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Primary intimal sarcoma of the left atrium presenting with constitutional symptoms

2017; Oxford University Press; Volume: 2017; Issue: 7 Linguagem: Inglês

10.1093/omcr/omx031

2053-8855

Edite Marques Mendes, António Ferreira, Paula Felgueiras, Augusta Silva, Carlos Alberto Ribeiro, Diana Guerra, Daniel Melo, José Manuel Lopes,

Cardiac Structural Anomalies and Repair

Intimal (spindle-cell) sarcomas are exceptionally rare and are highly aggressive cardiac tumors. The authors describe a case of a 43-year-old female, presenting with a 3-month history of constitutional symptoms with fever, night sweats, anorexia and weight loss, associated with productive cough and pleural effusion that was admitted with clinical suspicion of pulmonary tuberculosis. The patient developed sudden acute heart failure symptoms during hospitalization, leading to mechanical ventilation. Computed tomography scan with contrast showed a cardiac tumor filling the left atrium causing compression of pulmonary veins. Surgical resection was performed and histologic examination revealed an intimal sarcoma. Although commenced on adjuvant chemotherapy, local tumor recurrence occurred with pericardium invasion. The patient died within 4 months of initial diagnosis. This report aims to describe an unusual presentation of this rare disease entity, and to discuss its highly aggressive clinical course.