PERICARDIAL TERATOMA

Teratomas arising from the pericardium represent a rare cause of an intrathoracic mass, but can cause severe cardiorespiratory compromise. They are usually diagnosed during infancy due to congestive cardiac failure but may cause pericardial tamponade from a large pericardial effusion or cardiac failure due to inflow or outflow obstruction.
They constitute 18-25% of pediatric cardiac tumors.

The tumor is composed of all three germ layers and may therefore contain calcium, bone and fat.

It usually attaches to the aortic root and pulmonary vessels by a pedicle (1).

ULTRASOUND (1-10)

• Predominantly solid, intrathoracic mass intimately related to the heart.
• ± Posterior shadowing if calcium or bone is present.
• It may be echogenic without shadowing if only fat is present.
• Almost all have an associated pericardial effusion, which may cause cardiac tamponade, hydrops and death (4).
• Although they are intrapericardial, most are extracardiac and are attached to the root of the aorta or pulmonary artery by fine adventitial vessels.
• Heart is displaced and looses its normal axis.
• Mediastinal shift.
• Fetal hydrops.
• ± Polyhydramnios.
• Pulmonary hypoplasia.
• Congestive cardiac failure and hydrops by any of three mechanisms:

• Tamponade from the pericardial effusion.
• Direct compression of the vena cava.
• Direct compression of the aorta and / or pulmonary vessels.
• Rate of growth is very variable.
• May grow very slowly.
• Mass may rapidly increase in size between subsequent scans.

Case 1
Case 2

OUTCOME

Ref	GA at Diagnosis	Pericardial Effusion	Hydrops	Outcome
(2) (1) (6) (7) (8)	32 26 29 36 32	Yes Yes	No Yes Yes No No	Birth at term. Surgery. Alive and well Intrauterine demise at 29 weeks Intrauterine demise at 30 weeks 46,XY. Surgery, alive and well at 9 months

REFERENCES

1. Cyr DR, Guntheroth WG, Nyberg DA et.al. Prenatal diagnosis of an intrapericardial teratoma. A cause for non immune hydrops. J Ultrasound Med 1988;7:87-90.
2. DeGeeter B, Kretz JG, Nisan I et.al. Intrapericardial teratoma in a newborn infant: A cause for non-immune hydrops. Ann Thorac Surg 1983;35:664-668.
3. Farooki ZQ, Arciniegas E, Hakimi M et.al. J Clin Ultrasound 1985;10:125.
4. Sklansky M, Greenberg M, Lucas V et.al. Intrapericardial teratoma in a twin fetus: Diagnosis and management. Obstet Gynecol 1997;89:807-809.
5. Arciniegas E, Hakimi M, Farooki ZQ et.al. Intrapericardial teratoma in infancy. J Thorac Cardiovasc Surg 1980;79:306-311.
6. Weinraub Z, Gembruch U, Fodisch HJ et.al. Intrauterine mediastinal teratoma associated with non-immune hydrops fetalis Prenat Diagn 1989;9:365.
7. Todros T, Gaglioto P, Presbitero P. Management of a fetus with intrapericardial teratoma diagnosed in utero. J Ultrasound Med 1991;10:287-290.
8. Rasmussen S, Wei Sek H, Harder J et.al. Intrapericardial teratoma, ultrasonic and pathologic features. J Ultrasound Med 1987;6:159-16
9. Alegre M, Torrents M, Carreras E et.al. Prenatal diagnosis of intrapericardial teratoma. Prenat Diagn 1990;10:199-202.
10. Benatar A, Vaughan J, Nicolini U, et.al. Prenatal pericardiocentesis: its role in the management of intrapericardial teratoma. Obstet Gynecol 1992;79:856-858.

REPORTED CASES

Ref	GA (wks)		Tumor size (cm)	Pericardial effusion	Hydrops	Poly	Treatment	Newborn	Outcome
	Diagnosis	Delivery
1	32	Term	5^*	+	-	NR	Surgery	Asymptomatic	Healthy
2	32	32	4	+	+	+	-	Dead	Dead
3	26	29	6	+	+	+	-	Dead	Dead
4	36	38	2	+	-	+	Surgery	Distress	Healthy
5	36	37	2.6	+	-	-	Surgery	Distress	Healthy
6	23	25	2.3	+	+	NR	-	Dead	Dead
7	28	37	4	+	-	-	Surgery	Distress	Healthy
8	24	34	4.5	+	+	NR	Surgery	Distress	Healthy
9	25	28	5.8^*	+	+	NR	-	Dead	Dead
9	25	34	2.3	+	+	+	Surgery	Distress	Healthy
10	26	32	3.8	+	+	+	Surgery	Distress	Healthy
11	26	28	4.2	+	+	NR	-	Dead	Dead
12	28	32	7.5^*	NR	+	NR	Surgery	Distress	Healthy
13	34	37	5.4	+	+	NR	Surgery	Distress	Healthy
14	20	35	9^*	+	+	NR	Surgery	Distress	Healthy
15	35	38	3^*	+	+	+	-	Dead	Dead
16	20	23	3	+	+	NR	TOP	Dead	Dead
17	32	34	6^*	+	+	+	Surgery	Distress	Healthy
18	34	35	2.8	+	+	-	Surgery	Distress	Healthy
19	30	39	3.5	+	-	-	Surgery	Asymptomatic	Healthy
20	26	34	4.5^*	+	+	NR	Surgery	Distress	Healthy
20	NR	34	7^*	NR	NR	NR	Surgery	Distress	Dead
20	34	37	3^*	+	NR	NR	Surgery	Distress	Healthy
21	37	37	4	+	NR	NR	Surgery	Asymptomatic	Healthy
22	19	20	2.6	+	+	-	TOP	Dead	Dead
23	28	36	4.5	Small	+	-		Died day 1	Dead
^* Postnatal size. TOP – Termination of pregnancy. NR Not reported.

DIFFERENTIAL DIAGNOSIS

• Diaphragmatic hernia.
• Mass arising from myocardium (rhabdomyoma, fibroma).
• Cardiac tumor (rhabdomyoma, fibroma, myxoma, hemangioma).
• Ventricular aneurysm.
• Other intrathoracic masses (cystic adenomatoid malformation, broncho- pulmonary sequestration).

REFERENCES

1. De Geeter B, Kretz J, Nisand I, Eisenmann B, Kieny M, Kieny R. Intrapericardial teratoma in a newborn infant: use of fetal echocardiography. Ann Thorac Surg 1983; 35: 664-665.

2. Rasmussen S, Hwang W, Harder J, Nicholson S, Davies D, Nimrod C. Intrapericardial teratoma. Ultrasonic and pathological features. J Ultrasound Med 1987; 6: 159-162.

3. Cyr D, Guntheroth W, Nyberg D, Smith J, Nude.lman S, Ek M. Prenatal diagnosis of a intrapericardial teratoma. A cause for nonimmune hydrops. J Ultrasound Med 1988; 7: 87-90

4. Alegre M, Torrents M, Carreras E, Mortera C, Cusí V, Carrera J. Prenatal diagnosis of intrapericardial teratoma. Prenat Diagn 1990; 10: 199-202.

5. Todros T, Gaglioti P, Presbitero P. Management of a fetus with intrapericardial teratoma diagnosed in utero. J Ultrasound Med 1991; 10: 287-290.

6. Catanzarite V, Mehalek K, Maida C, Mendoza A. Early sonographic diagnosis of intrapericardial teratoma. Ultrasound Obstet Gynecol 1994; 4: 505-507.

7. Benatar A, Vaughan J, Nicolini U, Trotter S, Corrin B, Lincoln C. Prenatal pericardiocentesis: its role in the management of intrapericardial teratoma. Obstet Gynecol 1992; 79: 856-859.

8. Paw P, Jamieson S. Surgical management of intrapericardial teratoma diagnosed in utero. Ann Thorac Surg 1997; 64: 552-554.

9. Bruch S, Adzick S, Reiss R, Harrison M. Prenatal therapy for pericardial teratomas. J Pediatr Surg 1997; 32: 1113-1115.

10. Tollens T, Casselman F, Devlieger H, Gewillig M, Vanderberghe K, Lerut T, Daenen W. Fetal cardiac tamponade due to an intrapericardial teratoma. Ann Thorac Surg 1998; 66: 559-560.

11. Riskin-Mashiah S, Moise J, Wilkins I, Ayres N, Fraser C. In utero diagnosis of intrapericardial teratoma: a case for in utero open fetal surgery. Prenat Diagn 1998; 18: 1328-1330.

12. Daniels C, Cohen D, Phillips J, Rowland D. Prenatal detection of a pericardial teratoma. Circulation 1999; 99: 1-2.

13. Fujimori K, Honda S, Akutsu H, Ariga H, Ujiie N, Yanagida S, San A. Prenatal diagnosis of intrapericardial teratoma: a case report. J Obstet Gynaecol Res 1999; 25: 133-136.

14. Sklansky M, Greenberg M, Lucas V, Gruslin-Giroux A. Intrapericardial teratoma in a twin fetus: diagnosis and management. Obstet Gynecol 1997; 89: 807-809.

15. Perez-Aytes A, Sanchís N, Barbal A, Artés A, Domenes J, Chirivella M, Baamonde A. Non-immunological hydrops fetalis and intrapericardial teratoma: case report and review. Prenat Diagn 1995; 15: 859-863.

16. Lindner V, Gasser B, Favre R, Kohler M, Vetter J. Anasarque foeto-placentaire et tératome intra-péricardique foetal. À propos d'un cas de diagnostic anténatal. Ann Pathol 1999; 19: 131-134.

17. Valioulis I, Aubert D, Lassauge F, Slimane M. Intrapericardial teratoma diagnosed prenatally in a twin fetus. Pediatr Surg Int 1999; 15: 284-286.

18. Bovea C, Fernandez G, Matallín P, Bernabéu C, Casanova M, Martinez J. Diagnóstico prenatal de un teratoma intracardíaco: a propósito de un caso. Prog Diag Prenat 2000; 12: 74-78.

19. Bustamante T, Azpeitia J, Miralles M, Jiménez M, Santos-Briz A, Rodríguez-Peralto J. Prenatal sonographic detection of pericardial teratoma. J Clin Ultrasound 2000; 28: 194-198.

20. Pratt J, Cohen D, Mutabagani K, Davis T, Wheller J. Neonatal intrapericardial teratomas: clinical and surgical considerations. Cardiol Young 2000; 10: 27-31

21. Sepúlveda W, Gómez E, Gutierrez J. Intrapericardial teratoma. Ultrasound Obstet Gynecol 2000; 15: 547-548.

22. J. A. Ramírez ¹, C. R. Mon ¹, E. O. Perez ¹, B. D. Parugues ¹, G. R. Pascual.Ultrasound Obstet Gynecol 2004;23: 416-418

23. Suchet I. Unpublished data.