Predictors of Disease-Free and Overall Survival in Retroperitoneal Sarcomas: A Modern 16-Year Multi-Institutional Study from the United States Sarcoma Collaboration (USSC)

Abstract

Background. Retroperitoneal sarcomas (RPS) comprise approximately 15% of all soft-tissue sarcomas and frequently associated with significant morbidity and as little as 30% 5-year survival. Here, we provide a large, contemporary, and multi-institutional experience to determine which tumor, patient, and treatment characteristics are associated with long-term outcomes in RPS. Methods. 571 patients with primary RPS were identified from the United States Sarcoma Collaboration (USSC). RPS patients who underwent resection from January 2000 to April 2016 were included with patient, tumor, and treatment-specific variables investigated as independent predictors of survival. Survival analyses for disease-free and overall survival were conducted using Kaplan–Meier and Cox proportional hazards model methods. Results. The study cohort was 55% female, with a median age of 58.9 years (IQR: 48.6–70.0). The most common tumor histiotypes were liposarcoma (34%) and leiomyosarcoma (28%). Median follow-up was 30.6 months (IQR: 11.2–60.4). Median disease-free survival was 35.3 months (95% CI: 27.6–43.0), with multivariate predictors of poorer disease-free survival including higher grade tumors, nodal-positive disease, and multivisceral resection. Median overall survival was 81.6 months (95% CI: 66.3–96.8). Multivariate predictors of shorter overall survival included higher grade tumors, nodal-positive and multifocal disease, systemic chemotherapy, and grossly positive margins (R2) following resection. Conclusions. The strongest predictors of disease-free and overall survival are tumor-specific characteristics, while surgical factors are less impactful. Nonsurgical therapies are not associated with improved outcomes despite persistent interest and utilization. Complete macroscopic resection (R0/R1) remains a persistent potentially modifiable risk factor associated with improved overall survival in patients with retroperitoneal sarcomas.

Increasing Incidence of Liposarcoma: A Population-Based Study of National Surveillance Databases, 2001–2016

“…Hence, retroperitoneal tumors have fewer treatment options and a poorer prognosis [34]. In agreement with published results [10,11,33], our analysis confirms that patients diagnosed with retroperitoneal tumors indeed have the lowest survival rates ( Table 4).…”

“…Patients with well-differentiated tumors located in areas where excision is no longer possible, such as retroperitoneum, have lower survival (63.9%). Tumors in the retroperitoneum are often larger in size and asymptomatic until they become too big for resection [33]. Hence, retroperitoneal tumors have fewer treatment options and a poorer prognosis [34].…”

A multi‐institutional validation study of prognostic nomograms for retroperitoneal sarcoma

“…Similarly, DSS was determined by age and tumor grade. Previous studies have also suggested that tumor grade and the adequacy of surgical resection margins are key prognostic factors for OS and DFS following resection of retroperitoneal STS 6,21,22 . These findings suggest a more simplified, parsimonious predictive model, incorporating only three significant clinicopathologic features, may potentially offer comparable or better prognostic accuracy and discriminative ability for patients with retroperitoneal STS than either the Sarculator or MSKCC nomograms.…”

Cross‐flow microfiltration for isolation, selective capture and release of liposarcoma extracellular vesicles

“…LPS poses a clinical challenge due to its anatomical location, with studies showing that tumors found in the retroperitoneum are more likely to recur post‐surgical resection compared to the tumors located at the extremities (Azumi et al., 1987; Fabre‐Guillevin et al., 2006). In addition, the challenges with single tissue biopsies (Aguilar‐Mahecha et al., 2017; Dolgin, 2016), tumor heterogeneities (Arkun et al., 1997), and the potential for distant metastases (10%‐50%) (Tseng et al., 2013) underlie poor prognosis (Hasegawa et al., 2000) and poor overall survival rates (Schwartz et al., 2019). Among the sub‐types of LPS, de‐differentiated liposarcoma (DDLPS) poses a special challenge due to the high rates of recurrence and the higher potential for metastatic disease compared to other LPS sub‐types (Bill et al., 2016; Tseng et al., 2013).…”