Cardiac Tumors




Abstract


Cardiac tumors are usually benign (mainly rhabdomyoma, teratoma, fibroma, myxoma, or hemangioma), with no association to other anomalies and good prognosis. The exception to this is multiple rabdomyomas that very frequently associate tuberous sclerosis.




Keywords

cardiac tumors, rhabdomyoma, teratoma, fibroma, myxoma, hemangioma, tuberous sclerosis

 




Introduction


Cardiac tumors are rare and usually benign with few cardiac complications; most are asymptomatic and eventually involute. Cardiac complications include arrhythmias, obstruction of the ventricular outflow tracts, and secondary cardiogenic shock leading to fetal hydrops and death. Prenatal diagnosis is helpful to monitor these cases and, if necessary, to schedule delivery or perform in utero treatment to improve secondary heart failure.




Disease


Definition


Cardiac tumors are benign (95%) or malignant (5%) neoplasms arising primarily in the inner lining, muscle layer, or the surrounding pericardium of the heart.


Prevalence and Epidemiology


Cardiac tumors are uncommon, with an estimated incidence during fetal life of approximately 1.4 : 1000. The estimated prevalence from pediatric autopsy series is 0.017 : 1000 to 0.28 : 1000.


Etiology and Pathophysiology


Most cardiac tumors are benign (95%). Rhabdomyoma is the most common cardiac tumor during fetal life and childhood ( Fig. 94.1 ). It is followed in frequency by teratoma, fibroma, myxoma, and hemangioma.




Fig. 94.1


Single rhabdomyoma. Hyperechoic tumor is in the apex of the ventricular wall.


Cardiac tumors are usually isolated, with no association with chromosomal anomalies or other structural malformations. The only exception is rhabdomyomas, which show a very high association (75% to 90%) with tuberous sclerosis ( Fig. 94.2 ). Detection of multiple cardiac tumors should raise a strong suspicion of rhabdomyoma and tuberous sclerosis. A detailed family history should be obtained in such patients, and genetic counseling should be offered. Tuberous sclerosis is a rare multisystemic neuroectodermal disease characterized by multiple cardiac, intracranial, renal, pulmonary, and skin tumors. Rhabdomyomas generally regress after birth, although the associated neurodevelopmental complications (four-fifths of patients have epilepsy, and two-thirds have delayed neurodevelopment) dominate the clinical picture and should be an important part of the prenatal counseling of parents.




Fig. 94.2


Multiple rhabdomyomas. Hyperechoic tumors spread by the myocardium, ventricular walls, and septum.


Prenatally, cardiac tumors are usually detected in the third trimester. Most keep on growing during pregnancy without hemodynamic consequences. However, depending on the size, number, and location, complications such as arrhythmias, coronary flow reduction, or outflow tract or foramen ovale obstruction can occur, triggering heart failure, fetal hydrops, or eventually perinatal death. It is mandatory to perform complete structural and functional echocardiography and to arrange regular follow-up.


Manifestations of Disease


Clinical Presentation


The clinical presentation includes a cardiac mass and occasionally fetal hydrops.


Imaging Technique and Findings


Ultrasound.


The tumor characteristics on ultrasound depend on the histologic content:


Jul 7, 2019 | Posted by in OBSTETRICS & GYNAECOLOGY IMAGING | Comments Off on Cardiac Tumors
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