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Journal of Indian Association of Pediatric Surgeons
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Table of Contents   
CASE REPORT
Year : 2021  |  Volume : 26  |  Issue : 6  |  Page : 459-461
 

successful surgical management of a rare combination of intrapericardial teratoma and thymoma in an infant


1 Department of Cardiovascular and Thoracic Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Anesthesia, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission14-Jul-2020
Date of Decision15-Sep-2020
Date of Acceptance28-Oct-2020
Date of Web Publication12-Nov-2021

Correspondence Address:
Dr. Vidur Bansal
Department of Cardiovascular and Thoracic Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.JIAPS_259_20

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   Abstract 


Primary cardiac tumors are rare, accounting for <0.2% of all childhood tumors. They can be diagnosed prenatally. Intrapericardial teratoma is a rare benign tumor that presents either due to the mass effect of the tumor or secondary pericardial effusion. Thymus is an important part of the immune system in the pediatric age group. Thymic lesions are rare causes of anterior mediastinal pathology. Their occurrence in children is rarer, nevertheless knowledge about their pathologies helps in clinching the correct diagnosis. We report a case of combined intrapericardial teratoma and thymoma that has not been reported previously in the literature.


Keywords: Cardiac tumors, mediastinum, thymoma, tumor markers


How to cite this article:
Mishra AK, Bansal V, Patel R, Upadhyay V, Sekar A, Halder V, Singh A. successful surgical management of a rare combination of intrapericardial teratoma and thymoma in an infant. J Indian Assoc Pediatr Surg 2021;26:459-61

How to cite this URL:
Mishra AK, Bansal V, Patel R, Upadhyay V, Sekar A, Halder V, Singh A. successful surgical management of a rare combination of intrapericardial teratoma and thymoma in an infant. J Indian Assoc Pediatr Surg [serial online] 2021 [cited 2021 Dec 4];26:459-61. Available from: https://www.jiaps.com/text.asp?2021/26/6/459/330374





   Introduction Top


Intrapericardial teratoma is a rare benign tumor with an incidence of 5–6/100,000 children that usually causes cardiovascular and respiratory distress.[1] The first case was reported by Joel in 1890. Most intrapericardial teratomas are benign and may contain well-differentiated tissues. Complete surgical resection is the treatment of choice. Long-term follow-up with serial monitoring of alfa-fetoprotein (AFP) levels is required for possible recurrence, especially when the tumor is immature or when resection is incomplete.

Thymoma is a lympho-epithelial neoplasm characterized by the presence of epithelial cells and mature lymphocytes. Thymoma usually presents in the fourth to sixth decades of life.[2] Thymoma is extremely rare in children and adolescents, with <36 cases of pediatric thymic neoplasms registered over 12 years across four European nations.[3] As a result of this rarity, it is difficult to predict prognosis or formulate treatment guidelines for these patients.


   Case Report Top


A pregnant woman was referred to our institute for evaluation of a mediastinal mass detected in utero in the fetus. She was counseled for institutional delivery and a close follow-up of the baby. At birth, there was no respiratory distress and physical examination revealed no specific abnormality. Electrocardiogram showed sinus rhythm, right-axis deviation, and right ventricular hypertrophy. Chest X-ray [Figure 1]b revealed a large mediastinal mass predominantly on the right side. Immediate surgical intervention was deferred because of the immature myocardium and the clinically stable condition of the child. The child was followed up with imaging, echocardiography, and tumor marker levels until 4 months of age [Figure 1]c. Antenatal [Figure 1]a and postnatal echocardiography revealed a 2.7 cm × 2.2-cm multilocular mass in relation to the anterior aspect of the right atrium and aorta with normal biventricular function. Contrast-enhanced computed tomography (CT) chest revealed a 4.8 cm × 4.4 cm × 4.7-cm multiseptate cystic lesion in the prevascular space, predominantly on the right side, seem to be compressing the heart, with no differential attenuating lesion seen separately to suggest a secondary pathology. The thymic tissue was not separately visualized. AFP levels reduced from 3000 IU/mL (normal range: 0–5.8 IU/mL) on day 8 of life to 109 IU/ml at 4 months of age. Lactate dehydrogenase and beta-human chorionic gonadotropin levels were within the normal range. This patient was operated at 4 months of age via a median sternotomy. On opening the sternum, a large thymic mass (5 cm × 4 cm × 2.7 cm) was found which seemed suspicious as preoperative imaging had shown only one large intrapericardial mass. After excision of this thymic mass, the pericardium was incised as it was bulging, raising the suspicion of another intrapericardial mass. The second mass (4.5 cm × 4.5 cm × 3.5 cm) was found adherent to the ascending aorta and the main pulmonary artery and was excised [Figure 2]. Feeder vessels were seen arising from the right coronary artery and aorta. The mass was excised in toto and sent for histopathological analysis along with the thymic mass. During the entire procedure, cardiopulmonary bypass was kept as a standby. The child was discharged on postoperative day 6 in a stable condition. The child is doing well in the follow-up clinic. Histopathology revealed a mature teratoma and a Type-B1 thymoma.
Figure 1: (a) Antenatal two-dimensional echocardiography showing the anterior mediastinal mass; (b) preoperative chest X-ray showing widening of the mediastinum; (c) computed tomography scan showing the multiseptate cystic anterior mediastinal mass

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Figure 2: (a) Intraoperative picture showing thymoma; (b) intraoperative picture showing the intrapericardial teratoma; (c) microphotograph of the thymic mass showing densely packed, small, mature lymphocytes along with scattered, round-to-oval epithelial cells (single arrow) and occasional Hassall's corpuscles (double arrow) (microphotograph, H and E, ×200); (d) microphotograph of the mediastinal mass showing tissue comprising squamous epithelium, intestinal glands, respiratory epithelium, smooth muscle bundles, and glial tissue (microphotograph, H and E, ×100)

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   Discussion Top


Primary intrapericardial tumors are rarer (incidence: 5–6/100,000 children) than secondary involvement of the pericardium from neoplasms of other organs and hematological malignancies.[1] Teratomas correspond to 19%–25% of these tumors, and most of these are mature type.[3] Intrapericardial teratomas arise from the base of the heart and are attached to the root of the pulmonary artery and aorta. In fetal life, the effusion or mass effect may cause extrinsic compression on the heart and cardiac tamponade. Rupture of the cyst in the pericardium due to the multicystic nature of pericardial teratomas leads to the development of effusions in the pleural and pericardial spaces. Diagnosis may be feasible in utero with prenatal echocardiography, which helps in early management. CT is advocated to delineate the tumor and its relation with other mediastinal structures. Teratoma is commonly classified using the Gonzalez–Crussi histological grading system or by their content (solid, cystic, and mixed).[4] Most intrapericardial teratomas are benign and may contain well-differentiated tissues. Complete surgical resection is the treatment of choice, first done by Beck in 1938. Long-term follow-up is required for possible recurrence, especially when the tumor is immature or when resection is incomplete. Chemotherapy and radiotherapy are not very useful in teratoma. Serial monitoring of the AFP levels is a useful marker for early diagnosis of recurrence of the tumor.[5]

In children, thymic lesions comprise only 2%–3% of all mediastinal tumors, with thymomas being still rarer (<1%).[2] Approximately 20% of the children with thymomas have associated paraneoplastic syndromes, of which around 70% are myasthenia gravis. The WHO system, recently updated in 2015, classifies thymoma into two major types – Types A and B. Type B thymomas are further sub-classified into B1, B2, and B3.[6] Surgical resection is the mainstay of treatment for epithelial lesions of the thymus, with chemo-radiotherapy being reserved for invasive and malignant diseases. In children, treatment protocols have not been clearly defined due to paucity of cases; however, most authors have favored regimens similar to adult regimens.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Laforgia N, Calderoni G, Di Mauro A, Marzullo A, Troise D, Annecchino P. A case of neonatal intrapericardial teratoma. Clinical and pathological findings. Acta Paediatr 2011;100:90-1.  Back to cited text no. 1
    
2.
Weissferdt A, Kalhor N, Bishop JA, Jang SJ, Ro J, Petersson F, et al. Thymoma: A clinicopathological correlation of 1470 cases. Hum Pathol 2018;73:7-15.  Back to cited text no. 2
    
3.
Stachowicz-Stencel T, Orbach D, Brecht I, Schneider D, Bien E, Synakiewicz A, et al. Thymoma and thymic carcinoma in children and adolescents: A report from the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT). Eur J Cancer 2015;51:2444-52.  Back to cited text no. 3
    
4.
Harms D, Zahn S, Göbel U, Schneider DT. Pathology and molecular biology of teratomas in childhood and adolescence. Klin Padiatr 2006;218:296-302.  Back to cited text no. 4
    
5.
Bader D, Riskin A, Vafsi O, Tamir A, Peskin B, Israel N, et al. Alpha-fetoprotein in the early neonatal period A large study and review of the literature. Clin Chim Acta 2004;349:15-23.  Back to cited text no. 5
    
6.
Marx A, Chan JK, Coindre JM, Detterbeck F, Girard N, Harris NL, et al. The 2015 World Health Organization classification of tumors of the thymus: Continuity and changes. J Thorac Oncol 2015;10:1383-95.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2]



 

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