Intrapericardial Paraganglioma

6. FarkouhM,DangasG,LeonM,SmithC,NestoR,BuseJB,etal. Design for the Future Revascularization Evaluation in patients with Diabetes mellitus: Optimal management of Multivessel disease (FREEDOM) trial. Am Heart J. 2008;155:215-33. 7. Serruys PW, Morice MC, Kappetein P, Colombo A, Holmes DR, Mack M, et al. Percutaneous coronary intervention versus coronary-artery bypass for severe coronary artery disease. N Engl J Med. 2009;360:961-72.


Intrapericardial Paraganglioma
To the Editor, Pheochromocytomas are tumours producing catecholamines, released mainly by secreting chromaffin cells originating from embryonic neurectodermal cells. Extra-adrenal tumours are also called paragangliomas, and there are 2 types. The first are chemodectomas, which are negative chromaffin tumours originating in the parasympathetic chain that are often not functioning in their intrapericardial location. 1 The second are positive chromaffin pheochromocytoma from the sympathetic nervous system. Although these tumours are a cause of hypertension in the general population, their surgical removal may cure this. Its incidence in the general population is between 0.001% and 0.01%. 2 Thoracic paragangliomas comprise only 1%-2% of cases of pheochromocytomas and are normally located in the posterior mediastinum. 3 Intracardiac or intrapericardial pheochromocytomas are extremely rare, with no more than 50 reported cases. They are usually located in the left atrium, although they have been found in the right atrium, the atrial septum or, as in our case, at the level of the aortic root in front of the left atrium and in the immediate vicinity of the coronary ostia, 4,5 in which case they may be painful. 6 The diagnosis and exact location of the tumour is crucial for planning surgery.
We report the case of a 38-year-old woman, previously operated on for breast fibroadenoma which was the only incident in her background, who attended the emergency department for a respiratory infection. Her chest x showed she had an impaired cardiac silhouette. The physical, laboratory and electrocardiogram examinations were normal. The echocardiogram ( Figure 1) showed a mass of 4 cm in diameter below the pulmonary artery and at the level of the interventricular sulcus from the left atrium to the aorta (marked with a dot on the figure). A chest computed tomography (CT) was performed ( Figure 2), which showed a heterogeneous lesion of 4×4 cm at the level of the heart, above the left ventricle and connected to the pericardium. The possibilities of pericardial mass were considered (teratoma, mesothelioma lipoma or melanin tumour). Magnetic resonance imaging ( Figure 2) confirmed these findings, as well as showing that it was hyperintense with contrast, with signs of vascularisation. The patient underwent thoracic surgery with resection of the intrapericardial mass and pulmonary artery enlargement with a pericardial patch. Positron emission tomography (PET) was performed, which showed no other lesions. The pathology  We report the case of a male aged 19, a native of Equatorial Guinea with typhoid and malaria as the only relevant history. He arrived in Spain 18 months earlier and since then has had dyspnoea, chills, joint pain, fatigue and palpitations. For this reason, he came to the emergency room of our hospital and was admitted to cardiology for study.
The patient was tachycardic and had an elevated jugular venous pressure. Auscultation revealed a third tone, a pansystolic III/IV murmur in the right sternal border and hypoventilation in both lung bases. He presented 6 cm pulsatile hepatomegaly and bilateral pitting oedema to the roots of both thighs.
Tests were normal. The electrocardiogram showed a common atrial flutter with a ventricular response of 100 beats/min. Chest radiography revealed cardiomegaly at the expense of right cavities and bilateral pleural effusion.
An echocardiogram was performed which showed a striking dilation of the right atrium and obliteration of the apex and inlet tract of the right ventricle with severe infundibular dilation. The tricuspid valve was in its normal position, but with thickening and retraction of its leaflets ( Figure 1A). Colour Doppler showed severe tricuspid regurgitation ( Figure 1B). The left cavities were normal.
A catheterisation was conducted, which revealed increased right ventricular pressure (34/21 mm Hg, with dip-plateau morphology) and the right atrium (18 mm Hg). The right ventriculography confirmed the obliteration of the apex and body of the right ventricle, severe tricuspid regurgitation and aneurysmal dilatation of the right atrium. Coronary arteries were normal.
Cardiac magnetic resonance confirmed the findings described by the above techniques and showed enhancement at the apex of the right ventricle, compatible with fibrotic tissue (Figure 2).
Given these findings, we considered the following diagnostic possibilities: 1. Ebstein's disease: does not meet the echocardiographic diagnostic criteria, since there is not a 2 cm displacement of the tricuspid septal leaflet with respect to the mitral.
2. Agenesis of the right ventricle: requires rightleft shunt with cyanosis, and clinical manifestations appear in the neonatal period.
3. Loeffler's disease: is more typical in temperate countries, and usually has a more acute presentation and with eosinophilia. report revealed that the mass corresponded to a paraganglioma, which was considered benign by the extension study. A year later the patient is still asymptomatic with normal catecholamine analytical results.
This tumour is extremely rare. The absence of symptoms and signs indicating pheochromocytoma or other cardiac symptoms makes it difficult to diagnose. Seventy-five percent of patients have abnormal chest x-rays, as in our case. Although sensitivity is low for intrapericardial tumours. Metaiodobenzylguanidine scintigraphy, chest CT and MRI scans are essential for diagnosis and location. In our case, PET-CT was performed, of which we unfortunately have no record. In magnetic resonance images, paragangliomas are highly vascular tumours and the capacity of this technique for the diagnosis and anatomic characterisation of the tumour is greater than CT, as there is more tissue contrast even without administering contrast drugs. 7 This, therefore, is the technique of choice. In our patient, catecholamine determinations were negative and no symptoms indicating tumour secretory activity had been noticed. All intrapericardial paragangliomas require resection.