Key result
Rare desmoplastic small round cell tumor triggers STEMI and cardiac tamponade in a young adult.
Why the study?
Desmoplastic small round cell tumors are very rare and aggressive diseases that can present atypically with cardiac manifestations such as ST-segment elevation myocardial infarction and cardiac tamponade in young adults.
Case Report (n=1)
STEMI and cardiac tamponade can be rare initial presentations of desmoplastic small round cell tumors in young adults.
Answers are grounded in this paper and cite their sources.
Neoplasm should enter the differential for young adults with STEMI; this single case leaves open whether broader evaluation protocols are needed.
Desmoplastic small round cell tumors (DSRCT) are very rare and aggressive diseases typically present with abdominal or retroperitoneal masses. We present a case of a young female who presented with ST-segment elevation myocardial infarction and cardiac tamponade and who was found to have DSRCT. The patient was coded at the emergency department. Left heart catheterization showed normal coronary arteries, and pericardiocentesis removed 1,260 mL of bloody pericardial effusions. The patient was stabilized, and a positron emission tomography scan revealed left intrahilar, hilar, and cardiophrenic masses with associated hypermetabolic right hilar, left hilar, subcarinal, costophrenic, aortopulmonary, paratracheal, and mediastinal lymphadenopathy. Cardiac magnetic resonance imaging showed multiple masses visualized in the pericardium, one mass anterior to the right ventricular outflow tract/pulmonary artery, and a second mass adjacent to the right ventricular apex. Computed tomography abdomen/pelvis showed no evidence of metastatic malignancy in the abdomen/pelvis. A biopsy of lung mass and lymph nodes showed desmoplastic small round cell tumors with sarcoma fusion gene detected (Ewing sarcoma RNA-binding protein 1-Wilms' tumor 1). We performed cycle 1 of chemotherapy, including doxorubicin, vincristine, and cyclophosphamide, and the patient was transferred to an oncology center for further care. This case suggested that one of the differential diagnoses of lung and pericardium masses at a young age can be desmoplastic small round cell tumors. This case also highlighted that ST-segment elevation myocardial infarction can be secondary to neoplasm, especially at a young age besides myocardial infarction.
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Takaoka et al. (2024) conducted a case report in Desmoplastic Small Round Cell Tumors (DSRCT) presenting with STEMI and cardiac tamponade (n=1). Chemotherapy (doxorubicin, vincristine, cyclophosphamide) was evaluated. A 24-year-old female presenting with STEMI and cardiac tamponade was found to have a rare desmoplastic small round cell tumor, highlighting that STEMI can be secondary to neoplasm in young adults.
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