• Join/Renew
  • Contact
    Contact Us
    USA OFFICE
    19 Mantua Road, Mt. Royal,
    NJ 08061, U.S.A.
    Phone: 856-423-8955
    Fax: 856-423-3420
    E-Mail: hq@scmr.org

Number 10-01: Myofibroblastic tumor of the right atrium in a 2-year old boy

Case from: Michael D Seckeler, MDa*, Christopher Clarke, MDa, James Gangemi, MDb, Robin D LeGalloc, Andrew Hoyer, MDa
a
Department of Pediatrics, bDepartment of Surgery, cDepartment of Pathology, University of Virginia, Charlottesville, Virginia, US.

Clinical history: A 2 year-old male with a history of one week febrile illness and cough was evaluated by his pediatrician and diagnosed with bronchitis and provided supportive treatment. However, the patient developed new respiratory distress.

A chest x-ray showed marked cardiomegaly and an echocardiogram revealed a large pericardial effusion (Movies1-3) with evidence of cardiac tamponade.

 


Movie 1


Movie 2

Movie 3

 

Movie 4

 

After urgent pericardiocentesis, a suspicious mass was noted in the right atrium on a repeat echocardiogram (Movie 4).
Cardiac magnetic resonance (CMR) imaging was performed to further evaluate the characteristics of the mass (Movies 5+6) and a rest 1st pass perfusion was performed (Movie 7).


Movie 5                                Movie 6           

 
Movie 7


Figure 1

 


Figure 2

CMR tissue characterization: In the TSE T1 images the mass appeared hyperintense (Figure 1). The mass remained hyperintense also when a fat saturation pulse was added, confirming that its nature was not lipomatous (Figure 2). 
In addition, the pericardium appeared abnormally thicked.    

The LGE images suggested that the mass had a fibrotic component (Figures 3 and 4).  

 
Figure 3
 



Figure 4

Based on the CMR findings, the patient went to surgery for an open biopsy.
On gross examination, there was markedly abnormal pericardium with gelatinous material in the pericardial space. Because of concern for a malignant tumor, the mass was resected.
On microscopic examination, the mass was found to be an inflammatory myofibroblastic tumor. The tumor shows a spindled cell population with a mixed inflammatory infiltrate with areas rich in plasma cells (Figure 5). The spindle cells have a stellate shape with large, hyperchromatic nuclei with coarse chromatin and prominent nucleoli (Figure 6).  


Figure 5


Figure 6

The patient recovered well from surgery and was discharged home.

Perspective: Cardiac tumors in children are quite rare, and the most common are rhabdomyomas. Myofibroblastic tumors are rare and usually occur in children, most commonly in the lungs or gastrointestinal tract. Fewer than 20 cases of primary cardiac myofibroblastic tumors have been described in the literature (ref 1). While generally benign, they can cause symptoms if they become obstructive. In general, treatment is complete excision, and up to 25% may recur (ref 2). While CMR images have been reported in an adult with myofibroblastic tumor, this is the first case to present CMR images of a myofibroblastic tumor (ref 3) of the heart in a child. This case illustrates how CMR may be used to better define the extent and composition of intracardiac tumors and aid in the decision for surgical resection, even in children.

References

1. Adler A, Fimbres A, Marcinak J, Johnson A, Zheng X, Hasegawa S, Shulman ST. Inflammatory pseudotumor of the heart caused by Listeria monocytogenes infection. J Infect. 2009;58:161-3.

2. Anvari MS, Soleimani A, Abbasi A, Boroumand MA, Marzban M, Karimi AA, Yazdanifard P, Shirani S, Sahebjam M. Inflammatory myofibroblastic tumor of the right ventricle causing tricuspid valve regurgitation. Tex Heart Inst J. 2009;36:164-7.

3. Moon JC, Sheppard MN, Lloyd G, Patel NR, Pennell DJ, Mohiaddin RH. Cardiac pseudotumor: tissue characterization by cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 2003;5:497-500.

Submit your case here

COTW handling editors: Chiara Bucciarelli-Ducci and Rory O'Hanlon

Benefits include (1) Annual Meeting and Educational Workshops at a reduced fees (2) Free authorship in the open access journal Journal of Cardiovascular Magnetic Resonance (3) Scientific forum for: publications, presentations, equipment specifications (4) Exclusive online content ... More

Discrepancies between cardiovascular magnetic resonance and Doppler echocardiography in the measurement of transvalvular gradient in aortic stenosis: the effect of flow vorticity

Garcia J, Capoulade R, Le Ven F, Gaillard E, Kadem L, Pibarot P and Larose ?
Journal of Cardiovascular Magnetic Resonance 2013, 15:84 (20 September 2013)

More: JCMR-online.com; view JCMR archive

SCMR/EuroCMR Joint Scientific Sessions

Save date:

February 5 – 7, 2015

Nice Acropolis Convention Centre Nice, France

More? or

Past Meetings

Platinum Level

Silver Level

New course: HCM: 18 cases, lectures.
Introductory course: 35 cases, lectures
Sample certificate. Guests? Have a go (no certificate)

Users: 1434. Certificates 270. 

NEW moodle site: http://education.scmr.org
Both
count for level 1, 2 or 3 training

More: list courses ...

forum always on

Echo LV Mass versus CMR
Discrepancies in Left Ventricular Mass Calculation Based on Echocardiography and Cardiovascular Magnetic Resonance Measurements in Patients with Left Ventricular Hypertrophy.Seo HY et al. J Am Soc Echocardiogr. 2015.... On: 07/26/2015 By: jfernandes4125 ... Read more?

Want to be trained in CMR from a distance?
Very interesting setup for training developed by German researchers:A Multicenter Cardiovascular MR Network for Tele-Training and Beyond: Setup and Initial ExperiencesFabian Muehlberg, MDa, Daniel Neumannb, Florian.... On: 07/19/2015 By: jfernandes4125 ... Read more?

Which CMR parameter detects significant CAD?
Individual component analysis of the multi-parametric cardiovascular magnetic resonance protocol in the CE-MARC trialJ Cardiovasc Magn Reson. 2015 Jul 15;17:59 - PDF here. On: 07/18/2015 By: jfernandes4125 ... Read more?

Anatomical versus functional assessment of CAD
Anatomical versus functional assessment of coronary artery disease: direct comparison of computed tomography coronary angiography and magnetic resonance myocardial perfusion imaging in patients with intermediate pre-test probability.Int J.... On: 07/13/2015 By: jfernandes4125 ... Read more?

© The Society for Cardiovascular Magnetic Resonance
2040.MySQL: 0.0000 s, 13 request(s), PHP: 0.1560 s, total: 0.1560 s  •  By BlueFigment.com