Primary cardiac fibroma in infants

Figures

• 

  Figure 1. Transthoracic echocardiography. (a) corresponds to an apical 5-chamber view that revealed a giant mass (35 mm × 40 mm) located at the IVS involving the cardiac crux. The mass produces a narrowing of the left ventricular outflow tract, with a minimum diameter of 3 mm (asterisk). (b) corresponds to a posterior parasternal short axis view that shows the giant mass occupying practically all of the ventricular cavities. IVS: Interventricular septum

• 

  Figure 2. Cardiac magnetic resonance imaging. The cardiac magnetic resonance imaging showed a large solitary tumor confined at the IVS measuring 36 cm × 35 cm × 46 mm, occupying most of both ventricular cavities. The mass was homogeneously isointense on T1-weighed imaging (a), and slightly hyperintense on T2-weighted imaging (b), with no hypocaptant lesions inside. There was a heterogeneous hypercaptation in the external wall of the tumor, with internal hypocaptation in late gadolinium enhancement sequences (c and d)

• 

  Figure 3. Pathology. (a) corresponds to the macroscopic examination. The explanted heart weighed 64 g, and contained a large (4 cm × 3.1 cm × 4.3 cm), nonencapsulated, solid, fasciculated, firm, and white/gray mass, without necrosis or cystic degeneration. The mass was localized within the interventricular septum and extended from the apex to the base, including the cardiac crux and compressing the coronary arteries. The tumor was bulging into both ventricles, occupying practically whole of both ventricular cavities. The (b)-left image corresponds to the microscopic examination of the tumor obtained by endomyocardial biopsy. The tumor was composed of monomorphic spindle cells arranged in a collagen stroma, with abundant elastic fibers, showing little or no atypia. The anatomopathological study showed infiltrate into the uninvolved cardiac muscle (M) as bands of fibroblasts (F) (H and E, ×100). The (b)-right image corresponds to the microscopic examination of the tumor in the explanted heart. The anatomopathological study revealed a cardiac muscle showing high immunoreactivity for desmin (m) which contracts with fibroblastic cells negativity (f) (Desmin, ×40). T: Tumor, LV: Left ventricle,…