Multidimensional characteristics, prognostic role, and preoperative prediction of peritoneal sarcomatosis in retroperitoneal sarcoma

Abstract

BackgroundPeritoneal sarcomatosis (PS) could occur in patients with retroperitoneal sarcomas (RPS). This study aimed to expand the understanding of PS on its characteristics and prognostic role, and develop a nomogram to predict its occurrence preoperatively.MethodsData of 211 consecutive patients with RPS who underwent surgical treatment between 2011 and 2019 was retrospectively reviewed. First, the clinicopathological characteristics of PS were summarized and analyzed. Second, the disease-specific survival (DSS) and recurrence-free survival (RFS) of patients were analyzed to evaluate the prognostic role of PS. Third, preoperative imaging, nearly the only way to detect PS preoperatively, was combined with other screened risk factors to develop a nomogram. The performance of the nomogram was assessed.ResultsAmong the 211 patients, 49 (23.2%) patients had PS with an incidence of 13.0% in the primary patients and 35.4% in the recurrent patients. The highest incidence of PS occurred in dedifferentiated liposarcoma (25.3%) and undifferentiated pleomorphic sarcoma (25.0%). The diagnostic sensitivity of the preoperative imaging was 71.4% and its specificity was 92.6%. The maximum standardized uptake value (SUVmax) was elevated in patients with PS (P<0.001). IHC staining for liposarcoma revealed that the expression of VEGFR-2 was significantly higher in the PS group than that in the non-PS group (P = 0.008). Survival analysis (n =196) showed significantly worse DSS in the PS group than in non-PS group (median: 16.0 months vs. not reached, P < 0.001). In addition, PS was proven as one of the most significant prognostic predictors of both DSS and RFS by random survival forest algorithm. A nomogram to predict PS status was developed based on preoperative imaging combined with four risk factors including the presentation status (primary vs. recurrent), ascites, SUVmax, and tumor size. The nomogram significantly improved the diagnostic sensitivity compared to preoperative imaging alone (44/49, 89.8% vs. 35/49, 71.4%). The C-statistics of the nomogram was 0.932, and similar C-statistics (0.886) was achieved at internal cross-validation.ConclusionPS is a significant prognostic indicator for RPS, and it occurs more often in recurrent RPS and in RPS with higher malignant tendency. The proposed nomogram is effective to predict PS preoperatively.

CT and MR Imaging of Retroperitoneal Sarcomas: A Practical Guide for the Radiologist

“…Histology has a role in tracing the recurrence pattern: subtypes such as high-grade LMS show a higher risk of developing distant metastasis, rather than local recurrence, after complete resection [1]. Another peculiarity observed in 13% of RPS patients is intraperitoneal dissemination, known as peritoneal sarcomatosis (PS) [21]. Theoretically, RPS should be restricted to the retroperitoneal space.…”

“…However, sometimes the anatomical boundary is ruptured, either by primary disease or iatrogenically, causing PS, seen as lesions along the peritoneal surfaces or intraperitoneal viscera. PS is related to advanced disease and dismal prognosis [21].…”