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Ann Thorac Surg 2005;79:e13-e14
© 2005 The Society of Thoracic Surgeons

Case report

A Bronchogenic Cyst of the Right Ventricular Endocardium

Malte Weinrich, MDa,*, Henning F. Lausberg, MDb, Stefan Pahl, MDc, Hans-Joachim Schäfers, MD, PhDb

a Department of Abdominal, Visceral and Vascular Surgery, University Hospital of the Saarland, Homburg/Saar, Germany
b Department of Thoracic and Cardiovascular Surgery, University Hospital of the Saarland, Homburg/Saar, Germany
c Institute of General and Special Pathology, University Hospital of the Saarland, Homburg/Saar, Germany

Accepted for publication September 17, 2004.

* Address reprint requests to Dr Weinrich, Department of Abdominal, Visceral and Vascular Surgery, University Hospital of the Saarland, D-66421 Homburg/Saar, Germany (E-mail: chmwei{at}uniklinik-saarland.de).


   Abstract
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 Abstract
Introduction
 Comment
 References
 
In a 73-year-old male patient with a history of prostate cancer, a right ventricular endoluminal tumor was diagnosed by echocardiography. An endocardial papillary fibroelastoma or myxoma appeared possible; a malignant tumor could not be ruled out. The tumor was resected using extracorporeal circulation and cardioplegic arrest. Histopathology study revealed a bronchogenic cyst with ciliated epithelium.


   Introduction
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 Abstract
 Introduction
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 References
 
Tumors of the heart are rare and are commonly diagnosed postmortem. Whereas these tumors are most frequently metastases, primary cardiac tumors also occur [1, 2]. These are most often benign, and myxoma and papillary fibroelastoma are the most common [1–4]. The widespread use of echocardiography has increased the number of cardiac tumors diagnosed intra vitam during the last years.

Resection of apparently benign cardiac tumors has been recommended to avoid potential complications such as cardiac failure, dysrhythmias, conduction disturbances, syncope, sudden cardiac death, or embolization that can be prevented by early resection [2, 4]. Another rationale for resection of cardiac tumors is to rule out malignancy or diagnose a malignant tumor while it is still resectable.

A 73-year-old man with a history of prostate cancer managed surgically underwent echocardiography for mild dyspnea on exertion. Ventricular and valve functions were normal, and a mass within the right ventricular cavity was discovered incidentally. The tumor had a diameter of 10 mm and was attached to the right side of the interventricular septum (Fig 1). Coronary angiography as well as complete tumor staging did not reveal any abnormalities.



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  		Fig 1. Transthoracic echocardiographic four-chamber view showing the tumor in the right ventricle (LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle; Tu = tumor).

 
A papillary fibroelastoma seemed to be the most likely diagnosis. A metastasis of the known prostrate carcinoma appeared unlikely, but malignancy could not be ruled out. The decision was made to remove the tumor.

The chest was opened by median sternotomy, and extracorporeal circulation was initiated by aortic and bicaval cannulation. After cardioplegic arrest, the right atrium was opened and the right ventricle was inspected through the tricuspid valve. A pedunculated tumor arising from the interventricular septum could be seen. The tumor was removed completely, including the area of the septal wall to which the tumor was attached. Weaning from extracorporeal circulation was uneventful, and the patient was discharged on the fifth postoperative day after a regular postoperative course.

Macroscopically, the tumor consisted of a thin-walled cyst attached to the resected endomyocardium. The lumen contained a colorless, thread-forming fluid. Microscopically, the cyst lining was ciliated columnar epithelium with interspersed goblet cells, resembling ciliated respiratory epithelium, and the cyst wall contained fibrous connective tissue (Fig 2). Histology and immunohistochemistry studies excluded a relationship between the tumor and the prostate cancer.



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  		Fig 2. Hematoxylin & eosin stained photomicrograph (magnification x200) showing the cyst lining of ciliated columnar epithelium with interspersed goblet cells and surrounding fibrous connective tissue.

 

   Comment
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 Abstract
 Introduction
 Comment
References
 
The differential diagnosis of malignant cardiac tumors includes metastases and primary tumors like sarcomas. Metastases account for 99% of all malignant cardiac disorders [1], and their incidence depends on the type of primary cancer [5, 6]. Although prostate cancer is common, cardiac metastasis is rare [5, 6]. In addition, cardiac metastases are commonly localized in the myocardium and epicardium and only rarely occur in the endocardium [5, 6]. Thus, the possibility of a cardiac metastasis of the preexisting prostate cancer appeared unlikely but not impossible.

Primary cardiac tumors are mostly benign [1, 3, 4] and, using imaging procedures, the histology can often be predicted. Papillary fibroelastomas are typically pedunculated and arise from heart valves or chordae tendineae [1]. Myxomas mostly arise from the atrial septum [1, 3]. Chronic thrombus formation may resemble the appearance of a myxoma and has to be considered as a differential diagnosis. Primary malignant cardiac tumors are usually sarcomas, commonly located in the ventricular myocardium [1].

It is not unequivocally clear whether all cardiac tumors should be resected. The decision for operation is easy to make for symptomatic tumors, and asymptomatic left-sided tumors of the heart are often resected to prevent the risk of embolization. In the present case, we chose to remove this right-sided tumor for primarily diagnostic and potentially therapeutic reasons.

To our surprise, we found a bronchogenic cyst, which belongs to the inhomogenous group of heterotopias. Heterotopias are rare benign or malignant tumors of ectopic tissue misplaced during embryogenesis [1]. For the differentiation of heterotopias, the number of associated germ layers is of particular interest. Whereas teratomas consist of all three germ layers and cystic tumors of the atrioventricular node consist of only one, bronchogenic cysts contain both mesoderm and endoderm [2, 7]. Although in the present case no cartilage or smooth muscle cells but merely fibrous connective tissue was seen histologically, the right ventricular localization and the size of the pedunculated tumor correlate more with a bronchogenic cyst than with a cystic tumor of the atrioventricular node, which is usually only detected microscopically [7].

Intrapericardial bronchogenic cysts are rare and are located on the epicardial surface or within the myocardium projecting into one of the cardiac cavities [7]. In the only review dealing with a larger number of intracardiac bronchogenic cysts, the majority were found in the right side of the heart, as seen in the present case, and only a few were located in the left side or their localization was not specified [8].

In conclusion, bronchogenic cysts are part of the differential diagnosis of cardiac tumors. Resection allows a definitive diagnosis and should possibly be recommended for the low-risk patient.


   References
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 Abstract
 Introduction
 Comment
 References
 

  1. Burke A, Virmani R. Classification and incidence of cardiac tumorsIn: Rosai J, Sobin LH, editors. Atlas of tumor pathology, third series, fascicle 16, tumors of the heart and great vessels. Washington, DC: Armed Forces Institute of Pathology; 1995. pp. 1-11.
  2. Reynen K. Benigne Tumoren des Herzens Z Kardiol 1993;82:749-762.[Medline]
  3. Basso C, Valente M, Poletti A, Casarotto D, Thiene G. Surgical pathology of primary cardiac and pericardial tumors Eur J Cardiothorac Surg 1997;12:730-738.[Abstract]
  4. Perchinsky MJ, Lichtenstein SV, Tyers GFO. Primary cardiac tumors—forty year's experience with 71 patients Cancer 1997;79:1809-1815.[Medline]
  5. Karwinski B, Svedsen E. Trends in cardiac metastasis. APMIS 1989;97:1018–24..
  6. Klatt EC, Heitz DR. Cardiac metastases Cancer 1990;65:1456-1459.[Medline]
  7. Burke A, Virmani R. Heterotopias and tumors originating from ectopic tissuesIn: Rosai J, Sobin LH, editors. Atlas of tumor pathology, third series, fascicle 16, tumors of the heart and great vessels. Washington, DC: Armed Forces Institute of Pathology; 1995. pp. 111-125.
  8. Deenadayalu RP, Tuuri D, Dewall RA, Johnson GF. Intrapericardial teratoma and bronchogenic cystReview of literature and report of successful surgery in infant with intrapericardial teratoma. J Thorac Cardiovasc Surg 1974;67:945-952.[Medline]



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