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Primary Leiomyosarcoma of the Inferior Vena Cava : A Case Report of Radical Surgery with Dacron Reconstruction

เนื้องอก Leiomyosarcoma ของเส้นเลือดดำใหญ่ในช่องท้อง : กรณีศึกษาการผ่าตัดเส้นเลือดดำใหญ่ในช่องท้องทดแทนด้วยเส้นเลือดเทียม Dacron

Supatcha Prasertcharoensuk (สุภัชชา ประเสริฐเจริญสุข) 1, Narongchai Wongkonkitsin (ณรงชัย ว่องกลกิจศิลป์) 2, Sakda Waraasawapati (ศักดา วราอัศวปติ) 3, Chawalit Pairojkul (ชวลิต ไพโรจน์กุล) 4, Ake Pugkhem (เอก ปักเข็ม) 5




หลักการและวัตถุประสงค์: ผู้ป่วยหญิงอายุ 33 ปี มีอาการปวดท้องซีกขวาเรื้อรังโดยไม่มีก้อนในช่องท้องหรือขาบวมเลยเป็นเวลา 1 ปี เอกซเรย์คอมพิวเตอร์สงสัยมะเร็งตับ 2.9 ซม. ได้ทำการตรวจคลื่นแม่เหล็กไฟฟ้าเพิ่มเติม พบเนื้องอกที่ผนังของเส้นเลือดดำใหญ่

วิธีการรักษา: แผนการรักษาประกอบด้วยการผ่าตัดเอาเนื้องอกในเส้นเลือดดำใหญ่ในช่องท้องและตับส่วนที่ 1 ออก มีความจำเป็นต้องทดแทนเส้นเลือดดำใหญ่ด้วยเส้นเลือดเทียม Dacron ขนาด 20 มม. และเส้นเลือดดำของไตซีกซ้าย 12 มม.

ผลการศึกษา: ผลจากการใส่เส้นเลือดเทียม Dacron  มีภาวะคงเปิดของเส้นเลือดเทียมดี

สรุป: การใช้เส้นเลือดเทียม Dacron เพื่อทดแทนเส้นเลือดดำใหญ่ในช่องท้องเป็นอีกทางเลือกหนึ่งที่ดี

 

Background and Objective :A 33 year-old woman present with one year right-sided abdominal pain without abdominal mass or leg edema, investigated with CT was suspected hepatocellular carcinoma 2.9 cm but MRI suspect leiomyosarcoma of IVC .

Methods : Treatment plans included radical resection with caudate lobe resection need 20 mm Dacron graft for IVC reconstruction and 12 mm for left renal vein re-implantation.

Results : The treatment resulted good patentcy of Dacron graft

Conclusion : Dacron graft vena caval replacement is considered as a good alternative graft forIVC reconstruction.

Introduction

Primary tumors of the inferior vena cava (IVC) are rare,less than300 cases reported in the literature , that leiomyosarcoma(LMS) representing the vast majority (95%)1,2, usually present in late middle age women with abdominal discomfort due to an abdominal mass3. The mainstay of treatment is surgical resection, if  there is no evidence of metastasis , other treatment outcome is not quite clear4.  We report pararenal vena cava leiomyosarcoma with emphasis on the surgical procedure and reconstruction of caval continuity with Dacron graft.

Case report

A 33 year-old woman presented with 1year right-sided abdominal pain without abdominal mass or leg edema, investigated with Computed Tomography revealed a 2.9 cm rim enhancing hypodense mass with rapid wash out in segment 1 of liver compress on IVC and portal vein , as shown in Fig 1. Another investigation using Magnetic Resonance Imaging (MRI) revealed  3.1 cm lobulated intraluminal filling defect attach to medial wall of upper IVC compromised of lumen, as shown in Fig 2, Leiomyosarcoma of IVC is diagnosis.

 

Fig 1 CT shows 2.7 x 2.9 cm rim enhancing hypodense mass with rapid wash out in segment 1 of the liver with compression on IVC and portal vein

 

Fig 2 MRI shows 2.8 x 2.8 x 3.1 cm, lobulated intraluminal filling defect attach to medical wall of upper IVC causing compromised of IVC

 

The patient was scheduled for surgical resection, after mobilized left and right lobe liver , tumor was identified as Fig 3. Radical resection was performed , IVC reconstruction was needed with 20 mm Dacron, left renal vein re-implanted with 12 mm Dacron as shown in Fig 4.

 

Fig 3 Intra-operative finding shows tumor at IVC after mobilized left and right lobe liver, and ligate short hepatic vein

 

Fig 4 Intra-operative finding shows IVC was reconstructed with 20 mm Dactron, left renal vein was re-implanted with 12 mm Dacron

 

 

A well-circumscribed rubbery mass, measuring 4x3x3 cm, showing homogeneous cut surfaces in Fig 5 withmicroscopic finding show low grade leiomyosarcoma in Fig 6.

 

Fig 5 Gross pathology shows the tumor excision specimen was a well-circumscribed rubbery mass, measuring 4 x 3 x 3 cm, and homogenous cut surfaces.

 

Fig 6 Micrograph of low grade leiomyosarcoma, showed indulated extraluminal expansile mass at vascular wall (A), comprised of cellular spindle proliferation in fascicles (B) and area of pleomorphism with few mitosis (C). Tumor cells expressed membranous and cytoplasmic staining for smooth muscle actin antibody (D).

 

After an eventful recovery, the patient was discharged . Recurrent tumor at surgical bed after 18 months was followed up with good patentcy of Dacron graft as in Fig  7 , afterward the patient passed away 30 months after surgery.

 

Fig 7 CT shows recurrent of tumor at surgical bed in 18 month follow up with patent of Dacron Interposition graft

 

Discussion

Leiomyosarcoma is the most common primary malignancy of blood vessels, arise anywhere in the vascular system, most recorded cases are in the IVC5. Perl first described it in 1871 and the first surgical resection was in 1928 by Mechior6,7. Tumor growth within the IVC is intra-luminal in 5%, extra-luminal in 62%, and both intra- and extra-luminal in 33%8. Histopathological features are identical to those found in LMS of other sites. There is no standardized histological grading system for IVC tumors , some authors have recommended using the criteria for retroperitoneal LMSs, which is based on mitotic count5.

The presentation of LMS depends on its location along the IVC, which can be divided into three segments. Segment I (lower) is below the renal veins, involved in 36% of cases, presenting symptoms 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 , 44% of cases, symptoms may include abdominal pain, nephrotic syndrome, and renal hypertension. Segment III (upper) is from the right atrium to the hepatic veins, 20% of cases, present with weight loss, nausea, Budd-Chiari syndrome, and cardiac arrhythmias9-12.  Tumor of this patient was located in segment II which make operation more difficult and can cause many symptoms , but the patient got only abdominal pain and operation was performed without any complication.

 

The best modality to get tissue diagnosis is endoscopic biopsy under ultrasound guidance13,  the principal aim of preoperative imaging assessment is to determine the local and distant extent of the disease using a combination of CT or MRI scanning, bone, and positron-emission tomography (PET) scans14-16.  Ascending or retrograde cavography may be used to evaluate involvement of major braches17.

Aggressive radical resection remains the current treatment of choice, palliative resections may temporarily improve symptoms but do not offer long-term survival18,19. Recurrence due to incomplete removal of tumor is common4.The surgical management of partial resections of the IVC is a matter of current debate and includes ligation, primary repair/cavoplasty, or replacement with a graft.    Reconstruction of the IVC is not always required, because gradual occlusion of the IVC allows the development of venous collaterals. However, when pararenal leiomyosarcoma of the IVC is present, reconstruction of the IVC and the renal vein is necessary to prevent transient or permanent renal dysfunction20,21.

Historically, the prognosis of primary IVC leiomyosarcoma has been poor. In those with metastatic disease at presentation, survival is usually 3 - 6 months. In those who have an attempted curative resection, 5- and 10-year survival rates are 30- 53% and 7.4%, respectively3,4,10  with the principle determinant of survival being surgical resectability with tumor-free margins; the post resection recurrence rate is around 50% 22.

Several technical considerations ,  when PTFE is used, some suggest that externally supported PTFE prevents collapse from respiratory compression and from surrounding structures, while others believe that it incorporates poorly and predisposes to entero-prosthetic fistula formation with the duodenum23. Under-sizing of the PTFE has been recommended with the rationale that the resulting increase in blood flow velocity within the graft might reduce thrombotic risk24. Others recommend a larger diameter graft, as PTFE tends to form a thick pseudo-intima that may result in obstruction25.  We used  20 mm Dacron  graft without  external support in IVC reconstruction and 12 mm Dacron graft for left renal re-implantation, after follow up for 5 years there is no compression , thrombosis or entero-prosthetic fistula formation.Compared with previous reports, this patient had comparable of survival and patency even different of graft.

 

Conclusion

          In this report, the feasibility and safety of Dacron graft vena caval replacement is documented. Therefore , it can be considered a good alternative graft to reconstruction of IVC in the case of a rare primary leiomyosarcoma.

 

References

1.       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.

2.       Tameo MN, Calligaro KD, Antin L, Dougherty MJ. et al. Primary leiomyosarcoma of the inferior vena cava: Reports of infrarenal and suprahepaticcaval involvement. J Vasc Surg 2010; 51: 221-4.

3.       Hollenbeck ST, Grobmyer SR, Kent KC, Brennan MF. Surgical treatment and outcomes of patients with primary inferior vena cava leiomyosarcoma. J Am Col Surg 2003; 197: 575-9.

4.       Dew J, Hansen K, Hammon J, McCoy T, Levine EA, Shen P. Leiomyosarcoma of the inferior vena cava: surgical management and clinical results. Am Surg 2005; 71: 497-501

5.       Hilliard NJ, Heslin MJ, Castro CY. Leiomyosarcoma of the inferior vena cava: three case reports and review of the literature. Ann Diagn  Pathol 2005; 9: 259-66.

6.       Perl L. Ein fall von sarkom der vena cava inferior. Virchows Arch F Path Anat 1871; 53: 378.

7.       Melchior E. Sarkom der vena cava inferior. Dtsch Eitschrift Chirurgie 1928; 213: 135-40.

8.       Ceyhan M, Danaci M, Elmali M, Ozmen. Leiomyosarcoma of the inferior vena cava. Diagn Interv Radiol 2007; 13: 140-3.

9.       Kulaylat MN, Karakousis CP, Doerr RJ, Karamanoukian HL, Obrien J, Peer R. Leiomyosarcoma of the inferior vena cava. J Surg Oncol 1997; 65: 205-17.

10.    Mingoli A, Cavallaro A, Sapienza P, DiMarzo L, Feldhaus RJ, Cavallari N. International registry of inferior vena cava leiomyosarcoma: analysis of a world series on 218 patients. Anticancer Res 1996; 16: 3201-5.

11.    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 Onc 2008; 34: 831-5.

12.    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.

13.    Hemant D, Krantikumar R, Amita J, Chawla A, Ranjeet N. Primary leiomyosarcoma of inferior vena cava, a rare entity: imaging features. Australas Radiol 2001; 45: 448-51.

14.    Blum U, Wildanger G, Windfuhr M, Laubenberger J, Freudenberg N, Munzar T. Preoperative CT and MR imaging of inferior vena cava leiomyosarcoma. Eur J Radiol 1995;20:23-7.

15.    Huang J, Liu Q, Lu JP, Wang F, Wang L, Jin AG. Primary intraluminal leiomyosarcoma of the inferior vena cava: value of MRI with contrastenhanced MR venography in diagnosis and treatment. Abdom Imaging 2011; 36: 337-41.

16.    Ganeshalingam S, Rajeswaran G, Jones RL, Thway K, Moskovic E. Leiomyosarcomas of the inferior vena cava: diagnostic features on cross-sectional imaging. Clin Radiol 2011; 66: 50-6.

17.    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.

18.    Kyriazi MA, Stafyla VK, Chatzinikolaou I, Koureas A , Chatziioannou A , Kondi-Paphiti A,  et al. Surgical challenges in the treatment of leiomyosarcoma of the inferior vena cava: analysis of two cases and brief review of the literature. Ann Vasc Surg  2010; 24: 826. e13-7.

19.    Wang Q , Jing J , Wang C , Lian G , Jin MS , Cao X . Leiomyosarcoma of the inferior vena cava level II involvement: curative resection and reconstruction of renal veins. World J Surgl Oncol 2012; 10: 120.

20.    Huguet C, Ferry M, Gavelli A: Resection of the suprarenal inferior vena cava: The role of prosthetic replacement. Arch Surg 1995 ; 130: 793-7.

21.    Alexander  A , Rehders  A , Raftel  A , Poremba  C , Knoefel WT , Eisenberger  CF. Leiomyosarcoma of the inferior vena cava: Radical surgery and vascular reconstruction. World J Surg Oncol 2009; 7: 56.

22.    Ito H, Hornick JL, Bertagnolli MM, George S , Morgan JA, Baldini EH, et al.  Leiomyosarcoma of the inferior vena cava: survival after aggressive management. Ann Surg Oncol 2007; 14: 3534-41.

23.    Illuminati G, Calio’ FG, D’Urso A, Giacobbi D,  PapaspyropoulosV,  Ceccanei G. Prosthetic replacement of the infrahepatic inferior vena cava for leiomyosarcoma. Arch Surg 2006; 141: 919-24.

24.    Sarkar R, Eilber FR, Gelabert HA, Quinones-Baldrich WJ. Prosthetic replacement of the inferior vena cava for malignancy. J VascSurg 1998; 28: 75-83.

25.    Glovicski P, Hollier LH, Dewanjee MK, Trastek VF, Hoffman EA, Kay MP. Experimental replacement of the inferior vena cava: factors affecting patency. Surgery 1984; 95: 657-66.

 

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