ABSTRACT
Introduction and importance: Primary malignant pericardial tumors are an entity that is infrequently encountered and may be a cause of pericardial effusion. Primary pericardial leiomyosarcoma are even rarer, and highly aggressive tumors, with no more than 200 cases reported in the literature. In this case report, we are presenting a rare case of a primary pericardial leiomyosarcoma that was diagnosed at our institution. We discuss the available diagnostic modalities and also shed light on alternative therapies when patients are not ideal surgical candidates. Case presentation: A 27-year-old male patient was admitted with a gradually worsening dyspnea associated with a deterioration of general condition. Echocardiography examination showed a circumferentiel pericardial effusion with significant fibrin deposits and pericardial thickening. An open surgical biopsy of the pericardium was indicated which was in favor of the diagnosis of pericardial leiomyosarcoma. Unfortunately, the patient died during the procedure. Clinical discussion: Despite its rarity, primary pericardial leiomyosarcoma should be considered as a differential diagnosis in the assessment of a pericardial effusion of an unknown etiology. Cardiac magnetic resonance imaging is considered to be the reference standard technique for evaluation of a suspected pericardial tumor. Surgical biopsies provide the best odds for detection of the cell of origin, but it's fought with periprocedural risks depending on the site of the tumor. Conclusion: Primary pericardial leiomyosarcomas appear to have a poor prognosis. Surgical approach is the primary modality of treatment. Chemotherapy and radiotherapy should be offered to patients who are not ideal surgical candidates.
