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Intrapericardial Teratoma

A predominantly solid mass (arrow) is seen within the thorax, associated with a large pericardial effusion

The heart (H) is compressed by the adjacent mass. Several small cystic areas were seen within the mass (small arrow).

Findings:

The sonogram demonstrates a mass within the thorax associated with a large pericardial effusion. The predominanly solid mass contains several small cystic areas and was intimately associated with the heart anteriorly.

 

Discussion:

The intrathoracic mass represents an intrapericardial teratoma, an uncommon primary cardiac tumor occuring in a fetus. The incidence of primary cardiac tumors varies between 0.0017 and 0.027% of all live births. Most are rhabdomyomas (60%), teratomas (20%) and fibromas (12%). Cardiac hemangiomas comprise about 2.8% of all primary cardiac tumors. Myxomas have never been found in fetuses.

Intrapericardial teratomas are rare lesions accounting for approximately 10% of all mediastinal masses in children. The tumor arises from multipotential cells belonging to the three germinal layers. In contrast to ovarian teratomas, intrapericardial teratomas usually contain no hair, sebaceous glands or squamous epithelium, although calcifications are reported. Intrapericardial teratomas are almost always pedunculated, with attachment to the aortic root or pulmonary vessels and are invariably associated with a pericardial effusion. . Most are juxtoposed to the right side of the heart causing vascular compression with growth but left-sided teratomas have been reported.

In fetal life, the effusion or mass effect may cause extrinsic compression on the heart and cardiac tamponade. It can also impede venous return with resultant hydrops fetalis and fetal death. After delivery, ventilation may be compromised by extrinsic airway compression or pulmonary hypoplasia and cardiac output may be compromised by mass effect or tamponade. Teratomas arise from the pericardium and are of mixed echogenicity with cystic areas or calcifications and almost all are associated with pericardial effusion. They may demonstrate increased vascularity on Doppler interrogation. Sonographically, rhabdomyomas appear as nodular masses in the atrial or ventricular myocardium and are usually multiple. Hemangiomas are of mixed echogenicity arising from the pericardium, myocardium or ventricular cavity. Fibromas are usually solitary masses in the ventricular myocardium or septum and occasionally associated with calcification. An osteoma appears as a solitary, sharply defined echogenic mass in the ventricular septum.

 

Color Doppler image demonstrating increased vascularity within the teratoma

The case above presented with hydrops, with scalp and body wall edema, ascites and a pericardial effusion.

When a fetus with non-immune hydrops and a thoracic mass is identified, the differential diagnoses of cystic adenomatoid malformations, extralobar pulmonary sequestration, as well as cardiac tumors should be considered. Cardiac tumors such as fibromas, rhabdomyomas, mesotheliomas and hemangiomas must be considered if the mass originates within the cardiac chambers or where a discrete border between the mass and myocardium cannot be visualized. Pathologically, calcifications and cystic areas within the tumor are common in intrapericardial teratomas. Atrial myxomas are mobile and usually arise from the left atrium and are not seen prenatally. Hemangiomas can arise from anywhere in the vicinity of the heart. The most common origin is the base of the heart adjacent to the right atrium. Obvious amounts of pericardial effusion are usually present.There are presently over a dozen cases in the English literature of intrapericardial teratomas diagnosed in utero. Cases diagnosed near term without signs of hydrops fetalis can be followed closely with surgery after delivery. When an intrapericardial teratoma is diagnosed with a large pericardial effusion that causes impending cardiac tamponade, in utero pericardiocentesis has been sucessful in salvaging the fetus.

 

References:

Sklansky M, Greenberg M, Lucas V, Gruslin-Giroux A. Intraperiardial teratoma in a twin fetus: Diagnosis and management Obstet Gynecol 89:807-9, 1997

Riskin-Mashiah S, Moise Jr. KJ, Wilkins I et al. In Utero diagnosis of intrapericardial teratoma: A case for in utero open fetal surgery. Prenatal Diagnosis 18:1328-1330, 1998

Benatar A, Vaughan J, Nicolini U, Trotter S et al. Prenatal pericardiocentesis: Its role in the management of intrapericardial teratoma. Obstet Gynecol79:856-859, 1992

Cyr DR, Guntheroth WG, Nyberg DA, Smith JR et al. Prenatal diagnosis of intrapericardial teratoma: A cause for nonimmune hydrops. J Ultrasound Med 7:87-90, 1988

Heath D. Pathology of cardiac tumors. Am J Surgery 94:183, 1957

Reynolds JL, Donahue JK, Devwall RA et al. Intrapericardial teratoma. A cause of acute pericardial effusion in infancy. Pediatrics 43:71, 1969

Banfield F, Dick M, Behrendt DM et al. Intrapericardial teratoma: A new and treatable cause of hydrops fetalis. Am J Dis Child 134:1174, 1980

Fung TY, Lau TK, Yam MC, Fai To K. Prenatal diagnosis of cardiac osteoma: A case report. Prenatal Diagnosis 18:1209-1211, 1998

Holley DG, Mrtin GR, Brenner JI, Fyfe DA et al. Diagnosis and management of fetal cardiac tumors: A multicenter experience and review of published reports. J Am College Cardiol. 26:516-520, 1995

Samltz A, Apitz J. Primary heart tumors in infancy and childhood (report of 4 cases and review of literature) Cardiology 67:12-16, 1981

Tseng JJ, Chou MM, Lee YH, Ho ESC. In utero diagnosis of cardiac hemangioma. Ultrasound Obstet Gynecol 13:363-365, 1999

Fujimori K, Honda S, Akutsu H, Ariga H et al. Prenatal diagnosis of intrapericardial teratoma: A case report. J Obstet Gynaecol Res. 25:133-6, 1999

 

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Peter W. Callen, M.D.
Professor of Radiology, Obstetrics, Gynecology and Reproductive Science
University of California Medical Center, San Francisco, California