International Journal of Cardiology 132 (2009) e5 – e7 www.elsevier.com/locate/ijcard
Letter to the Editor
Pericardial PEComa: Echocardiographic features Reza Mollazadeh a,⁎, Ali Reza Moaref a , Mohammad Ghazinoor b , Salma Sefidbakht c , Mohammad Ali Babai Bigi a , Bita Geramizadeh c , Shahab Shahrzad a , Yadollah Mahmoodi a b
a Cardiology Department, Nemazee Hospital, Shiraz University of medical science, Shiraz, Iran Cardiac Surgery Department, Nemazee Hospital, Shiraz University of medical science, Shiraz, Iran c Pathology Department, Shiraz Medical school, Shiraz University of medical science, Shiraz, Iran
Received 1 July 2007; accepted 6 July 2007 Available online 26 November 2007
Abstract The patient was a 19 year old man referred to our center due to progressive effort dyspnea and left sided pleuritic chest pain for 1 month. Echocardiographic examination showed a posteriorly located, giant, well demarcated mass, that compresses the left atrium. It was encased by moderate pericardial effusion. Histhopathological analysis disclosed neoplastic cells arranged around blood vessels with immunoreactivity for HMB-45 indicative of PEComa (Perivascular Epitheloid Cell differentiation). Six month follow up was uneventful. Up to the best of our knowledge, this is the first case of pericardial PEComa reported in the literature. © 2007 Elsevier Ireland Ltd. All rights reserved. Keywords: Echocardiography; PEComa; Pericardial; Tumor; Pericardial effusion
1. Case presentation The patient was a 19 year old man referred to our center due to progressive effort dyspnea and left sided chest pain on deep inspiration for 1 month. He was able to perform all house hold chores for his family without significant distress. He stated that he did not use any specific medication. He denied any medical problems in his younger and elder siblings. On physical examination, the pulse was 80 beats/ min and regular, and blood pressure was 130/80 mmHg. The jugular veins were not distended. Paradoxical pulse was not apparent. The lungs were clear. No murmur or friction rub was audible on the cardiac auscultation. The abdomen was soft with no signs of ascites or hepatosplenomegaly. Superficial lymphadenopathy was not present. Chest X-ray showed marked cardiomegaly with clear lung fields. An
⁎ Corresponding author. Cardiology Department, Nemazee Hospital, Zand Avenue, Shiraz, Iran, P.O. Box: 71435-1414. Tel.: +98 917 313 3749; fax: +98 711 6261089. E-mail address:
[email protected] (A.R. Moaref). 0167-5273/$ - see front matter © 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2007.07.088
ECG showed minor ST-T changes. Trans-thoracic echocardiography showed a giant well demarcated mass in the posterior of the left atrium compressing it with concomitant moderate pericardial effusion (Movie 1, Fig. 1). Apical long axis view demonstrated the mass adhered to atrioventricular groove tightly (Movie 2). There was no right atrium or right ventricle diastolic collapse. Further delineation of mass with transesophageal echocardiography revealed many echo dense spots in the mass (Movie 3 and 4) with extension of the tumor more toward the apex and the left side (Movie 4, Fig. 2). Mitral inflow pattern was normal. Pulmonary and systemic veins were free of mass. To discover nature of the mass and rule out possibility of malignancy, the patient was sent to the operating room. After median sternotomy, pericardium was opened through a longitudinal midline incision. A mass inside pericardial cavity, located in the atrioventricular groove with extension toward posterior of left ventricle apex and adhesion to epicardium (Fig. 3) was detected. It measured 16 × 10 × 6 cm (Fig. 4). Mass was excised and 250 cm 3 pericardial fluid was drained. Histhopathological analysis disclosed neoplastic cells arranged around blood vessels with immunoreactivity for
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Fig. 3. Pericardium encased the mass.
Fig. 1. Trans-thoracic echocardiography showed a giant well demarcated mass in the posterior of the left atrium compressing it with concomitant moderate pericardial effusion.
HMB-45 indicative of PEComa (Perivascular Epitheloid Cell differentiation). Six month follow up was uneventful. Pericardium can be involved with malignancy primarily or as a metastatic presentation of a tumor far away (more common). Metastatic carcinoma to the pericardium usually originates in the lung in the form of direct extension or lymphatic permeation. Other less common tumors are breast carcinoma, malignant melanoma, and lymphoma [1]. Primary tumors of pericardium are much rarer. Although pericardial mesothelioma is the most common primary pericardial cancer, its frequency is much less than similar tumor in the pleura or pericardium [2]. Other primary tumors of the pericardium are exceptionally rare: germ cell tumor, angiosarcoma, myolipoma, lymphoma [1]. PEComas (tumors showing perivascular epithelioid cell differentiation) are a family of related mesenchymal neoplasms that include angiomyolipoma, lymphangiomyomatosis, clear cell ‘sugar’ tumor of the lung, and a group of
Fig. 2. Transesophageal echocardiography in trans-gastric position revealed the mass in the posterior of left ventricle.
morphologically and immunophenotypically similar lesions arising at a variety of visceral and soft tissue sites. These tumors all share a distinctive cell type, the perivascular epithelioid cell or ‘PEC’ (which has no known normal tissue counterpart). PEComas appear to arise most commonly at visceral (especially gastrointestinal and uterine), retroperitoneal, and abdominopelvic sites, with a subset occurring in somatic soft tissue and skin [3]. Nearly all PEComas show immunoreactivity for both melanocytic (HMB-45 and/or melan-A) and smooth muscle (actin and/or desmin) markers. Tazelar et al. [4] presented a 29 year old man with a seven-year history of atrial fibrillation presented with symptoms of mitral stenosis. Intraoperatively a left atrial tumor growing through the interatrial septum to involve right atrium and extending throughout the left lateral wall into the pericardial sac was detected. Grossly, the tumor appeared to extend very near the pulmonary veins. The patient died shortly thereafter of cardiac failure thought to be caused by nontumorous coronary artery thromboemboli. His histhopathological analysis was in favor of primary extra pulmonary sugar tumors, or “PEST's”. Trans-thoracic echocardiography (preferably complemented with transesophageal technique) can locate the abnormal cardiac mass and its extensions and contour; existence or absence of calcification, free fluid in the mass; and concomitant pericardial effusion, although rarely, if ever, it can identify the nature of mass [5,6].
Fig. 4. Large mass was excised.
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Up to the best of our knowledge, this is the first case of pericardial PEComa reported in the literature. Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.ijcard.2007.07.088. References [1] Adenle AD, Edwards JE. Clinical and pathologic features of metastatic neoplasms of the pericardium. Chest 1982;81(2):166–9 Feb.
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[2] Cardillo I, Spugnini EP, Verdina A, Galati R, Citro G, Baldi A. Cox and mesothelioma: an overview. Histol Histopathol 2005;20(4):1267–74 Oct. [3] Hornick JL, Fletcher CDM. PEComa: what do we know so far? Histopathology 2006;48:75–82. [4] Tazelaar HD, Batts KP, Srigley JR. Primary extra pulmonary sugar tumor (PEST): a report of four cases. Mod Path 2001;14:615–22. [5] Pepi M, Muratori M. Echocardiography in the diagnosis and management of pericardial disease. J Cardiovasc Med (Hagerstown) 2006;7(7):533–44 Jul. [6] Wadler S, Chahinian P, Slater W, Goldman M, Mendelson D, Holland JF. Cardiac abnormalities in patients with diffuse malignant pleural mesothelioma. Cancer 1986;58:2744–50.
