Ann Thorac Surg 2004;78:e6-e8
© 2004 The Society of Thoracic Surgeons
Case report
Immature intrapericardial teratoma in a newborn with elevated alpha-fetoprotein
Nathalie Roy, MDa,
Dominic J. Blurton, MDb,
Anthony Azakie, MDa,
Tom R. Karl, MD*a
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
 |
Abstract
|
|---|
A large intrapericardial mass was incidentally found in a fetus at 32 weeks gestational age, which was accompanied by a significant pericardial effusion. The baby was delivered by cesarean section and had surgical removal of the tumor on the second day of life. Alpha-fetoprotein was elevated. The pathologic analysis demonstrated a grade I immature teratoma with foci of hepatoid tissue. The patient had an uneventful postoperative recovery. A review of the literature and pathologic implications are discussed.
|
Introduction
|
|---|
Teratomas are tumors that present in infants and children as well as adults. Pediatric cases are usually found in the sacrococcygeal region, in gonads, in the mediastinum, and intracranially. Intrapericardial teratomas are rare and are usually diagnosed in the neonatal period or in utero. They are associated with large pericardial effusions, hydrops, and tamponade.
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.
View larger version (86K):
[in this window]
[in a new window]
|
Fig 1. Subcostal echocardiogram demonstrating a large multilobulated intrapericardial mass (T) attached to the ascending aorta (Ao), and adjacent to the right ventricle (RV). A large pericardial effusion is also present (PE). The left ventricle is also identified (LV).
|
|
Pathologic examination of the specimen revealed an encapsulated, multilobulated mass measuring 4.5 x 2.5 x 2.5 cm, consisting mostly of mature teratomatous elements with a prominent glial component (Fig 2).
Scattered foci of immature neural epithelial tissue were present, consistent with a grade I immature teratoma. Immature mesenchymal elements, endometrioid glands with subnuclear vacuoles and foci of hepatoid tissue positively staining for 
-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.
View larger version (75K):
[in this window]
[in a new window]
|
Fig 2. Gross pathologic specimen of intrapericardial mass consisting of multiple cystic areas. The mass was attached to the adventitia of the aorta and was completely resected using electrocautery.
|
|
|
Comment
|
|---|
Intrapericardial teratomas were first described by Joel [1] in 1890, and to date there have been approximately 60 cases reported in the literature. These tumors are usually detected in the neonatal period or in utero and have exceptionally been diagnosed in older children [2]. They are usually associated with large pericardial effusions caused by a rupture of cysts and obstruction of venous return, pleural effusions, ascites, and hydrops. There may be severe respiratory distress and hemodynamic compromise [3–6]. The diagnosis is usually made by echocardiogram, which typically shows the mass pedunculated from the aorta projecting anterior and to the right side of the heart. The use of computed tomography and magnetic resonance imaging is advocated to define the tumor and its relation with the heart, and to assess the mediastinum, although the presence of a second tumor is very rare [5, 7].
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.
|
References
|
|---|
- Joel V.J. Ein teratoma auf der arteria pulmonalis innerhalb des herzbeutels. Anatomie 1890;122:381.
- Ertugrul T., Dindar A., Elmaci T.T., Kilicaslan I. An intrapericardial teratoma with endocrine function. J Cardiovasc Surg (Torino) 2001;42:781-783.[Medline]
- Guirgis N.M., Azancot A.B., Blot P. Unusual evolution of pericardial mass diagnosed by fetal echocardiography. Perdiatr Cardiol 1991;12:57-59.
- 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;79:856-858.[Medline]
- Tollens T., Casselmann F., Devlieger H., et al. Fetal cardiac tamponade due to an intrapericardial teratoma. Ann Thorac Surg 1998;66:559-560.[Abstract/Free Full Text]
- Riskin-Mashiah S., Moise K.J., Jr, Wilkins I., Ayres N.A., Frasier C.D., Jr In utero diagnosis of intrapericardial teratoma: a case for in utero open fetal surgery. Prenat Diagn 1998;18:1328-1330.[Medline]
- Beghetti M., Prieditis M., Rebeyka I.M., Mawson J. Images in cardiovascular medicine. Intrapericardial teratoma. Circulation 1998;97:1523-1524.[Free Full Text]
- Laquay N., Ghazouani S., Vaccaroni L., Vouhé P. Intrapericardial teratoma in newborn babies. Eur J Cardiothorac Surg 2003;23:642-644.[Abstract/Free Full Text]
- Heifetz S.A., Cushing B., Giller R., et al. Immature teratomas in children: pathologic considerations. A report from the combined pediatric oncology group/children's cancer group. Amer J Surg Pathol 1988;22:1115-1124.
- Norris H.J. Immature ovarian teratomas in children. Amer J Surg Pathol 1999;23:1160-1161.
- Hawkins E.P. Reply. Letter to the editor. Amer J Surg Pathol 1999;23:1161-1162.
- Norris H.G., Zirkin H.J., Benson W.L. Immature (malignant) teratoma of the ovary: a clinical and pathologic study of 58 cases. Cancer 1976;37:2359-2372.[Medline]
- Malogolowkin M.H., Ortega J.A., Krailo M., et al. Immature teratomas. Identification of patients at risk for malignant recurrence. J Natl Cancer Inst 1989;81:870-874.[Abstract/Free Full Text]
- Tsuchida Y., Endo Y., Saito S., Kaneko M., Shiraki K., Ohmi K. Evaluation of alpha-fetoprotein in early infancy. J Pediatr Surg 1978;13:155-156.[Medline]
- Ward AM. Biological markers of germ cell tumors: the current state of the art (abstract). Nineteenth Annual Meeting of the International Society of Pediatric Oncology. Jerusalem, Israel, September, 1987:13–8
- Mann JR, Pearson D, Barrett A, et al. UKSCCSG Malignant germ cell tumor studies: the value of serum AFP and HCG monitoring (abstract). Nineteenth Annual Meeting of the International Society of Pediatric Oncology. Jerusalem, Israel, September 1987:13–8
- Carachi R., Campbell P.E., Chow C.W., Mee B.B. Alpha-feto-protein-(AFP)-secreting intra-pericardial teratoma-report of a case diagnosed on CT scanning. Z Kinderchir 1986;41:369-370.[Medline]
- Billmire D., Vinocur C., Rescoria F., et al. Malignant mediastinal germ cell tumors: an intergroup study. J Pediatr Surg 2001;36:18-24.[Medline]
Top
Abstract
Introduction
