Progress in Pediatric Cardiology, cilt.82, 2026 (ESCI, Scopus)
Background: Malignant pericardial mesothelioma is an exceptionally rare tumor in children and typically presents with nonspecific findings that mimic inflammatory pericardial diseases, frequently leading to diagnostic delay. Objectives: To describe the clinical presentation, diagnostic challenges, and outcome of a pediatric patient with malignant pericardial mesothelioma presenting as recurrent treatment-resistant pericardial effusion. Methods: We report a 15-year-old girl who presented with pleuritic chest pain, exertional dyspnea, and a large circumferential pericardial effusion. Initial pericardiocentesis yielded serohemorrhagic fluid with elevated protein and lactate dehydrogenase levels; cytology demonstrated reactive mesothelial cells without atypia. Extensive microbiologic and rheumatologic evaluation was unrevealing, and the patient was initially managed as presumed idiopathic/inflammatory pericarditis with colchicine, corticosteroids, and subsequently an interleukin-1 receptor antagonist. Results: Despite sequential anti-inflammatory therapy, the effusion recurred, and inflammatory markers remained persistently elevated. Serial imaging demonstrated progressive diffuse circumferential pericardial thickening without a discrete mass, raising suspicion of an infiltrative process. Due to worsening symptoms and persistent effusion, the patient underwent surgical pericardio-pleural window creation, which revealed a markedly thickened pericardium encasing the heart. Histopathology confirmed epithelioid malignant mesothelioma, and PET/CT subsequently showed diffuse pericardial hypermetabolism with mediastinal lymph node involvement. The patient deteriorated rapidly after oncologic referral and died 67 days after histopathologic diagnosis. Conclusion: Malignant pericardial mesothelioma in children can strongly resemble inflammatory pericarditis and may initially produce inconclusive cytology results. When a child presents with repeated, treatment-resistant pericardial effusion and increasing pericardial thickening, it is crucial to promptly consider malignant causes and perform a tissue biopsy without delay to ensure a timely diagnosis.