarenal IVC with no evidence of metastasis. He underwent complete excision of the tumor with interposition graft repair of IVC with 16-mm coated polyester graft. Biopsy showed high-grade spindle cell neoplasm suggestive of LMS. He was discharged pain-free and hemodynamically stable and was doing well at 1-month postsurgery but was lost to follow-up thereafter.

 Discussion



IVC LMS is a malignant and the most common primary tumor of IVC predominantly seen in females in their fourth and fifth decades.[1],[2] For surgical resection, the affected segment of IVC can be divided into lower or segment I, i.e. infrarenal (affected in about 30%); middle or segment II that extends from the origin of the renal veins to the origin of the hepatic veins,[3],[4] represents the most affected segment (55%); segment III or upper segment extending from hepatic veins to the right atrium, is the least affected (20%).

The tumor typically arises from mesenchymal cells of IVC wall and usually fills the lumen and causes symptoms due to obstruction or compression like in our patients.[3],[5] The deep location of the tumor accounts for the late presentation and mostly silent course. Patients usually have vague upper abdominal and back pain. When the tumor is quite large, a mass might be palpable in the right hypochondrium or flank. Once IVC is thrombosed, presentation might vary from lower limb swelling with or without deep-vein thrombosis, subcutaneous collateral circulation, and thrombophlebitis. Once hepatic veins are compressed, patients might even have Budd–Chiari syndrome.[6]

CT angiogram involving the thorax, abdomen, and pelvis is the most useful investigation as the tumor size and extent with patency of IVC lumen, presence of collateral network, hepatic, and pulmonary metastases (common) can be known.[7] If accessible, image-guided biopsy maybe performed for diagnosis prior to resection. The optimal treatment of LMS of IVC is still a subject of controversy. Nevertheless, in all published cases, complete surgical resection has provided the best disease-free survival period for the patients.[5],[8] Oncologists have tried postoperative radiotherapy and chemotherapy with alkylating agents to improve disease-free survival and prevent recurrence, but the results have not been promising.[6],[9]

Surgical resection may be followed by reconstruction of IVC or just ligation of the IVC depending on the involved segment and the presence of collateralization.[8],[10] Surgical complexity depends on the location of the tumor. Tumors in the infrarenal segment are easier to treat while those in segment III are generally deemed unresectable and carry the highest mortality. Reconstruction of IVC has been carried out using ringed Polytetrafluoroethylene (PTFE) graft or less commonly polyester with varying patency rates.[6],[10] We used polyester graft in our patients because of the ready availability of the same at our center. The patients are treated with therapeutic anticoagulation like we did in our patients, for improved patency in some studies.[11] Creation of arteriovenous fistula between aorta/iliac/femoral arteries to the graft or iliac veins/IVC and IVC banding have been practised in some case series with varying results; although, we did not resort to any of them.[11],[12]

Thrombosis of the graft and infection are the major complications of IVC reconstruction.[9] In spite of best surgical resection with adjuvant chemotherapy and/or radiotherapy, the recurrence rates remain high (up to 55% at 2 years) and 5-year survival rates are still low with some centers reporting from 35% to 50%.[6],[8],[9]

 Conclusion



IVC LMS is rare and aggressive and continues to pose therapeutic challenge to oncologists and vascular surgeons worldwide. Even with wide complete resection and best of supportive care, prognosis remains grim. Surgical reconstruction of IVC also is complex, especially with involvement of suprahepatic IVC segment. However, it is safe to conclude that IVC resection and replacement are feasible and relatively safe with good early results. Regular surveillance is needed in the postoperative period for the early diagnosis of recurrence.

Informed consent

Informed consent has been obtained from the patient for publication of the case report and accompanying images.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Abell MR. Leiomyosarcoma of the inferior vena cava; review of the literature and report of two cases. Am J Clin Pathol 1957;28:272-85.
2Cacoub P, Piette JC, Wechsler B, Ziza JM, Blétry O, Bahnini A, et al. Leiomyosarcoma of the inferior vena cava. Experience with 7 patients and literature review. Medicine (Baltimore) 1991;70:293-306.
3Sharma A, Ayappan MK, Raju R, Mathur K, Pawar P. Leiomyosarcoma of infrarenal inferior vena cava: A single institution experience and review of literature. Indian J Vasc Endovasc Surg 2018;5:203-7.
4Hines OJ, Nelson S, Quinones-Baldrich WJ, Eilber FR. Leiomyosarcoma of the inferior vena cava: Prognosis and comparison with leiomyosarcoma of other anatomic sites. Cancer 1999;85:1077-83.
5Zhang H, Kong Y, Zhang H, He X, Zhang HY, Liu C, et al. Leiomyosarcoma of the inferior vena cava: Case report and treatment of recurrence with repeat surgery. Ann Vasc Surg 2010;24:417.e5-9.
6Kulaylat MN, Karakousis CP, Doerr RJ, Karamanoukian HL, O'Brien J, Peer R, et al. Leiomyosarcoma of the inferior vena cava: A clinicopathologic review and report of three cases. J Surg Oncol 1997;65:205-17.
7Hollenbeck ST, Grobmyer SR, Kent KC, Brennan MF. Surgical treatment and outcomes of patients with primary inferior vena cava leiomyosarcoma. J Am Coll Surg 2003;197:575-9.
8Mingoli A, Sapienza P, Cavallaro A, Di Marzo L, Burchi C, Giannarelli D, et al. The effect of extend of caval resection in the treatment of inferior vena cava leiomyosarcoma. Anticancer Res 1997;17:3877-81.
9Sessa B, Iannicelli E, Caterino S, D'Angelo F, Milione M, Ziparo V, et al. Imaging of leiomyosarcoma of the inferior vena cava: Comparison of 2 cases and review of the literature. Cancer Imaging 2010;10:80-4.
10Gloviczki P, Hollier LH, Dewanjee MK, Trastek VF, Hoffman EA, Kaye MP, et al. Experimental replacement of the inferior vena cava: Factors affecting patency. Surgery 1984;95:657-66.
11Kieffer E, Alaoui M, Piette JC, Cacoub P, Chiche L. Leiomyosarcoma of the inferior vena cava: Experience in 22 cases. Ann Surg 2006;244:289-95.
12Illuminati G, Calio' FG, D'Urso A, Giacobbi D, Papaspyropoulos V, Ceccanei G, et al. Prosthetic replacement of the infrahepatic inferior vena cava for leiomyosarcoma. Arch Surg 2006;141:919-24.