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. 2017 Oct;14(4):3909-3916.
doi: 10.3892/ol.2017.6706. Epub 2017 Aug 2.

Leiomyosarcoma of the inferior vena cava: Survival rate following radical resection

Frederico José Ribeiro Teixeira Jr  1   2 , Sergio Dias do Couto Netto  2 , André Luis de Freitas Perina  1 , Fabio C M Torricelli  3 , Luciana Ragazzo Teixeira  4 , Antonio Eduardo Zerati  4 , Fabio de Oliveira Ferreira  1 , Eduardo Hiroshi Akaishi  1 , William Carlos Nahas  3 , Edivaldo Massazo Utiyama  2
Affiliations

Leiomyosarcoma of the inferior vena cava: Survival rate following radical resection

Frederico José Ribeiro Teixeira Jr et al. Oncol Lett. 2017 Oct.

Abstract

Leiomyosarcoma (LMS) of inferior vena cava (IVC) is a rare neoplasm affecting approximately 1/100,000 people. The prognosis is poor and potential curative intent occurs through challenging operations, such as vena cava resection, occasionally multivisceral when required, and vascular reconstruction. There are few retrospective series regarding this retroperitoneal neoplasm, and the aim of the present study was to discuss the experience at the São Paulo Cancer Institute and Clinics Hospital of University of São Paulo Medical School, São Paulo, Brazil. The current study is a retrospective review of 7 patients treated in the two tertiary hospitals between 2005 and 2013. Oncological and operative aspects were discussed, primarily regarding surgical aspects highlighting en bloc resection, vascular reconstruction, and the overall survival and recurrence rates. All the patients were treated with radical intent, 4 of whom underwent multivisceral resection, with the kidney being the most resected organ. The location of the IVC tumor was described using Kulaylat's description and the median tumor size was 10 cm. Vascular reconstruction was necessary in 4 patients. The overall survival rate at 3 and 5 years was 100, and 25%, respectively. The disease-free survival rate at 3 and 5 years was 57 and 20%, respectively. In conclusion, IVC LMS is a rare and severe retroperitoneal neoplasm, with multivisceral resections remaining a surgical challenge. The treatment requires numerous experienced surgeons and the impact of microscopic free margins remains unclear. Vascular reconstruction depends on several aspects regarding primarily the topography of the tumor.

Keywords: inferior vena cava sarcoma; leiomyosarcoma; retroperitoneal sarcoma; retroperitoneal tumor; sarcoma; vena cava tumor.

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Figures

Figure 1.
Figure 1.
The classification of vena cava sarcoma according to Kulaylat et al (12).
Figure 2.
Figure 2.
Abdominal computed tomography scan revealing an inferior vena cava sarcoma infiltrating the right renal vessels, indicated by the arrow.
Figure 3.
Figure 3.
Retroperitoneal tumor dissected in a patient with leiomyosarcoma of the inferior vena cava.
Figure 4.
Figure 4.
Image captured during inferior vena cava reconstruction of a patient with leiomyosarcoma.
Figure 5.
Figure 5.
Computed tomography scan revealing an inferior vena cava sarcoma infiltrating both renal vessels. The arrows highlight the vessel's invasion.
Figure 6.
Figure 6.
Image captured during inferior vena cava reconstruction of a patient with leiomyosarcoma.
Figure 7.
Figure 7.
Kaplan-Meier survival curve for the overall survival rate at 5 years of patients with leiomyosarcoma of the inferior vena cava.
Figure 8.
Figure 8.
Kaplan-Meier survival curve for the disease-free survival rate at 5 years of patients with leiomyosarcoma of the inferior vena cava.
See this image and copyright information in PMC

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