Abstract
BACKGROUND: Pericardial mesothelioma (PM) is an extremely rare cancer with a poor prognosis and no consensus on diagnostic and therapeutic management. We conducted a registry-based study on PM cases in Italy diagnosed between 1993 and 2021. METHODS: Based on data from the Italian National Mesothelioma Registry (ReNaM), we performed a descriptive analysis of PM cases including clinical presentation, diagnostic work-up, asbestos exposure, and therapeutic management. Overall survival was calculated. Hazard ratios (HRs) and 95% confidence intervals (CI) for selected variables were estimated with univariate and multivariate Cox models. RESULTS: We identified 72 cases (46 men and 26 women). Median age was 66 years (range 22-89). The most frequent histological subtypes were epithelioid and unspecified mesothelioma. Almost two-thirds of cases had been exposed to asbestos. The most common clinical presentation was pericardial effusion. Overall median survival was 2.8 months (95% CI 1.2-6.6) and older age at diagnosis was a negative prognostic factor. Clinical and treatment data were available for 47 patients (65%). Approximately one out of two patients underwent surgery (palliative, in one-third of cases). Adjuvant therapy was administered to seven patients (15%). In cases with treatment information, sarcomatoid subtype (HR 2.74, 95% CI: 1.06-7.06) was a negative prognostic factor; adjuvant therapy was associated with better survival (HR 0.38, 95% CI 0.14-1.02), but confounding by indication cannot be excluded. CONCLUSIONS: We confirmed the very poor prognosis of PM. An international network collecting PM cases with treatment information is needed to improve patient quality of life and survival.