Primary pericardial mesothelioma

Primary pericardial mesothelioma is an extremely rare malignant tumor arising from the mesothelial cells lining the pericardium, the sac surrounding the heart. It accounts for a very small fraction of all mesothelioma cases, with most mesotheliomas occurring in the pleura (lung lining) or peritoneum (abdominal lining). Unlike pleural mesothelioma, the association with asbestos exposure in pericardial mesothelioma is less clearly established, though some cases have reported prior asbestos exposure. The disease primarily affects the cardiovascular system. As the tumor grows within the pericardial space, it can cause pericardial effusion (fluid accumulation around the heart), constrictive pericarditis, cardiac tamponade, chest pain, dyspnea (shortness of breath), fatigue, and heart failure symptoms. Patients may also present with constitutional symptoms such as weight loss and fever. The clinical presentation is often nonspecific, which contributes to frequent delays in diagnosis. Many cases are diagnosed incidentally during cardiac surgery or at autopsy. Treatment options are limited and the prognosis is generally poor. Surgical resection (pericardiectomy) may be attempted when feasible, and some patients receive chemotherapy, often with pemetrexed and cisplatin regimens used for other mesothelioma subtypes. Radiation therapy has also been employed in select cases. Pericardiocentesis or pericardial window procedures may be performed for symptomatic relief of pericardial effusion. Due to the rarity of this condition, there are no standardized treatment protocols, and management is largely guided by case reports and small case series. The median survival after diagnosis is typically measured in months, though outcomes vary depending on the stage at diagnosis and the feasibility of surgical intervention.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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