Characterization of Cardiac Tumors in Children by Cardiovascular Magnetic Resonance Imaging: A Multicenter Experience

OBJECTIVES: Individual centers have relatively little experience with diagnostic imaging of cardiac tumors in children, because of their low prevalence. The accuracy of cardiac MRI diagnosis on the basis of a pre-defined set of criteria has not been tested.The aim of this study was to report the results of an international multicenter experience of cardiac magnetic resonance imaging (MRI) evaluation of cardiac tumors in children, each with histology correlation or a diagnosis of tuberous sclerosis, and to determine which characteristics are predictive of tumor type.

METHODS: An international group of pediatric cardiac imaging centers was solicited for case contribution. Inclusion criteria comprised: 1) age at diagnosis 18 years; 2) cardiac MRI evaluation of cardiac tumor; and 3) histologic diagnosis or diagnosis of tuberous sclerosis. Data from the cardiac MRI images were analyzed for mass characteristics. On the basis of pre-defined cardiac MRI criteria derived from published data, 3 blinded investigators determined tumor type, and their consensus diagnoses were compared with histologic diagnoses.

RESULTS: Cases (n = 78) submitted from 15 centers in 4 countries had the following diagnoses: fibroma (n = 30), rhabdomyoma (n = 14), malignant tumor (n = 12), hemangioma (n = 9), thrombus (n = 4), myxoma (n = 3), teratoma (n = 2), and paraganglioma, pericardial cyst, Purkinje cell tumor, and papillary fibroelastoma (n = 1, each). Reviewers who were blinded to the histologic diagnoses correctly diagnosed 97% of the cases but included a differential diagnosis in 42%. Better image quality grade and more complete examination were associated with higher diagnostic accuracy.

CONCLUSIONS: Cardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.

PMID:

Jacobo Kirsch

Associate Center Director, Imaging Institute
Cardiopulmonary Imaging, Section Head
Cleveland Clinic Florida
Weston, FL
Posted in Magnetic Resonance Imaging and tagged , , , .

One Comment

  1. Very nice paper! Classified appropriately as original research, but great as a review paper!

    Interesting that they had twice as many fibromas than rhabdomyomas!

    When reading the paper I saw that 2 patients with benign cardiac tumors had died in the peri-operative period. I was wondering if the patients underwent surgery because the tumors were affecting the cardiac function, or because the treating physician was uncertain of the diagnosis.
    An important point is mentioned in the discussion: “Importantly, no malignant tumor was misdiagnosed as benign on the basis of the pre-defined criteria. However, malignancy was included as part of the differential diagnosis for several benign tumors.” – That answers my above question.

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