Fig. 17.1
T2-weighted image with fat saturation from an MRI of the chest in an infant shows a heterogeneous, high-signal-intensity mass filling the left ventricle in a patient with a cardiac rhabdomyosarcoma
17.2.4 Echocardiography Findings
The rhabdomyosarcoma will appear as a solid, hyperechoic mass with irregular borders [8]. Valvular dysfunction should be assessed if the mass involves the valves.
17.2.5 Imaging Recommendations
17.3 Differential Diagnosis
Angiosarcoma
Fibrosarcoma
Osteosarcoma
Leiomyosarcoma
Liposarcoma
Lymphoma
Intrapericardial pheochromocytoma
Metastatic disease
17.4 Pathology
17.4.1 Etiology
These tumors are usually sporadic. The etiology is unknown.
17.4.2 Frequency
Although they are the most common malignant primary cardiac tumor in infants and children, overall they account for only 4–7 % of cardiac sarcomas and are considered rare [1].
17.4.3 Gross Pathologic and Surgical Features
Large regions of central necrosis
Embryonal subtype most common in children [2]
Pleomorphic subtype occurs in adults and is much less common [2]
A portion of the tumor should always involve the myocardium (unlike angiosarcoma)
Pericardial involvement is usually nodular masses instead of a sheetlike spread [14]
Tumor rarely invades past the parietal pericardium [14]
17.5 Clinical Issues
17.5.1 Presentation
Male predilection [15]
No predilection for a certain cardiac chamber, so signs and symptoms vary
17.5.1.1 Most Common Signs/Symptoms
17.5.2 Treatment
Surgical resection can offer palliation of symptoms from valvular obstruction and improve survival. Heart transplantation has been performed for unresectable disease [18].
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
