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Findings: Rounded, well-circumscribed mass (approximately 4 cm in diameter) adherent to free wall of right ventricle. Flow void is seen in right ventricular out flow indicating a patent PA with blood flow, but with tumor obscuring most of right ventricle. Signal intensity on T2 images is intermediate consistent with a lesion with relatively little free water.

Diagnosis: Cardiac fibroma.

Discussion: Primary tumors of the heart are rare with large autopsy studies reporting an incidence of between 0.0017 % and 0.03%. Most tumors are benign (75%), with the first and second most common being atrial myxomas (50% of all cardiac tumors) and rhabdomyoma (20%), respectively. The two most common pediatric tumors are rhabdomyoma (85% are found in children less than 15 years old - half are also found to have tuberous sclerosis) and fibroma. Fibromas, 4% of benign primary cardiac tumors, are almost uniformly found in the ventricle (R=L) and in children (M=F). Murmur, symptoms of heart failure, ischemia, arrhythmia, abnormal CXR, and failure to thrive often initiate a cardiac evaluation. While echocardiography can suggest the diagnosis of a cardiac tumor, MR is proving to be the gold standard in the pre-surgical evaluation. MR has been critical in defining tumor morphology, myocardial involvement, and potential for resectability. Surgical planning is often guided from MR result, with imaging at times demonstrating echocardiographic false positives - hence preventing an unnecessary surgical procedure. While benign tumors tend to have a good prognosis, tumor embolization, alteration in atrial/ventricular morphology, associated arrhythmias with sudden death (1/3 of all cases), and impedance to normal blood flow can cause significant morbidity and mortality, hence necessitating surgical treatment. Surgical correction, even with extensive cardiac involvement, is often curative with a low operative mortality and an excellent long term prognosis. The risk for potential malignant arrhythmias can remain following extensive tumor resection hence requiring adequate surveillance and treatment.

Pathology: Well differentiated fibroblast producing abundant cartilage is seen on pathologic biopsy.

References:
1. Lund JT, Ehman RL, Julsrud LJ, et al. Cardiac Masses:
Assessment by MR Imaging. AJR 1989 152:469-473.

2. Silverman NA. Primary Cardiac Tumors.
Annals of Surgery 1980 191:127-136.

3. Williams DB, Danielson GK, McGoon DC, et al. Cardiac Fibroma:
Long-term survival after excision. J Thorac Cardiovasc Surg 1982 84:230-236.

4. Salcedo EE, Cohen GI, White RD, et al. Cardiac Tumors:
Diagnosis and Management. Current Problems in Cardiology 1992 2:79-137.

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