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Ann Thorac Surg 2004;78:e6-e8
© 2004 The Society of Thoracic Surgeons
a Division of Pediatric Cardiothoracic Surgery, San Francisco, California, USA
b Division of Pediatric Cardiology, Children's Hospital, University of California, San Francisco, California, USA
Accepted for publication October 30, 2003.
* Address reprint requests to Dr Karl, Division of Pediatric Cardiothoracic Surgery, University of California San Francisco, 505 Parnassus Ave, Room S-549, San Francisco, CA, USA 94143-0118
e-mail: karlt{at}surgery.ucsf.edu
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Introduction |
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A 35-year-old gravida mother presented in week 32 of her pregnancy with ruptured membranes after a previously uneventful pregnancy and a normal first ultrasound. At presentation the fetal ultrasound demonstrated a large pericardial effusion and a cystic mass near the atrium. Ascites was present but the fetus was not hydropic. After administration of corticosteroids, the baby was delivered by cesarean section and intubated. On arrival to our institution, examination of the infant revealed a well perfused and nondysmorphic female weighing 2,180 g. She had no heart murmur. There was a distended abdomen without significant hepatomegaly and she had mild peripheral edema. Her chest roentgenogram showed a small aerated right thorax and a very large cardiac shadow occupying the mediastinum and left hemithorax, but despite that she was easy to ventilate and required no inotropic support. Her echocardiogram demonstrated a large cystic intrapericardial mass to the right of the right atrium with an attachment to the ascending aorta. A large pericardial effusion was present without signs of tamponade (Fig 1). She was brought to the operating room on the second day of her life. A median sternotomy was performed. After removal of the thymus a large amount of serous fluid was drained from the pericardial sac. The intrapericardial mass was cystic and measured 4.5 cm in length with its base attached to the adventitia of the ascending aorta above the right coronary artery. The mass was completely removed using electrocautery. Excess pericardium was removed and partially closed, followed by sternal closure. The infant was extubated 12 hours after surgery and had an uneventful recovery.
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-fetoprotein were also found. The -fetoprotein level was 91,853 µg/L (normal level, < 6.4), but the level decreased to 66,000 µg/L 2 weeks after surgery. A computed tomographic scan of the chest after resection demonstrated no recurrence of pericardial fluid or residual tumor. The ascites had resolved and no other significant findings were noted in the chest, abdomen, or brain. She was discharged to the referring institution for continuation of her neonatal care and is followed-up with serial computed tomographic scans and measures of serum -fetoprotein. The baby is now growing and developing appropriately. The echocardiogram shows normal cardiac and mediastinal anatomy 6 months postoperatively. The -fetoprotein levels fell to 265, 225, 171, and 139 µg/L at intervals of 3, 4, 5, and 6 months postoperatively, respectively.
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A rapidly growing mass can develop from 20 to 40 weeks of gestational age and this represents the time of detection for approximately one third of the cases reported. A fetus presenting early in the second trimester with an effusion and hydrops is a complex problem because of prematurity; some centers have successfully preformed transabdominal pericardiocentesis and open fetal surgery has been suggested [6] in the case of a severely compromised fetus [4, 8]. In most cases presenting in the third trimester, a cesarean section is performed preoperatively and surgery takes place on a semi-urgent basis. Pericardiocentesis may be performed, although this can result in an acute decompensation and circulatory collapse secondary to the obstruction of the venous return by the large mass once the pericardial fluid is removed.
The surgical procedure for removal of an intrapericardial teratoma is usually uncomplicated. The mass is rarely associated with an intracardiac condition and removal does not necessitate cardiopulmonary bypass. The mediastinum should be explored to ascertain the absence of another teratoma [5]. Once resected, the prognosis is usually good, as most tumors are benign and require no further treatment.
Teratomas typically contain all three germ cell layers (ie, endodermic, mesodermic, and neuro-ectodermic). There is controversy regarding the grading of immature elements in teratomas in children [9–11]. Typically the grade has been related to the maturity of the neuroepithelial tissue, but foci of yolk sac tumor (YST) has also been recognized as a prognostic factor for recurrence [12, 13]. Microscopic foci of YST can present as a hepatoid pattern, but this well-differentiated form is almost always associated with typical YST in the same specimen [9] in distinction to the findings in our patient who did not exhibit typical YST. Elevated -fetoprotein levels are seen with YST, and immature teratomas are associated with poor prognosis and a risk for recurrence, although the significance of an elevated -fetoprotein level is questionable in the first year of life. The AFP level is close to 100,000 µg/L at birth and decreases after an inverse logarithmic curve to approximately 100 µg/L at 1 month of life, and then to a normal level at 1 year of age [14]. It has been suggested that the rapidity of -fetoprotein decay after resection is a more accurate prognostic factor in immature teratomas than any isolated value [15, 16].
We found two prior case reports of intrapericardial immature teratoma. One baby presented at 3 months of age with respiratory distress, an elevated -fetoprotein level, and a very large intrapericardial tumor that was completely excised. The pathologic diagnosis consisted of an immature teratoma with YST elements. One year after surgical resection the infant was free of recurrence [17]. The other patient was a fetus that died in utero of rapid progression of tumor and hydrops. Again, the pathologic examination demonstrated a grade III immature teratoma with YST elements [6].
Immature teratomas of different anatomical location in children have been reviewed in a few series and there were only one or two cases of intrapericardial teratomas that were present in each series [10, 13, 18]. These tumors have a low risk of recurrence and most centers would not recommend adjuvant treatment after resection.
In conclusion, intrapericardial teratomas are rare tumors that usually present in the fetal or neonatal period. Urgent intervention for hemodynamic reasons is usually required, but the prognosis after surgery is good, even in the presence of immature elements.
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This article has been cited by other articles:
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  I. Manoly, N. Viola, D. Fowler, K. Roman, and M. Haw Intrapericardial Teratoma in Neonates: A Surgical Emergency World Journal for Pediatric and Congenital Heart Surgery, April 1, 2011; 2(2): 321 - 323. [Abstract] [Full Text] [PDF]
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 G. M. Iacona, M. A. Barber, M. Medina, and R. Abella Intrapericardial teratoma in a low birth weight preterm infant: a successful multidisciplinary approach Interact CardioVasc Thorac Surg, February 1, 2011; 12(2): 287 - 289. [Abstract] [Full Text] [PDF]
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