Pericardial mesothelioma (PM) is an extremely rare tumour for which only case reports, small case series, and case reviews have been published. This is a large population-based study on PM in Italy using data from the Italian National Mesothelioma Registry (Registro Nazionale Mesoteliomi, ReNaM). Information collected includes detailed clinical data on diagnosis and treatment and asbestos exposure. This study confirmed the extreme rarity of [pericardial mesothelioma (PM)] with crude incidence rates for men and women in 1993–2021 of 0.60 and 0.30 per 10 million person-years, respectively. Epidemiological characteristics of the tumour included late age at onset, male predominance, frequent occupational asbestos exposure, and an extremely poor median survival of 2.8 months. Surgery, when feasible, is the mainstay of treatment. The role of adjuvant treatments is unclear. An early diagnosis combined with standard treatment guidelines could improve patient prognosis and quality of life.
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 [pericardial mesothelioma (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 [pericardial mesothelioma (PM)] . An international network collecting PM cases with treatment information is needed to improve patient quality of life and survival.
1. Introduction
Pericardial mesothelioma (PM) is an extremely rare cancer accounting for 1–2% of all mesotheliomas [1] and 4% of heart and pericardial tumours [2]. This malignancy arises from pericardial mesothelial cells and affects mostly males, with a gender ratio (male versus female) ranging from 1:1 to 3:1 [3,4,5,6]. In a recent Italian study [7], the gender ratio for PM was 1.95, increasing from 1.5 in 1993–2003 to 2.6 in 2004–2015. The Surveillance, Epidemiology, and End Results (SEER) Program from the US National Cancer Institute calculated mean annual standardised incidence rates for pericardial mesothelioma of 0.35 and 0.36 per 10 million person-years in men and in women, respectively [7].
Asbestos exposure is the primary risk factor: a recent case–control study in Italy estimated a fourfold increased PM risk for occupational asbestos exposure [7].
[Pericardial mesothelioma (PM)] symptoms include fatigue, shortness of breath, chest pain, cough (frequently non-productive), and peripheral oedema; in fact, the clinical onset is generally related to signs of constrictive pericarditis, pericardial effusion, cardiac tamponade, and/or heart failure [5,8]. Other clinical unusual presentations are myocardial infarction, peripheral lymphadenopathy, and superior vena cava thrombosis [9,10,11].
PM survival is poor, typically between 6 and 18 months; moreover, up to 12.6% of cases are diagnosed after death [5]. Metastases occur in 25% to 45% of patients, and involve mostly regional lymph nodes, lungs, kidneys, and liver [8]. There is no consensus for disease management. Surgery seems to be associated with survival benefit in selected cases. Platinum-based chemotherapy showed increased survival in several reported case series [3,5,8]. Recently, a longer overall survival was observed in a few patients treated with trimodality therapy consisting of surgical resection, adjuvant chemotherapy, and sequential adjuvant intensity-modulated radiation therapy [12].
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