Table of Contents  
CASE SERIES
Year : 2018  |  Volume : 5  |  Issue : 3  |  Page : 203-207

Leiomyosarcoma of infrarenal inferior vena cava: A single institution experience and review of literature


Department of Vascular & Endovascular Surgery, Sri Ramachandra Medical College, Chennai, Tamil Nadu, India

Date of Web Publication8-Aug-2018

Correspondence Address:
Dr. M K Ayappan
Department of Vascular & Endovascular Surgery, Sri Ramachandra Medical College, Chennai, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_81_17

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  Abstract 


We report three cases of primary leiomyosarcoma (LMS) of inferior vena cava (IVC). Vascular LMSs are rare tumors, arising most frequently from IVC. These tumors have a female predominance. Their diagnosis is often challenging, as patients may present with nonspecific complaints such as dyspnea, malaise, weight loss, abdominal pain, or back pain, preceding the diagnosis by several years. LMS of the IVC most frequently occurs in the middle segment. The final diagnosis can be made by an ultrasound or computed tomography-guided biopsy. Due to limited experience with this disease, optimal management of IVC LMS is unknown. Curative surgical resection remains the current treatment of choice for primary LMS of IVC. Neoadjuvant therapy may be given to downsize the tumor and increase resectability rates. Nonetheless, there is no proven role for adjuvant therapy, and recurrence is common. We, hereby, report three cases of this rare entity with emphasis on management.

Keywords: Inferior vena cava, leiomyosarcoma, PTFE


How to cite this article:
Sharma A, Ayappan M K, 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

How to cite this URL:
Sharma A, Ayappan M K, 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 [serial online] 2018 [cited 2018 Dec 10];5:203-7. Available from: http://www.indjvascsurg.org/text.asp?2018/5/3/203/238740




  Introduction Top


Vascular leiomyosarcoma are rare malignant tumours of smooth muscle cells of media. It is now known that these tumours are most common malignant tumour of IVC. In this case series, we report reconstruction of ivc without immunosuppression.


  Case Reports Top


Case 1

Case 1 is a 60-year-old female with right-sided abdominal pain for 1 year, with no other known comorbidities. Physical examination revealed tenderness in the right upper quadrant and mild edema of lower extremities. Computed tomography (CT) revealed a heterogeneously enhancing mass with questionable involvement of inferior vena cava (IVC) and renal vessels [Figure 1]. The patient was diagnosed with a retroperitoneal sarcoma by a general surgeon who scheduled surgical excision. A midline incision was performed, and the vascular surgery service was consulted intraoperatively when it became evident that the tumor involved the IVC and was adherent to duodenum and right ureter and cava reconstruction would be mandatory for curative resection [Figure 2].
Figure 1: Heterogeneously enhancing mass with suspected involvement of inferior vena cava in first case

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Figure 2: Inferior vena cava tumor found adherent to duodenum and ureter in the first case

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The tumour involved infrarenal IVC, which looped and controlled and after which intravenous heparin was administered. There was enough normal IVC below renal veins for proximal control, which was taken just below their origins. Distal control was taken just above the iliac bifurcation. The tumor was resected in en bloc with affected IVC. The tumor was found adherent to ureter. The repair was performed using 18 mm Dacron graft in end-to-end fashion. Patient underwent right ureter Double J stenting intraoperatively [Figure 3]. Specimen was sent for pathological examination [Figure 4] which revealed features suggestive of leiomyosarcoma (LMS) with intraluminal growth and negative margins positive for smooth muscle actin (SMA) and S-100. Is SMA which are tumor markers found positive in soft-tissue sarcomas [Figure 5]. The patient was placed on low-dose heparin and warfarin therapy. Adjuvant therapy was not administered and the patient was discharged to go home on postoperative day 5 and was doing well 16 months postoperatively. Follow-up duplex ultrasound has shown chronic laminar thrombus in the posterior wall of the graft causing mild (<30%) stenosis. The patient remains on warfarin therapy to help maintain graft patency and is followed up every 6 months with serial duplex ultrasounds, as well as CT scans to monitor for recurrence.
Figure 3: Repair of inferior vena cava using Dacron graft in end-to-end fashion

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Figure 4: Pathological specimen of inferior vena cava tumor from the first case

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Figure 5: Tumor cells positive for smooth muscle actin

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Case 2

Case 2 is a 61-year-old female with a history of vague abdominal pain in the epigastric and right hypochondrium for last 3 months;. On physical examination, there was no tenderness and palpable mass. Patient underwent CT venography, which showed 8.8 cm × 5.5 cm well-defined heterogeneous mass arising from the infrarenal IVC with preservation of surrounding fat planes [Figure 5] and [Figure 6].
Figure 6: Showing infrarenal inferior vena cava tumor with preservation of fat planes in the second case

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The patient underwent exploratory laparotomy with excision of tumor and primary repair of the IVC was done without reconstruction as patient had well-developed venous collaterals and primary repair looked feasible. The patient did not develop swelling during the postoperative period.

The pathological examination showed high-grade LMS with tumor cells positive for SMA.

The patient was placed on low-dose heparin and warfarin therapy. Patient has been followed for 11 months and without any recurrences.

Case 3

A 35-year-old female was admitted in general surgery ward with complaints of upper abdominal pain for the past 6 months and edema of bilateral lower limbs for 20 days. On physical examination, there was tenderness in the right hypochondrium and epigastrium.

The patient underwent ultrasonography abdomen which showed retroperitoneal mass. On CT abdomen, we observed well-enhancing retroperitoneal mass inferior to pancreas and in close relation to third part of duodenum [Figure 7]. The patient was diagnosed as retroperitoneal sarcoma, and was planned for excision by general surgeon, but on table the tumour was found arising from ivc extending into both common iliac veins, vascular team was called and excision with reconstruction was planned [Figure 8]. The tumor was isolated and intravenous heparin was given.
Figure 7: Well-enhancing retroperitoneal mass in close relation to three parts of duodenum

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Figure 8: Infrarenal inferior vena cava control and clamped

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Proximal control of infrarenal IVC was taken and distally bilateral common iliac control was taken and clamped.

The tumor was resected and sent for pathological examination, and the infrarenal IVC was reconstructed using Dacron 16 mm × 8 mm × 8 mm graft [Figure 9] and [Figure 10]. The pathological margins were negative, and tumor was primary leiomyoma positive S-100 tumor cells.
Figure 9: Infrarenal inferior vena cava and bilateral common iliac vein reconstruction done using 16 mm × 8 mm × 8 mm Dacron graft

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Figure 10: Graft being used for reconstruction of inferior vena cava

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The patient has been put on low-dose oral anticoagulant and is being followed with Doppler for graft surveillance.


  Discussion Top


LMS of the (IVC) is a rare malignant tumor originating from the smooth muscle of the media.[1] Symptoms and resectability depend on the location and extension of the tumor as well as associated thrombosis.

More than half of all vascular LMSs occur at the IVC. Vascular LMS represents 2% of all LMSs and 0.5% of all soft-tissue sarcomas.[2],[3]

IVC LMS is a malignant tumor of mesenchymal origin that develops from smooth muscular fibers of the tunica media.[4] Perl first described it in 1871, and the first surgical resection was in 1928 by Mechior.[5],[6] LMS of the IVC is four times more common among women and is the most commonly diagnosed during the fifth to sixth decades of life.[1]

IVC LMS has a slow rate of growth and can remain asymptomatic for a long time, causing diagnosis to be delayed until advanced stages when prognosis is poor.

The presentation of LMS depends on its location along the IVC, which can be divided into three segments for this purpose. Segment I (lower) is below the renal veins and is involved in 36% of cases.[1],[4] Typical presenting symptoms for IVC LMS in this segment may include lower-extremity edema, deep-venous thrombosis, abdominal pain, and palpable mass. Segment II (middle) is from the hepatic veins to the renal veins and is involved in 44% of cases. Symptoms in this region may include abdominal pain, nephrotic syndrome, and renal hypertension. Segment III (upper) is from the right atrium to the hepatic veins, is involved in 20% of cases, and can present with weight loss, nausea, Budd—Chiari syndrome, and cardiac arrhythmias.[7],[8]

Diagnosis of IVC LMS is often incidental or at autopsy. When symptoms are present, CT or magnetic resonance imaging is useful to determine the extent of tumor involvement. The confirmation of diagnosis is always done by biopsy. Prognosis is better for tumors involving the middle segment of the IVC as compared with the upper segment. Surgical resection with negative margins, as was the case in all of our patients, has been shown to be the only treatment that improves survival.[1],[2],[4] Tumors involving the lower segment are best treated with total excision of the tumor and involved portion of the IVC and either primary IVC closure, patch angioplasty with autologous vein or prosthetic patch, or replacement with interposition polytetrafluoroethylene (PTFE) or Dacron or banked venous homograft.[9] The goal of cancer surgery is to resect for cure, which requires resecting all visible and microscopic tumors. Hence, this principle also applies for tumor invading IVC.

Undersizing of the PTFE or Dacron has been recommended with the rationale that the resulting increase in blood flow velocity within the graft might reduce thrombotic risk.[10] The use of ring PTFE is common in replacement of IVC.[11] The ring reinforcement, in theory, resists respiration compression better and thus prevents graft collapse that may be a factor in promotion of graft thrombosis. Others recommend a larger diameter graft, as PTFE tends to form a thick pseudointima that may result in obstruction.[12] We used (18 and 16 mm) grafts in our patients.

Creation of an arteriovenous fistula (AVF) either between the aorta and IVC or iliac vein, or between the femoral vessels, has been advocated. AVF creation is theorized to improve patency and prevent the need for anticoagulation by elevating blood flow velocity.[13] However, complications such as limb edema and congestive heart failure have been attributed to AVFs. We did not create AVF in our patients.

Finally, long-term postoperative anticoagulation is recommended by some authors to prevent thrombosis, while others report acceptable patency without warfarin therapy.[13],[14] We used intravenous heparin bridging with oral warfarin therapy in our patients.

Following curative resection of the tumor, recurrence rates have been reported to be as high as 57% with a 5-year survival of 50% in a large series by Mingoli et al.[15] Adjuvant treatment with radiation or chemotherapy has not been proven to improve survival and was not used in our cases. Radical tumor resection has resulted in 5- and 10-year survival rates of 49.4% and 29.5%, respectively. Despite advances in treatment, perioperative mortality ranges as high as 15%.[16] The local tumor recurrence has been found as high as 57.3%. Although it was initially reported that high-grade tumors increased the risk of death, it has been found that tumor grade did not predict recurrence or survival. A retrospective analysis conducted at University of California, Los Angeles (UCLA) also found no difference in survival based on age, gender, tumor size, and lymph node status. Treating LMS of the IVC remains a challenge. Over the years, new techniques have resulted in decreased morbidity and mortality associated with operative management, yet local recurrence remains high. The rarity of this disease poses a great obstacle in its investigation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and nitials 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 Top

1.
Mingoli A, Cavallaro A, Sapienza P, Di Marzo L, Feldhaus RJ, Cavallari N, et al. International registry of inferior vena cava leiomyosarcoma: Analysis of a world series on 218 patients. Anticancer Res 1996;16:3201-5.  Back to cited text no. 1
    
2.
Kwon TW, Sung KB, Cho YP, Kim DK, Yang SM, Ro JY, et al. Pararenal leiomyosarcoma of the inferior vena cava. J Korean Med Sci 2003;18:355-9.  Back to cited text no. 2
    
3.
Hollenbeck 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   Back to cited text no. 3
    
4.
èKulaylat MN, Karakousis CP, Doerr RJ, Karamanoukian HL, Obrien J, Peer R. Leiomyosarcoma of the inferior vena cava. J Surg Oncol 1997;65:205-17.  Back to cited text no. 4
    
5.
Perl L. Ein fall von sarkom der vena cava inferior. Virchows Arch F Path Anat 1871;53:378.  Back to cited text no. 5
    
6.
Melchior E. Sarkom der vena cava inferior. Dtsch Eitschrift Chirurgie 1928;213:135-40.  Back to cited text no. 6
    
7.
Spinelli A, Schumacher G, Benckert C, Sauer IM, Schmeding M, Glanemann M, et al. Surgical treatment of a leiomyosarcoma of the inferior vena cava involving the hepatic and renal veins confluences: Technical aspects. Eur J Surg Oncol 2008;34:831-5.  Back to cited text no. 7
    
8.
Guerrero MA, Cross CA, Lin PH, Keane TE, Lumsden AB. Inferior vena cava reconstruction using fresh inferior vena cava allograft following caval resection for leiomyosarcoma: Midterm results. J Vasc Surg 2007;46:140-3.  Back to cited text no. 8
    
9.
Fiore M, Locati P, Mussi C, Guarino A, Piva L, Santinami M, et al. Banked venous homograft replacement of the inferior vena cava for primary leiomyosarcoma. Eur J Surg Oncol 2008;34:720-4.  Back to cited text no. 9
    
10.
Sarkar R, Eilber FR, Gelabert HA, Quinones-Baldrich WJ. Prosthetic replacement of the inferior vena cava for malignancy. J Vasc Surg 1998;28:75-81.  Back to cited text no. 10
    
11.
Huguet C, Ferri M, Gavelli A. Resection of the suprarenal inferior vena cava. The role of prosthetic replacement. Arch Surg 1995;130:793-7.  Back to cited text no. 11
    
12.
Gloviczki 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.  Back to cited text no. 12
    
13.
Kieffer 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.  Back to cited text no. 13
    
14.
Luminati G, Calio' FG, D'Urso A, Giacobbi D, Papaspyropoulos V, Ceccanei G. Prosthetic replacement of the infrahepatic inferior vena cava for leiomyosarcoma. Arch Surg 2006;141:919-24.  Back to cited text no. 14
    
15.
Mingoli 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.  Back to cited text no. 15
    
16.
Karacagil S, Bowald S, Almgren B, Bergqvist D. Influence of twist on vein and PTFE graft hemodynamics: An ex vivo experimental study. Panminerva Med 1997;39:95-9.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]



 

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