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.
背景：心包间皮瘤（PM）是一种极为罕见且预后不良的恶性肿瘤，其诊断与治疗策略尚未形成共识。本研究基于意大利1993年至2021年间确诊的PM病例开展了一项登记系统研究。方法：依托意大利国家间皮瘤登记系统（ReNaM）的数据，我们对PM病例的临床表现、诊断流程、石棉暴露史及治疗管理进行了描述性分析，计算总生存期，并采用单变量与多变量Cox模型评估特定变量的风险比（HR）及其95%置信区间（CI）。结果：共纳入72例病例（男性46例，女性26例），中位年龄66岁（范围22-89岁）。最常见的组织学亚型为上皮样型与未分型间皮瘤。近三分之二病例存在石棉暴露史。心包积液是最常见的临床表现。总中位生存期为2.8个月（95% CI 1.2-6.6），高龄诊断为不良预后因素。47例患者（65%）可获得临床与治疗数据：约半数患者接受手术治疗（其中三分之一为姑息性手术），7例患者（15%）接受辅助治疗。在具有治疗信息的病例中，肉瘤样亚型（HR 2.74，95% CI: 1.06-7.06）为不良预后因素；辅助治疗与生存改善相关（HR 0.38，95% CI 0.14-1.02），但无法排除适应症偏倚的混杂影响。结论：本研究证实PM预后极差。亟需建立收集含治疗信息的PM病例的国际协作网络，以改善患者生存质量并延长生存期。
Pericardial Mesothelioma: Diagnostic and Therapeutic Management, a Population-Based Study in Italy
肿瘤(癌症)患者之家