Intrapericardial teratoma in the perinatal period

Excision of an intrapericardial immature teratoma in a 26-week premature neonate

“…As many as 15% of immature teratomas will be malignant, most often due to immature neuroectodermal components [3-5]. They are typically encapsulated with a vascular stalk, arising from the aorta or pulmonary artery, with the mass anterior to the heart with compression of the right atrium and ventricle [6]. …”

“…The incidence of cardiac tumors was first estimated using autopsy studies and found to be between 0.001 and 0.2% with the incidence of teratomas in infants accounting for 9% of those cases [6]. More recent estimates have been made using echocardiographic databases with an incidence of cardiac tumors between 0.17 and 0.20% in the largest studies [2,7].…”

“…Imaging after birth should include echocardiography to evaluate the size and characteristics of the tumor and effusion, cardiac function and mass effect. The diagnostic findings include multicystic structure, location at the right anterior heart border, attachment to the great vessels, and pericardial effusion [6]. Cystic components of the lesion appear echolucent and calcifications appear echogenic.…”

Intrapericardial teratoma with hydrops leading to in utero demise

“…Only one infant died, the one treated with in utero surgery. Among the 14 vaginal deliveries, there were seven in utero deaths, three terminations of pregnancy, one premature labor at 26 weeks' gestation (Bader et al, 2006), one intrapartum death and two uncomplicated term births in infants without hydrops (Ragupathy et al, 2003;MacKenzie et al, 2005). Planned delivery allows for better management of the neonate in specialized centers with neonatal intensive care and pediatric surgery facilities.…”

“…The timing of neonatal surgery is not standardized, and has been reported between the first hour and the 4th day of life. There are two cases in which surgery was performed later, on the 10th and 25th days of life (Ragupathy et al, 2003;MacKenzie et al, 2005) In conclusion, cardiac tumors are a rare cause of fetal hydrops, but are important to diagnose prenatally since therapy is possible in some cases. Pericardial teratomas develop rapidly and their prognosis is poor when the fetus is already hydropic.…”