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

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.

Simultaneous Surgery for Inferior Vena Cava Leiomyosarcoma with Multiple Hepatic Metastases: A Justified Challenge

“…Liver metastasis accounted for ∼21.5% of metastases, thus, representing a common site of metastases [3,5]. Although there have been some reported cases of concomitant hepatectomy and IVC resection and reconstruction [3,8,9], we could not find previously reported cases of simultaneous surgery for multiple liver metastases from IVC leiomyosarcoma. This would thus be the first reported case describing a simultaneous, aggressive and extended surgical approach in a patient with IVC leiomyosarcoma with multiple liver metastases.…”

“…The 5-year survival rate is reported to range between 31% to 68% following complete macroscopic resection [2–7]. In cases with macroscopic positive margins (R2) resection, the survival is poor with ∼0% in 5 years [8].…”

“…Previous publications have discussed the safety and advantages of radical and liberal excision for IVC leiomyosarcomas [3,5,8,12]. The surgical strategies can be tailored based on the extension of the tumor along the IVC.…”

Leiomyosarcoma of the inferior vena cava: a case report

“…Beyond achieving clear surgical margins, the data regarding the utilization of chemotherapy, radiation therapy, or both in combination with surgical resection is limited, with the best treatment strategy being unclear [ 8 ]. In our patient, we elected to forgo utilizing combination therapy, as she had clear surgical margins, and the benefit is not well supported in the literature.…”

Inferior vena cava leiomyosarcoma in an octogenerian

“…The effect of overall survival and disease free survival in patients with R0 or R1 resection is equivocal but it is clear that R2 resections have uniformly dismal prognosis with 0% survival in 5 years 14. Therefore, in pursuit of achieving negative margins, extensive multivisceral and vascular resections with reconstructions are reported to be performed 15. Due to the need for complex multivisceral resections, IVC LM management needs diverse surgical skill set including urology, vascular, general oncology and hepatobiliary surgery.…”