Key result
A 71-year-old male with primary cardiac desmoplastic small round cell tumor initially responded to the P6 chemotherapy protocol but experienced disease progression and is currently undergoing second-line treatment 19 months post-diagnosis.
Why the study?
Desmoplastic small round cell tumor is an aggressive malignancy usually found in the abdomen and pelvis but rarely described in the chest.
Case Report (n=1)
Answers are grounded in this paper and cite their sources.
This case highlights a rare presentation of primary desmoplastic small round cell tumor invading the pericardium and myocardium, leading to cardiac tamponade.
<ns3:p>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.</ns3:p>
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Guevara et al. (2022) conducted a case report in Cardiac desmoplastic small round cell tumor (DSRCT) (n=1). P6 chemotherapy protocol was evaluated on Clinical response and survival. A 71-year-old male with primary cardiac desmoplastic small round cell tumor initially responded to the P6 chemotherapy protocol but experienced disease progression and is currently undergoing second-line treatment 19 months post-diagnosis.
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