Cardiovascular Pathology 10 (2001) 147 – 149
Primary pericardial mesothelioma Sadik Eryilmaz, Mustafa S˛irlak*, Mustafa Bahadir ˙Inan, Esra Erden, Neyyir Tuncay Eren, Tu¨mer C˛orapc˛iog˘lu, Hakki Akalin Department of Cardiovascular Surgery, Ankara University Faculty of Medicine Hospital, Ankara, Turkey Received 28 February 2001; received in revised form 11 April 2001; accepted 19 April 2001
Abstract Primary pericardial mesothelioma is an extremely rare tumor. This case illustrates the typical late presentation of primary pericardial mesothelioma with symptoms and signs of constrictive pericarditis. An unusual feature was the complete encasement of the heart by the tumor. No satisfactory treatment was available. D 2001 Elsevier Science Inc. All rights reserved. Keywords: Mesothelioma; Pericardial; Pleural
1. Introduction Primary tumors of the pericardium are extremely rare. One of the largest necropsy series gives an incidence of 0.0022% in 500 000 cases [1,2]. Among these, mesothelioma is probably the most common type, followed by sarcoma, teratoma, fibroma, lipoma, and angioma [3]. The incidence in both sexes is almost equal, within the age range of 1 –79 years. The tumor is commonly diagnosed at an advanced stage and often results in evidence of constriction caused by tumor expansion or associated with serous or hemorrhagic pericardial effusion. The definitive diagnosis is often made after sternotomy or thoracotomy [4].
2. Case report A 29-year-old man admitted to hospital with a 4-month history of dyspnea and exertional chest pain. Weight loss, malaise, night sweating, and pretibial edemas were the accompanying disorders. He complained of a cupfull of sputum daily and coughing for the last month. He had mild jaundice. His blood pressure was 100/70 mm Hg. Auscultation revealed no pathologic bruits and murmurs. The lungs were moderately congested, and there was bilateral pleural effusion, which was prominent on the right side. Chest * Corresponding author. Tel.: +90-312-466-51-39; fax: +90-312-36248-25. E-mail address:
[email protected] (M. S˛irlak).
radiography showed cardiac enlargement. Bilateral pleural effusion, linear collapse, and pulmonary venous congestion were also noted. Echocardiography revealed the left ventricle end-diastolic volume as 41 mm, left ventricle endsystolic diameter as 27 mm, left atrial diameter as 43 mm, aortic root diameter as 33 mm, fractional shortening as 34.1%, pulmonary artery systolic pressure as 40 mm Hg, and normal cardiac valves. Thickened pericardium severely diminished the diastolic functions of the heart. While blood cell count was 19 109/l, total billirubin 2.55 mg/dl, GGT 85 U/l, ALT 44 U/l, and LDH 637 U/l. Other laboratory tests were normal. Blood, urine, and sputum cultures were sterile. Viral titers, tuberculin test, and autoantibody screen were negative. Pleural aspiration drew clear yellow fluid, with a protein content of 34 g/l. In systemic ultrasonography, right jugular vein was thrombosed. Lymph nodes smaller than 1.5 cm were detected in both cervical chains. Diffuse hepatomegaly suggesting vascular congestion was noted. In computed tomography, mediastinal, pretracheal, prevascular, and aortapulmonary lymph nodes, the greatest of which reach to 2 cm in diameter, were present. There was a huge amount of dense fluid collection in the pleura and also in the pericardium, but with lesser amount. In cardiac catheterization, diastolic pressures were almost equal in all cardiac chambers. Pulmonary capillary wedge pressure was 28 mm Hg; pulmonary artery pressure was 46/ 26 mm Hg; right ventricle pressure was 46/12 mm Hg; right atria pressure was 24 mm Hg; and left ventricle pressure was 105/12 mm Hg. Coronary arteries were normal.
1054-8807/01/$ – see front matter D 2001 Elsevier Science Inc. All rights reserved. PII: S 1 0 5 4 - 8 8 0 7 ( 0 1 ) 0 0 0 7 3 - 4
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After the median sternotomy, we saw that the heart and the great vessels were covered with tumoral mass. We tried to separate the surface of the heart and the surrounding tissues from this mass. However, it was impossible to resect the whole mass. Both pleuras were intact. We opened the pleuras and emptied the serous fluid and closed the patient. Although we used cytotoxic medication convenient to the pathological results, no regression was noted in the tumor and the patient died of respiratory arrest in the 3rd week. In the pathologic evaluation macroscopically, the mass was consisted of five amorphous pieces, the largest of which was 5 3.5 0.5 cm in diameter. The pieces were nodular and consisted of membrane-like rigid tissues. In sections, myxoid and solid white –gray areas were noted. Four-micronthick sections were prepared and stained with hematoxylin – eosin dye and also with LMWCK, HMWCK, Vimentin, CEA, EMA, Leu-M1, and Calretinin antibodies by streptoavidin – biotin peroxidase method. In the microscopic evaluation of the H&E-stained sections, various types of growth patterns like adenoid, papillamatous, and diffuse tumoral infiltration was noted. Tumor cells were generally round and hobnailshaped, with central nucleus and large eosinophilic cytoplasm. Moderate-degree pleomorphism and mitosis were noted in the atypical cells. Some cells were noted as peripheric nucleated, large vacuoles in cytoplasm, and signet-ringshaped. Stroma was generally hyalinised fibrous tissue type, and, in particular areas, there was dense signet-ring-shaped cells, in which stroma was loose and myxoid-type (Fig. 1). In atypical cells, diffuse cytoplasmic positivity with HMWCK and LMWCK and diffuse membranous positivity
with EMA were detected. With Vimentin, focal cytoplasmic staining was seen. In addition, diffuse granular cytoplasmic staining was detected in tumor cells with Calretinin. However, there was no positive specific staining with Leu-M1 and CEA. Especially negative staining with CEA is very important in differential diagnose with adenocarcinoma, which highly gives positivity with CEA. This case was diagnosed as malignant epithelial mesothelioma by the aforementioned findings. Because no other laboratory and clinical findings showing that the tumor was related to the pleura, it was concluded that it was primary pericardial mesothelioma.
3. Comment Mesothelioma is the most common of the pericardial malignant tumors [3]. Due to the spread of the tumoral mass, constrictions occur in the mediastinal peripheric tissues and on the heart surfaces. Echocardiography, computed tomography, study of the fluid, gallium or technetium radioisotope scanning, and cardiac catheterization are useful diagnostic tools [4,5]. Precise diagnosis is done by the pathologic evaluation of the surgical material. It is necessary for the diagnosis of the primary pericardial mesothelioma to show that the tumor has no relationship with the pleural surfaces. The total encasement of the heart is rare, but, recently, this kind of cases has been reported [4,6]. Pericardial tumor generally tightly sticks to the epicardium and may even invade the myocardium [7]. The
Fig. 1. Diffuse distribution pattern of atypical cells partly consists of signet-ring-shaped cells.
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tumor may even involve the coronary arteries and the conduction system of the heart. Symptoms due to compression of the great vessels can occur [4]. In our patient, right jugular vein was occluded. Gross pathologic view consists of invasive mass, solid, cystic, angiomatous, or multiple nodes [4]. Although the local spread of the tumor is common, extrathoracic spread is rare [4]. One should know that there would be more therapeutical options if the disease was diagnosed earlier. Surgery is done to prevent the compression of the heart and obstruction of the vessels and the other tissues. In case of pericardial effusion, local use of cytotoxic and sclerosing agents may be useful. Radiotherapy may temporary diminish the dimensions of the tumor [4]. In many cases, primary mesothelioma was diagnosed postmortem. In our case, it was diagnosed when the patient was alive.
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Esra Erden, MD is from the Department of Pathology, Ankara University Faculty of Medicine Hospital, Ankara.
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