Quick Fire Cases
Nicholas Aizcorbe, MD
Pediatric Cardiology Fellow
University of Utah
Shawn Shaji, MD
Pediatric Advanced Cardiac Imaging Fellow
University of Utah
Andrea Harman, RT
MRI technologist
Primary Children's Hospital
Evets Wood, RT
MRI technologist
Primary Children's Hospital
Laura Mims, DO
Pediatric Hematology/Oncology Fellow
University of Utah
Spencer B. Barfuss, MD
Assistant Professor of Pediatrics
Boston Children's Hospital
Barbara K. Han, MD
Professor
University of Utah
Taumoha Ghosh, MD
Assistant Professor
Primary Children's Hospital/University of Utah
Sanja Dzelebdzic, MD
Pediatric Cardiologist
University of Utah/Primary Children's Hospital
John Colquitt, MD
Associate Professor
University of Utah
Edem Binka, MD
Assistant Professor
University of Utah
A 6-month-old previously healthy female presented with worsening eye bruising and swelling. She was subsequently found to have hyperleukocytosis (WBC 109.2 K/mcL), thrombocytopenia (20K), anemia (Hgb 5.6 g/dl), peripheral blasts (55%), and severe coagulopathy (INR 3.7, fibrinogen < 30 mg/dL). Flow cytometry was consistent with acute myeloid leukemia (AML). A baseline pre-chemotherapy echocardiogram was performed and revealed normal biventricular systolic function, a small pericardial effusion, and a large intrapericardial mass in the right atrioventricular groove (AV) that was adherent to the right atrium (RA) (Figure 1). It appeared vascular and communication with the RA could not be excluded. Cardiac magnetic resonance imaging (CMR) was ordered to better characterize the mass and its relationship with the RA.
Diagnostic Techniques and Their Most Important Findings:
Standard functional assessment was performed followed by tumor tissue characterization (1.5T Siemens Magnetom Sola (Siemens Healthcare, Erlangen, Germany) including T1W imaging with and without fat saturation, T2W imaging with and without fat saturation, first pass perfusion, delayed enhancement (PSIR), and 3D SSFP. The images demonstrated normal biventricular function and a large intrapericardial mass in the posterior left AV groove that extended into the right (Figure 2). There was a vessel in the periphery of the mass, that could represent the coronary sinus. The mass was isointense on balanced SSFP cine, had slightly increased T1 signal, and was hyperintense on T2W images. There was uniform perfusion, slightly delayed compared to myocardium. There was no evidence of delayed enhancement.
Learning Points from this Case:
The imaging and tissue characterization was most consistent with myeloid sarcoma (chloroma). These tumors represent most common solid tumors in the context of AML. A similar mass was reported by Laste et al (2023) in a 64-year-old patient with AML. On induction chemotherapy day 14, repeat echocardiogram showed the mass had shrunk in size considerably (Figure 3). Cardiac CT performed the following day did not visualize the mass. Further confirming the diagnosis of myeloid sarcoma that reduced in size with induction chemotherapy. This is the first intrapericardial myeloid sarcoma reported in a pediatric patient.
