Multimodality imaging in cardiac myxofibrosarcoma

A 55-year-old male patient presented with chest discomfort, cough, and dyspnoea which had worsened in the prior 5 days. He had no significant medical history. He had elevated serum biomarkers including CEA-125 (119 U/mL, normal ≤35 U/mL), CK (1855 U/L, normal 24–195 U/L), CK-MB (124.2 µg/L, normal ≤5 µg/L), cTnI (25.4 µg/L, normal 0–0.0056 µg/L), and NT-proBNP (1408 pg/mL, normal 0–125 pg/mL). His electrocardiogram was a normal sinus rhythm with a normal rate. His echocardiogram showed a cardiac mass with small pericardial effusion. Coronary computed tomography angiography imaging revealed a large space-occupying mass in the pericardium encasing the pulmonary trunk (Panels A and B) without the ability to further characterize the tissue composition of the mass. Cardiac magnetic resonance (CMR) imaging was obtained for further evaluation. Short axis cine, two-chamber cine and four-chamber cine (see Supplementary material online, Video S1). Short-axis T1 and fat-suppressed T2-weighted imaging showed mixed T1 (Panel C) and mixed T2 signal intensities (Panel D) in the soft tissue mass, respectively. There is a higher T1 and T2 signal in the centre of the mass, which suggests that the mass may contain mucus protein (Panels C and D: white arrow). Cardiac magnetic resonance first perfusion showed early enhancement of some components (see Supplementary material online, Video S2). Short-axis late gadolinium enhancement image showed enhancement of the mass margin and pericardial enhancement (Panel E). The location of the inner delayed enhancement of the mass corresponding to the hypointense region of T2 maybe the most likely fibrous component (Panels D and E: blue arrow). T1 mapping (Panel F) and T2 mapping (Panel G) showed heterogenous T1 and T2 values within the mass, with areas of higher and lower values compared with normal myocardium. The CMR findings of this case suggest that there are components such as mucin, liquefied necrosis, and fibrous tissue in the tumour. The heterogenous appearance on tissue characterization in magnetic resonance imaging supports the diagnosis of myxofibrosarcoma. Multimodality imaging and haematoxylin–eosin staining in cardiac myxofibrosarcoma. The patient underwent tumour resection and atrial reconstruction. Postoperative pathology and immunohistochemistry confirmed cardiac myxofibrosarcoma. Haematoxylin–eosin staining (Figure 1) showed the spindle tumour cells arranged in fascicular, whorled, and storiform pattern, with mild cytologic atypia and myxoid stroma. Supplementary material is available at European Heart Journal – Case Reports online. The author thanks Drs Yuchi Han, Yinsu Zhu, Bin Liu, and Yinfeng Qian for useful discussions of the case. Consent: The authors confirm that written consent for submission and publication of this case report including images and associated text has been obtained from the patient in line with COPE guidance. Conflict of interest: None declared. Funding: None declared.