Perinatology Unit, Carabobo University, Valencia – Venezuela. UPUC@hotmail.com

Synonyms:  none

Definition: Rare primary tumor of the heart that emerge from the pericardium, although it can be located in the auricle or ventricle. Generally they arise from the base of the heart and get their nutrition from the aorta or pulmonary artery. It consists of multiple cysts within solid areas that can be projected through the chambers of the heart.

Histology: Histologically there can be elements of the three germinal layer, the cysts being covered by a variety of epithelium that include: stratified squamous epithelia, cubical , secretory  or respiratory  epithelia. The solid areas content mature or immature neuroglial, pancreatic, thyroidal , muscular , cartilaginous or bony tissue. Most of the tumor reported in neonates has been benign.

Case report:  See case 36

These are the specimen and histological section of the tumor.

Prevalence:   Rare, the frequency of primary cardiac tumors varies between 0,17-0.28:10.000, 90% being of a benign type. Teratomas correspond to 19 to 25%, of these and rhabdomyomas 40 to 60% and fibromas 12 to 25%.

Presentation: In the fetus: pericardial tumor with mixed echogenicity, smooth or nodular surface that can displace the heart from its normal position. Pericardial fluid is almost always present and this can lead to a cardiac tamponade with heart failure clinic and hydrops. This can result in intrauterine death or arrhythmias.

In the neonates: can cause dysnea, cardiomegaly, congestive heart failure, cardiac tamponade, neonatal hydrops and sudden death.

Etiology:  Unknown

Pathogenesis:   Unknown

Sonographic findings:

The findings include:

• Heterogeneous tumor with smooth or nodular surface, variable size (usually 2-6cm) localized in the heart base and in contact with the aorta and pulmonary artery,
• The mass can displace or compress the heart chambers.
• Deviated heart axis
• Pericardial effusion: it is characteristic, it can be moderate or severe. The effusion can have a paradoxical motion on color Doppler, moving in the opposite direction of the cardiac chamber.
• Congestive heart failure that can be demonstrated by pulsed Doppler

Differential diagnosis:   Mediastinal or intracardiac tumors

Associated anomalies:   None aside from the hydrops

Prognosis:   Favorable in the cases with early diagnosis, term pregnancy and mild manifestations. However, in fetuses born with pericardial effusion and hydrops the outcome in uniformly lethal.

Recurrence risk:  none

Management:   Prenatal: pericardiocentesis in the cases with severe pericardial fluid, heart failure or hydrops. Neonatal: total tumor resection.

References:

1. Fujimory K, Honda S,Akutsu H, Ariga H,Ujiie N, Yanagida k: Prenatal diagnosis of intrapericardial teratoma: a case report. J Obstet Gynaecol Res 1999, 25(2): 133-6
2. Valioulis I, Aubert D,Lassauge F, Slimane MA: Intrapericardial teratoma diagnosed prenatally  in a twin fetus. Pediatr Surg Int 1999, 15(3-4): 284-6
3. Riskin-Mashiah S,Moise KJ Jr., Wilkins I,Ayres NA, Fraser CD Jr : In utero diagnosis of intrapericardial teratoma : a case for in utero open fetal surgery . Prenat Diagn 1998,18(12):1328-30
4. Paw PT, Jamieson SW: Surgical management of intrapericardial teratoma diagnosed in utero. Ann Thorac Surg 1997, 64(2):552-4
5. Cyr DR, Guntheroth WG, Nyberg DA, Smith JR, Nudelman SR, Ek M: Prenatal diagnosis of an intrapericardiac teratoma. A cause for noninmune hydrops. J Ultrasound Med 1988, 7(2): 87-90
6. Sepulveda W, Pericardial Teratoma. Inter J Obst Gyn Ultrasound June 2000