Cardiac Masses - Cases

1350811 - Atypical presentation of a rare benign cardiac tumor: left ventricular papillary fibroelastoma

Friday, January 27, 2023

10:20 AM – 11:20 AM

Location: Pacific Jewel

Presenting Author(s)

Carlos Eduardo E. Rochitte, MD, PhD

Associate professor of Cardiology
Heart Institute, University of São Paulo
Sao Paulo, Sao Paulo, Brazil

Primary Author(s)

AS

Artur Sarmet, MD

Fellow in Cardiovascular Imaging
Heart Institute, University of São Paulo, Brazil

Co-Author(s)

KD

Kevin Rafael De Paula Morales, MD

Fellow in Cardiovascular Imaging
Heart Institute, University of São Paulo, Sao Paulo, Brazil
GB

Gabriela Barros, MD

Fellow in Cardiovascular Imaging
Heart Institute, University of São Paulo, Brazil
AV

Andre Vaz, MD

Fellow in Cardiovascular Imaging
Heart Institute, University of São Paulo, Brazil
RF

Rafaela Franklin, MD

Fellow in Cardiovascular Imaging
Heart Institute, University of São Paulo, Brazil
DR

Diana Rodrigues, MD

Fellow in Cardiovascular Imaging
Heart Institute, University of São Paulo, Brazil
PM

Pedro Matta, MD

Fellow in Cardiovascular Imaging
Heart Institute, University of São Paulo, Brazil
MR

Maria Rahal, MD

Fellow in Cardiovascular Imaging
Heart Institute, University of São Paulo, Brazil
EF

Eduardo Fonseca, MD, PhD

Radiologist
Heart Institute, University of São Paulo, Brazil

Description of Clinical Presentation: A 69-year-old man, asymptomatic, with hypertension, coronary artery disease and history of angioplasty 14 years ago, under investigation for the etiology of a recent ischemic stroke, presented on echocardiogram a mass in the LV lateral wall, described as compatible with myxoma. Cardiac magnetic resonance imaging was indicated for diagnostic elucidation.

Diagnostic Techniques and Their Most Important Findings:

CMR was performed with standard cardiac tumor protocol:

Cine-resonance on SSFP sequences for evaluation of global and segmental ventricular function.

Spin echo double IR and triple IR (T2-WI) for tissue characterization. Additional Double IR sequence with fatsat.

First-pass perfusion with gadolinium.

Late gadolinium enhancement (LGE) for assessment of fibrosis / infarction and myocardial viability.

CMR showed a pedunculated nodular formation, originating from the right lateral aspect of the anterolateral papillary muscle of the LV, mobile with the cardiac cycle, showing isointense signal on T1-weighted images and high signal on T2-WI with and without fat saturation, with no significant perfusion and peripheral heterogeneous delayed enhancement, with hypovascular central peduncle (Figures 1 and 2). The characteristics of the lesion correspond slightly better to myxoma, in addition to the fact that the location is extremely atypical for fibroelastoma. It was suggested in the report myxoma and, less probably, fibroelastoma.

Learning Points from this Case:

Mixomas are the most common primary benign cardiac tumors. They are well-defined ovoidal mobile lobulated masses frequently found in the left atrium, arising from the interatrial septum. It most frequently presents isointense signal in T1-WI and hyperintense signal in T2-WI. At first pass perfusion, it presents weaker enhancement than myocardium and about half of cardiac myxomas present heterogeneous LGE.

Papillary fibroelastoma (PF) is a rare primary benign cardiac tumor that usually involves cardiac valves. It represents about 7% of all primitive cardiac tumors. They are usually small lesions ( < 1,5 cm) composed of collagen and elastic fibers lined by endothelium with a short pedicle. The most common locations are the atrial side of the mitral valve and the aortic surface of the aortic valve leaflets. PF presents isointense signal in both T1- and T2-weighted images. On SSFP sequences, it appears as a well circumscribed mobile valve nodule with possible perilesional artefact. PFs may not show LGE, but when they do, it is usually homogeneous.

The patient underwent cardiac surgery and histopathological analysis confirmed papillary fibroelastoma (Figure 3). It has systemic embolization as a concerning complication. The most common clinical presentations described are cerebral vascular accident or transient ischemic attack. The therapeutic proposal is surgical resection aiming at the prevention of cerebral, pulmonary, coronary or peripheral embolic phenomenons.