A systematic review of the current management approaches in leiomyosarcoma of inferior vena cava—Results from analysis of 118 cases

Abstract

Introduction Primary intravenous leiomyosarcomas are rare vascular tumors with aggressive disease biology. The diagnosis and management have been challenging as little data exist from large databases. Methods A literature search was done to identify all cases of primary leiomyosarcomas in the last five years. Clinicopathological features and management strategies were evaluated. Results The median age was 53 years, predominantly females (2.5:1), presenting as metastases in up to 12.1% cases. Most tumors were locally advanced with a median size of 10cm. Inferior vena cava involvement from renal veins to infrahepatic veins remains the most frequent site (57.1%cases) while nearly half (52.8%) proceeded for surgery without histological proof. Most patients could undergo upfront resection (88.0%) with few patients receiving neoadjuvant chemotherapy (4.3%) or neoadjuvant radiotherapy (2.2%). Significant multivisceral resections included right nephrectomy (41.3%), liver resection (25.7%) and left nephrectomy (2.2%). Most patients (91.8%) needed an inferior vena cava graft placement with remarkable microscopically negative margins (85.5% cases). Doxorubicin and ifosfamide were the most frequently used combination chemotherapy regimens in both pre and postoperative settings with partial responses. The median overall and disease free survival among operated patients was 60 months and 28 months respectively. In multivariate analysis large tumor, extensive inferior vena cava involvement, and need for adjuvant chemotherapy appeared significant predictors for overall survival. Conclusions Aggressive upfront surgical resection with clear margin remains the key for long-term survival. Doxorubicin-based regimens were preferred as neoadjuvant chemotherapy while adjuvant treatment with chemotherapy, radiotherapy, or both may be considered in high-risk patients.

When Vessels and Sarcomas Combine: A Review of the Inferior Vena Cava Leiomyosarcoma

“…Regarding chemotherapy, the most used drugs were doxorubicin and ifosfamide. In this study, 88% of the patients were eligible for upfront resection, but it should be stated that 52.8% went to surgery without histological confirmation of disease, a percentage that nowadays would be almost impossible and counter-productive [81].…”

Inferior vena cava resection for a leiomyosarcoma: A case report

“…They account for around 0,7 % of cases of retroperitoneal leiomyosarcomas [9] . Approximately 500 cases of leiomyosarcomas have been described in the literature [10] , [11] . Generally, the diagnosis is made late, as it is a retroperitoneal tumor.…”

Case Report: Multidisciplinary management of primary inferior vena cava leiomyosarcoma: a comprehensive case study

“…Postoperative adjuvant chemotherapy and radiotherapy are frequently utilized as auxiliary treatments for IVC leiomyosarcoma (5). Doxorubicin and isocyclophosphamide, serving as cornerstone chemotherapy agents, are employed extensively (5).…”

“…Postoperative adjuvant chemotherapy and radiotherapy are frequently utilized as auxiliary treatments for IVC leiomyosarcoma (5). Doxorubicin and isocyclophosphamide, serving as cornerstone chemotherapy agents, are employed extensively (5). However, in comparison to the chemotherapy response observed in synovial sarcoma and liposarcoma, inferior response rates of only 10% to 25% are witnessed in IVC leiomyosarcoma (20).…”

“…The prognosis for IVC leiomyosarcomas is discouraging, as study show that only around 49.4% survive for 5 years and a meager 29.5% for 10 years (3). The main treatment option is complete surgical removal, and additional therapy after the surgery may offer some improvement in the prognosis (4,5). Nevertheless, the lack of agreement on the most suitable surgical approach and postoperative adjuvant therapy decisions is attributable to limited researches, predominantly consisting of individual case reports and small patient series.…”