Case Report: A pericardial effusion secondary to cardiac desmoplastic small round cell tumor

Abstract

Desmoplastic small round cell tumor (DSRCT) is an aggressive malignancy usually described in the abdomen and pelvis of adolescent males but rarely in the chest. A 71-year-old male presented with chest pain and was found to have pericardial effusion with cardiac tamponade. He underwent pericardiocentesis and pericardial window. Pericardial fluid and cardiac biopsy results confirmed DSRCT. The patient received the P6 protocol with a good but brief response. He had multiple hospitalizations in the following months for pericardial fluid recollection. Repeat imaging showed mediastinal metastasis. He is currently undergoing second-line chemotherapy treatment. We describe a unique case of primary DSRCT invading the pericardium and myocardium and exhibiting extensive mediastinal metastasis. This is the fourth report of cardiac DSRCT in the literature.