Fighting a rare heart malignancy: First expert consensus on the diagnosis and treatment of malignant pericardial mesothelioma released

Los Angeles — Primary malignant pericardial mesothelioma (PMPM) accounts for a fraction of all mesotheliomas, yet it presents one of the most significant challenges in thoracic oncology. Characterized by insidious progression and local invasiveness, the disease is frequently diagnosed at advanced stages, leading to a median survival of approximately six months if left untreated. To address the critical lack of standardized management strategies, the Chinese Alliance of Research for Mesothelioma (ChARM) has convened experts to develop the "Expert Consensus on the Diagnosis and Treatment of Malignant Pericardial Mesothelioma," published in the journal Med Research.

This consensus represents a significant step forward in the management of this rare malignancy. By integrating evidence-based research with multicenter clinical experience, the expert group has formulated comprehensive recommendations covering epidemiology, screening, diagnosis, and therapeutic interventions.

The Diagnostic Challenge

The consensus emphasizes that PMPM lacks specific clinical manifestations, often masquerading as constrictive pericarditis or heart failure. To combat high misdiagnosis rates, the experts recommend a "multimodal imaging" approach. While echocardiography serves as the preferred initial screening tool, the consensus advises integrating Computed Tomography (CT), Magnetic Resonance Imaging (MRI), and Positron Emission Tomography (PET/CT) to accurately assess tumor extent and metastasis.

Definitive diagnosis relies on histopathology. The guidelines strongly recommend pericardial needle biopsy over fluid cytology, which has low sensitivity. The consensus details specific immunohistochemical markers—such as Calretinin, WT-1, and BAP1—required to distinguish PMPM from other tumors. It notably highlights that genetic testing for BAP1 mutations should be considered, particularly in younger patients without asbestos exposure, as this may correlate with clinical outcomes.

A Shift in Treatment Paradigms

Historically, treatment for PMPM has been palliative. However, the new consensus outlines a more aggressive, multimodal therapeutic strategy. While radical surgery is recommended for localized tumors, the experts note that most cases require systemic therapy.

A key highlight of the consensus is the endorsement of immune checkpoint inhibitors. The introduction of PD-1/PD-L1 inhibitors, combined with platinum-based chemotherapy, has marked a paradigm shift, extending median survival in some cohorts to up to 24 months. The guidelines also stress the importance of a Multidisciplinary Team (MDT) approach—involving cardiologists, oncologists, pathologists, and surgeons—initiated within 48 hours of diagnosis to optimize personalized treatment plans.

Standardizing Prognosis and Follow-up

To ensure systematic monitoring, the consensus proposes a stratified prognostic model incorporating tumor biomarkers. This standardization aims to facilitate better tracking of disease progression and therapeutic efficacy, ultimately aiming to translate clinical research advances into tangible survival benefits for patients suffering from this rare disease.