PEC‐PRO: A new prognostic score from a series of 87 patients with localized perivascular epithelioid cell neoplasms (PEComas) treated with curative intent

Abstract

AbstractBackgroundPerivascular epithelioid cell neoplasms (PEComas) encompass a heterogeneous family of mesenchymal tumors. Previously described clinicopathologic features aimed at distinguishing benign from malignant variants but lacked prognostic value.MethodsThis retrospective analysis examined clinicopathologic data from patients who had localized PEComa across French Sarcoma Network centers. The authors analyzed 12 clinicopathologic features in a Cox proportional hazard framework to derive a multivariate prognostic risk model for event‐free survival (EFS). They built the PEComa prognostic score (PEC‐PRO), in which scores ranged from 0 to 5, based on the coefficients of the multivariate model. Three groups were identified: low risk (score = 0), intermediate risk (score = 1), and high risk (score ≥ 2).ResultsAnalyzing 87 patients who had a median 46‐month follow‐up (interquartile range, 20–74 months), the median EFS was 96.5 months (95% confidence interval [CI], 47.1 months to not applicable), with 2‐year and 5‐year EFS rates of 64.7% and 58%, respectively. The median overall survival was unreached, with 2‐year and 5‐year overall survival rates of 82.3% and 69.3%, respectively. The simplified Folpe classification did not correlate with EFS. Multivariate analysis identified three factors affecting EFS: positive surgical margins (hazard ratio [HR], 5.17; 95% CI, 1.65–16.24; p = .008), necrosis (HR, 3.94; 95% CI, 1.16–13.43; p = .030), and male sex (HR, 3.13; 95% CI, 1.19–8.27; p = 0.023). Four variables were retained in the prognostic model. Patients with low‐risk PEC‐PRO scores had a 2‐year EFS rate of 93.7% (95% CI, 83.8%–100.0%), those with intermediate‐risk PEC‐PRO scores had a 2‐year EFS rate of 67.4% (95% CI, 53.9%–80.9%), and those with high‐risk PEC‐PRO scores had a 2‐year EFS rate of 2.3% (95% CI, 0.0%–18.3%).ConclusionsThe PEC‐PRO score reliably predicts the risk of postoperative recurrence in patients with localized PEComa. It has the potential to improve follow‐up strategies but requires validation in a prospective trial.