| Abstract|| |
Intrapericardial teratoma is a germ-cell tumor that typically arises from the base of the heart and usually diagnosed in the fetal or neonatal period. Although benign, these tumors can be massive in size causing direct compression of the heart. Life-threatening complications such as fetal hydrops, cardiac failure, superior vena cava syndrome, and cardiac tamponade caused by these teratomas have been reported. Early surgical excision is curative. We present the images of a mature intrapericardial teratoma diagnosed in an asymptomatic neonate. The neonate was managed successfully by elective surgical excision.
Keywords: Germ-cell tumor, intrapericardial teratoma, surgical excision, two-dimensional echocardiography
|How to cite this article:|
Arvind A, Rajeshkumar R, Thakur D, Sridhar A, Sivaprakasm MC. Successful management of a neonate with antenatally detected mature intrapericardial teratoma. Ann Pediatr Card 2019;12:182-4
|How to cite this URL:|
Arvind A, Rajeshkumar R, Thakur D, Sridhar A, Sivaprakasm MC. Successful management of a neonate with antenatally detected mature intrapericardial teratoma. Ann Pediatr Card [serial online] 2019 [cited 2019 May 20];12:182-4. Available from: http://www.annalspc.com/text.asp?2019/12/2/182/257405
| Introduction|| |
Intrapericardial teratomas are one of the rare primary cardiac tumors in children. Teratomas are of embryonic origin, composed of multiple cell types derived from endodermic, mesodermic, and neuroectodermic germinal layers in varying degrees. They range from benign, well-differentiated (mature) cystic lesions to those that are solid and malignant (immature). The intrapericardial teratomas are usually detected in utero or in the neonatal period. Although benign, they may cause large pericardial effusion/tamponade and cardiac compression. Surgical excision is considered curative. We report the successful surgical management of a case of mature intrapericardial teratoma in a newborn, which was diagnosed prenatally.
| Clinical Summary|| |
A 7-day-old term female neonate with birth weight of 2.8 kg was evaluated for a large intrapericardial mass compressing the heart, detected antenatally by fetal echocardiography. Infant was asymptomatic. She was hemodynamically stable, and there were no signs of heart failure. The chest was clear. Heart sounds were normal and there was no murmur. Chest roentgenogram showed homogenous opacity occupying the entire right hemithorax. The right heart border could not be visualized. Electrocardiogram showed sinus rhythm. Echocardiography showed a heterogeneous intrapericardial multicystic mass, measuring 51 mm × 34 mm, adjacent to the right atrium, with mild pericardial effusion [Figure 1]. The tumor was externally compressing the superior vena cava and right atrium. Doppler interrogation showed unobstructed superior vena cava flow. Other right-sided structures including the inferior vena cava, tricuspid valve, right ventricle, pulmonary valve, and pulmonary arteries were not involved. The tumor was seen adjacent to the ascending aorta, but there was no evidence of compression of the aorta. It was a structurally normal heart with good biventricular function. Computed tomography (CT) chest confirmed the presence of an intrapericardial multiloculated cystic mass (57 mm × 41 mm × 42 mm) on the right side of the heart, adherent to right atrium, ascending aorta, superior vena cava, and right pulmonary artery [Figure 2].
|Figure 1: Echocardiographic subcostal four-chamber view (a) demonstrating a heterogeneous mass (arrow) with multiple hypolucent cystic areas compressing the right atrium, with mild pericardial effusion. Unobstructed superior vena cava flow on color Doppler (b). The tumor mass measures 5 cm × 3.5 cm on subcostal coronal view (c). LV: Left ventricle, RV: Right ventricle, LA: Left atrium|
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|Figure 2: (a and b) Computed tomography of the chest shows an intrapericardial mass (star) on the right side of the heart, adherent to the ascending aorta (arrow), and compressing the right atrium|
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The infant was taken for elective surgical excision of the tumor through median sternotomy without cardiopulmonary bypass. Large amount of straw-colored serous fluid was drained after pericardiotomy. Dissection around the tumor revealed a large, lobulated well-encapsulated mass with clean planes, arising from and adherent to the ascending aorta adventitia on the right side of the heart and compressing the right atrium [Figure 3]. During resection from the aorta, there was an inadvertent intimal-medial tear, leading to profuse bleeding. Emergency cardiopulmonary bypass was thus initiated, and complete surgical resection of the tumor was done successfully.
|Figure 3: (a) Gross pathological specimen of the bosselated intrapericardial mass measuring about 6 cm in greatest diameter, with a smooth external surface. Cystic areas are seen. (b) The intraoperative image of the tumor adherent to the ascending aorta|
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Gross examination of the tumor specimen showed a pale white cystic tissue measuring 6 cm × 5 cm × 4 cm, with a smooth external surface [Figure 3]a. Cut section showed multiple thin-walled cystic spaces, filled with clear fluid. No solid areas were seen. Histopathological examination revealed cystic spaces lined by columnar cells and focal squamous metaplasia. The adjacent stroma was composed of fibrous tissue, smooth muscle bundles, pancreatic tissue, and mature neuroglial tissue, suggestive of mature intrapericardial teratoma [Figure 4].
|Figure 4: Photomicrograph showing (a) mature glandular tissue (yellow arrow) and (b) mature neural tissue in rosettes (blue arrow) (H and E, ×10 and × 40, respectively)|
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The neonate was extubated within 6 h after surgery and started on feeds on the same day. Postoperative echo showed no residual mass and good biventricular function. There was a mild turbulence in the left ventricular outflow tract on color Doppler (mean gradient 12 mmHg). The infant was discharged on the 4th postoperative day. At follow-up after 3 months, the neonate was clinically stable; echo showed no evidence of recurrence and good biventricular function.
| Discussion|| |
Intrapericardial teratoma is a single, encapsulated multicystic tumor of embryonic origin, consisting of tissues derived from all the three germinal layers. As per Gonzalez–Crussi histopathological grading system, a mature teratoma (Grade 0) is benign and well-differentiated. These tumors are frequently diagnosed in utero or in newborn period. The intrapericardial teratoma is most frequently right sided, often firmly attached to the root of the aorta or pulmonary artery by a pedicle. Sometimes, the tumor capsule itself can be firmly attached to the aorta or to the pulmonary artery adventitia. Furthermore, the tumor blood supply usually emanates as nutrient vessels from the aortic vasa vasorum. Thus, there is a small risk of massive hemorrhage from the aorta during dissection. Surgical excision on cardiopulmonary bypass should, therefore, be considered.
Often wedged between the aorta and superior vena cava, the intrapericardial teratomas have a propensity to severely obstruct the superior vena cava, pulmonary artery, and ascending aorta, and may compress the right atrium and right ventricle. They may cause cardiac decompensation due to pericardial effusion and cardiac compression. On echocardiography, the tumor appears as a single, nonhomogeneous, lobular, intrapericardial mass, often associated with pericardial effusion. Cystic formations appear as echolucent areas, calcifications as echogenic foci. Compression of the great vessels and intracardiac chambers may be seen. Additional imaging like CT chest has the advantage in defining the relationship of the tumor to adjacent structures, including great vessels. Histopathological examination confirms the diagnosis.
Intrapericardial teratomas are rarely malignant or recurrent, particularly in infants and newborns. Surgical excision is the treatment of choice. Successful long-term results have been reported following surgery.
This is one of the few case reports of a neonatal intrapericardial teratoma from India., In retrospect, we believe, that although benign, urgent resection of the tumor immediately after diagnosis improves the outcome. In addition, in view of risk of massive hemorrhage for adherent tumors, surgical excision should be done under cardiopulmonary bypass to avoid catastrophic bleeding.
Declaration of the patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for the child's images and other clinical information to be reported in the journal. The guardian understands that the child's name and initials will not be published and due efforts will be made to conceal patient's identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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Dr. Annie Arvind
Apollo Children's Hospital, Thousand Lights, Chennai - 600 006, Tamil Nadu
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4]