J Korean Surg Soc.  2012 Feb;82(2):101-109. 10.4174/jkss.2012.82.2.101.

Multidisciplinary treatment and long-term outcomes in six patients with leiomyosarcoma of the inferior vena cava

• Kim JT
• Kwon T
• Cho Y
• Shin S
• Lee S
• Moon D

• KMID: 2212208
• DOI: http://doi.org/10.4174/jkss.2012.82.2.101

Abstract

PURPOSE
Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare primary soft tissue sarcoma. Few reports have detailed the tumor features, treatment strategies, and long-term outcomes in IVC LMS patients. The present report describes the treatment and long-term outcomes of six patients with IVC LMS.
METHODS
We reviewed six consecutive cases of IVC LMS treated at the University of Ulsan College of Medicine, Asan Medical Center from August 1998 to June 2010.
RESULTS
The patients comprised five females and one male, and had a median age of 44 years (range, 25 to 64 years). All tumors were suprarenal. The tumors were located between the hepatic and renal veins (i.e., middle segment; n = 5 [83%]), or above the hepatic veins (i.e., upper segment; n = 1 [17%]). Prosthetic IVC replacement using polytetrafluoroethylene grafts was performed in five patients, and the remaining patient underwent only tumor resection and IVC ligation. There were no intraoperative or postoperative deaths. The mean tumor size was 9.3 cm (range, 5 to 20 cm), and five of the six tumors were high grade. The mean follow-up period was 80 months (range, 6 to 118 months). The median survival period was 94 months. Recurrence occurred in all patients. Distant recurrence resulted in three patients undergoing lung resection and three patients undergoing thigh muscle resection.
CONCLUSION
IVC LMS is a rare but serious disease. Although surgical resection combined with chemoradiotherapy was not completely curative, it resulted in long-term patient survival, even in patients with advanced tumors.

Keyword

Leiomyosacrcoma; Adjuvant therapy; Inferior vena cava

MeSH Terms

Chemoradiotherapy
Female
Follow-Up Studies
Hepatic Veins
Humans
Leiomyosarcoma
Ligation
Lung
Male
Muscles
Polytetrafluoroethylene
Recurrence
Renal Veins
Sarcoma
Thigh
Transplants
Vena Cava, Inferior
Polytetrafluoroethylene

Figure

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  Fig. 1 Preoperative leiomyosarcoma (LMS) evaluation. LMS were preoperatively assessed using venography computed tomography scan, and venographic biopsy.

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  Fig. 2 A large leiomyosarcoma in the upper segment with middle hepatic vein involvement. The patient underwent ex situ excision with the whole liver and auto-transplantation after venoplasty.

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  Fig. 3 Overall survival rate. The 3- and 5-year survival rates were 80% and 60%, respectively.

Reference

1. Mingoli A, Cavallaro A, Sapienza P, Di Marzo L, Feldhaus RJ, Cavallari N. International registry of inferior vena cava leiomyosarcoma: analysis of a world series on 218 patients. Anticancer Res. 1996. 16(5B):3201–3205.

2. Cho SW, Marsh JW, Geller DA, Holtzman M, Zeh H 3rd, Bartlett DL, et al. Surgical management of leiomyosarcoma of the inferior vena cava. J Gastrointest Surg. 2008. 12:2141–2148.

3. Kulaylat MN, Karakousis CP, Doerr RJ, Karamanoukian HL, O'Brien J, Peer R. Leiomyosarcoma of the inferior vena cava: a clinicopathologic review and report of three cases. J Surg Oncol. 1997. 65:205–217.

4. Hines 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. 85:1077–1083.

5. 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.

6. 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. 14:3534–3541.

7. Kieffer E, Alaoui M, Piette JC, Cacoub P, Chiche L. Leiomyosarcoma of the inferior vena cava: experience in 22 cases. Ann Surg. 2006. 244:289–295.

8. Kraybill WG, Callery MP, Heiken JP, Flye MW. Radical resection of tumors of the inferior vena cava with vascular reconstruction and kidney autotransplantation. Surgery. 1997. 121:31–36.

9. 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. 197:575–579.

10. Hardwigsen J, Balandraud P, Ananian P, Saïsse J, Le Treut YP. Leiomyosarcoma of the retrohepatic portion of the inferior vena cava: clinical presentation and surgical management in five patients. J Am Coll Surg. 2005. 200:57–63.

11. Varela-Duran J, Oliva H, Rosai J. Vascular leiomyosarcoma: the malignant counterpart of vascular leiomyoma. Cancer. 1979. 44:1684–1691.

12. Dzsinich C, Gloviczki P, van Heerden JA, Nagorney DM, Pairolero PC, Johnson CM, et al. Primary venous leiomyosarcoma: a rare but lethal disease. J Vasc Surg. 1992. 15:595–603.

13. Beiles CB, Jones RM, Fell G. Recurrent leiomyosarcoma of the inferior vena cava. Aust N Z J Surg. 1997. 67:67–68.

14. Demers ML, Curley SA, Romsdahl MM. Inferior vena cava leiomyosarcoma. J Surg Oncol. 1992. 51:89–92.

15. Kasano Y, Tanimura H, Kumada K, Taniguchi K, Takifuji K, Ohnishi H, et al. Resectable leiomyosarcoma of inferior vena cava extended into the right atrium with the use of cardiopulmonary bypass and graft replacement. Surgery. 1995. 117:473–475.

16. Yuzer Y, Zeytunlu M, Makay O, Sozbilen M, Yuce G. Leiomyosarcoma of the inferior vena cava: report of a case. Surg Today. 2004. 34:370–373.

17. Pervaiz N, Colterjohn N, Farrokhyar F, Tozer R, Figueredo A, Ghert M. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer. 2008. 113:573–581.

18. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Sarcoma Meta-analysis Collaboration. Lancet. 1997. 350:1647–1654.

19. 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. 18:355–359.