Purpose: Integration of numerous prognostic variables not included in the conventional staging of retroperitoneal soft tissue sarcomas (RPS) is essential in providing effective treatment. The purpose of this study was to build a specific nomogram for predicting postoperative overall survival (OS) and disease-free survival (DFS) in patients with primary RPS.Patients and Methods: Data registered in three institutional prospective sarcoma databases were used. We included patients with primary localized RPS resected between 1999 and 2009. Univariate (Kaplan and Meier plots) and multivariate (Cox model) analyses were carried out. The a priori chosen prognostic covariates were age, tumor size, grade, histologic subtype, multifocality, quality of surgery, and radiation therapy. External validation was performed by applying the nomograms to the patients of an external cohort. The model's discriminative ability was estimated by means of the bootstrap-corrected Harrell C statistic.Results: In all, 523 patients were identified at the three institutions (developing set). At a median follow-up of 45 months (interquartile range, 22 to 72 months), 171 deaths were recorded. Five- and 7-year OS rates were 56.8% (95% CI, 51.4% to 62.6%) and 46.7% (95% CI, 39.9% to 54.6%. Two hundred twenty-one patients had disease recurrence. Five- and 7-year DFS rates were 39.4% (95% CI, 34.5% to 45.0%) and 35.7% (95% CI, 30.3% to 42.1%). The validation set consisted of 135 patients who were identified at the fourth institution for external validation. The bootstrap-corrected Harrell C statistics for OS and DFS were 0.74 and 0.71 in the developing set and 0.68 and 0.69 in the validating set.Conclusion: These nomograms accurately predict OS and DFS. They should be used for patient counseling in clinical practice and stratification in clinical trials.

To improve understanding of what is adequate in local treatment of extremity soft tissue sarcomas (ESTS), to maximize the ratio between local control, limb preservation and prognosis. Nine hundred ninety-seven consecutive patients affected by primary ESTS were reviewed. Size, depth, histotype and grade of the tumor, margin status (R0, R1, R2) of surgical resection, and adjuvant treatments were analyzed. Univariable and multivariable analysis were carried out. For the subgroup of R1 resection the presence/absence of the tumor at the inked surface and the presence/absence of an anatomic barrier were also considered. Five- and 10-year mortality estimates (95% confidence interval) were 0.29 (0.20–0.38) and 0.38 (0.28–0.49) in R1 cases, and 0.16 (0.13–0.19) and 0.19 (0.16–0.23) in R0 cases (P = 0.0003). Size, grade, depth, and histologic subtype were also significant predictor of mortality. Significant determinants for local relapse were surgical margins, radiation therapy, and histologic subtype. In the subset of R1 resections trends towards a better local control for R1 negative cases and histology other than myxofibrosarcoma were identified. Significant determinants for distant metastases were size, grade and histologic subtype of the tumor but not surgical margins. Quality of surgical margins independently predicted local control and survival. The effect on survival was directly mediated by local recurrence to proximal sites invading the abdomen/thorax, and this may indeed be the main way by which quality of surgery directly impacts the final prognosis of ESTS patients.

The purpose of the study was to retrospectively reassess in our institutional series at a longer follow-up the value of a systematic attempt to carry out wide resections in retroperitoneal soft tissue sarcoma. Three hundred and thirty-one consecutive patients surgically treated were analyzed. Since a shift toward a systematic more extended surgical approach took place starting from 2002, patients were divided in two groups according to the time of surgery. Overall survival (OS), crude cumulative incidence of local recurrence (LR) and distant metastases (DMs) were estimated. Cox model multivariate analysis was carried out. Five-year OS of patients operated in the recent period was 66%, compared with 48% for those operated in the previous period. This was associated with less LR (28% versus 49%), while the number of DMs was higher in the recent group (25% versus 12%). Beside the treatment period, the only independent determinant for survival was histological grade. The adoption of a policy of more liberal visceral en bloc resections was associated with a higher local control and OS. This benefit was evident in patients with grade I–II tumors, while DMs were a limiting factor in high-grade ones. New therapies are needed to control systemic disease as local surgery may improve local control.