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Letter to the Editor Rare cardiac tumor simulating ST elevated acute myocardial infarction Bruno Marmelo a, , Davide Moreira a , Anne Delgado a , Luís Abreu a , Emanuel Correia a , Miguel Correia a , Pedro Ferreira a , Antonio Marinho da Silva b , Jorge Oliveira Santos a a Centro Hospitalar TondelaViseu, Serviço de Cardiologia, Portugal b Centro Hospitalar e Universitário de Coimbra, Serviço de Cardiologia, Portugal article info Article history: Received 8 June 2014 Accepted 27 July 2014 Available online xxxx Keywords: Cardiac mass tumor st elevation clear cell sarcoma A 57-year-old Caucasian woman, appealed to our hospital with a typical pleuritic chest pain. The patient was in good condition and no abnormalities on physical examination with the exception for a sinus tachycardia (110/min) were noticed. Electrocardiogram which was subsequently performed revealed a convex ST segment elevation in leads V1V5 (up to 3 mm) suggesting acute myocardial infarction (Fig. 1). However, blood tests showed negative troponin I and myoglo- bin. CRP was elevated to 21.3 mg/dL. Otherwise she related mild systemic hypertension and a history of foot surgery (for cutaneous lesion?) 7 years ago. As clinical and blood tests suggested a non-ischemic etiology, this one showed a circumferential pericardial effusion being signicant at the left ventricular free wall, with good global and regional LV con- tractility. The parasternal long axis view showed severe septal hypertro- phy protruding inside the right ventricle (Fig. 2). At parasternal short axis view this hypertrophy looks like a heterogeneous mass of the septum and the anterior wall (Fig. 3). Then a suspicion for heart tumor was then considered. The addition of contrast (Sonovue®) enhanced the heterogenic aspect and a cleavage plane between the mass and the normal myocardium (Fig. 4). The patient had no specic clinical organ complaints other than the thoracic pain. A targeted physical examination was performed, showing no relevant abnormalities, including lymphadenopathy. Thoracic CT angiography conrmed a heterogeneous inltrative mass of 5.9 × 4.1 cm located in the anterior wall, mainly involving the right ventricle (RV), apex and the interventricular septum (IVS). Some lung parenquimal nodes b 10 mm were suggestive of metastasis (Fig. 5). An abdominopelvic CT excluded any primary tumor in those locations. All tumor markers were negative. The patient was referenced to a cardiac surgery center where they biopsied an epicardial exophytic lesion. Also a pleuropericardic window of 4 × 4 cm was open. Pleuritic pain disappeared thereafter. Histology of the biopsy material revealed only a brin mass and a chronic collageneous pericarditis. Since the histopathological result didn't match with the imagiological ndings, a cardiac MR was performed: a voluminous mass of 9 × 9 × 7 cm involving the IVS and anterior wall of the RV, with peri- and epicardial extension, and a sarcomatous ap- pearance (Fig. 6) was reported. Lung nodes were also related as pulmonary metastasis, the biggest measuring 23 mm. Given the unsuccessful surgery, the will of the patient for a less invasive diagnostic method and the inltrative aspect of the mass, right ventricle catheterization with endomyocardial biopsy was scheduled. A 23 mm Hg systolic gradient trough the outow tract was registered. Ventriculography showed a small right ventricular cavity, the body and outow tract being occupied with the mass (Fig. 7). The biopsy of 6 myocardial fragments revealed only a probable myocarditis in regression. As the diagnosis was still obscure and the mass and the metastasis were growing fast, biopsy of the lung nodules was then carried out through lateral thoracotomy. Finally the histopathological study reported a clear cell sarcoma. With the information collected retro- spectively we know now that the patient had had a primary lesion of this tumor in her left hallux, which was removed in 2006. However she was lost in follow up and never had an appointment after surgery. The information of the patient about the lesion was erroneous regarding the malignancy and the tissue affected. Clear cell sarcomas are very rare slow-growing tumors with high- grade malignancy, being also known as soft tissue melanoma. The primary lesion of this tumor occurs generally in the extremities, mostly knee, ankle and foot. Typically late metastasis and tumor recurrence are frequent. Given the rarity of this tumor there are no randomized ther- apeutic trials and the response to chemotherapy is rare. The patient died 7 months after the initial diagnosis. Conict of interest The authors report no relationships that could be construed as a conict of interest. International Journal of Cardiology xxx (2014) xxxxxx IJCA-18487; No of Pages 4 Corresponding author at: Serviço de Cardiologia do Centro Hospitalar Tondela Viseu, Avenida Rei Dom Duarte, 3504-509 Viseu, Portugal. http://dx.doi.org/10.1016/j.ijcard.2014.07.168 0167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved. Contents lists available at ScienceDirect International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard Please cite this article as: Marmelo B, et al, Rare cardiac tumor simulating ST elevated acute myocardial infarction, Int J Cardiol (2014), http:// dx.doi.org/10.1016/j.ijcard.2014.07.168

Rare cardiac tumor simulating ST elevated acute myocardial infarction

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Page 1: Rare cardiac tumor simulating ST elevated acute myocardial infarction

International Journal of Cardiology xxx (2014) xxx–xxx

IJCA-18487; No of Pages 4

Contents lists available at ScienceDirect

International Journal of Cardiology

j ourna l homepage: www.e lsev ie r .com/ locate / i j ca rd

Letter to the Editor

Rare cardiac tumor simulating ST elevated acute myocardial infarction

Bruno Marmelo a,⁎, Davide Moreira a, Anne Delgado a, Luís Abreu a, Emanuel Correia a, Miguel Correia a,Pedro Ferreira a, Antonio Marinho da Silva b, Jorge Oliveira Santos a

a Centro Hospitalar Tondela—Viseu, Serviço de Cardiologia, Portugalb Centro Hospitalar e Universitário de Coimbra, Serviço de Cardiologia, Portugal

⁎ Corresponding author at: Serviço de Cardiologia do CAvenida Rei Dom Duarte, 3504-509 Viseu, Portugal.

http://dx.doi.org/10.1016/j.ijcard.2014.07.1680167-5273/© 2014 Elsevier Ireland Ltd. All rights reserved

Please cite this article as: Marmelo B, et al, Rdx.doi.org/10.1016/j.ijcard.2014.07.168

a r t i c l e i n f o

Article history:

Received 8 June 2014Accepted 27 July 2014Available online xxxx

Keywords:Cardiacmasstumorst elevationclear cell sarcoma

nodes b10 mmwere suggestive of metastasis (Fig. 5).An abdominopelvic CT excluded any primary tumor in those

locations. All tumor markers were negative.The patient was referenced to a cardiac surgery center where they

biopsied an epicardial exophytic lesion. Also a pleuropericardic windowof 4 × 4 cmwas open. Pleuritic pain disappeared thereafter. Histology ofthe biopsy material revealed only a fibrin mass and a chroniccollageneous pericarditis. Since the histopathological result didn'tmatch with the imagiological findings, a cardiac MR was performed:a voluminous mass of 9 × 9 × 7 cm involving the IVS and anterior wallof the RV, with peri- and epicardial extension, and a sarcomatous ap-

A 57-year-old Caucasian woman, appealed to our hospital with atypical pleuritic chest pain. The patient was in good condition and noabnormalities on physical examination with the exception for a sinustachycardia (110/min) were noticed. Electrocardiogram which wassubsequently performed revealed a convex ST segment elevation inleads V1–V5 (up to 3 mm) suggesting acute myocardial infarction(Fig. 1). However, blood tests showed negative troponin I and myoglo-bin. CRP was elevated to 21.3 mg/dL.

Otherwise she related mild systemic hypertension and a history offoot surgery (for cutaneous lesion?) 7 years ago.

As clinical and blood tests suggested a non-ischemic etiology,this one showed a circumferential pericardial effusion being significantat the left ventricular free wall, with good global and regional LV con-tractility. The parasternal long axis view showed severe septal hypertro-phy protruding inside the right ventricle (Fig. 2). At parasternal shortaxis view this hypertrophy looks like a heterogeneous mass of theseptum and the anterior wall (Fig. 3). Then a suspicion for hearttumor was then considered.

The addition of contrast (Sonovue®) enhanced the heterogenicaspect and a cleavage plane between the mass and the normalmyocardium (Fig. 4).

The patient had no specific clinical organ complaints other than thethoracic pain. A targeted physical examination was performed, showingno relevant abnormalities, including lymphadenopathy. ThoracicCT angiography confirmed a heterogeneous infiltrative mass of 5.9 ×4.1 cm located in the anterior wall, mainly involving the right ventricle

entro Hospitalar Tondela Viseu,

.

are cardiac tumor simulating

(RV), apex and the interventricular septum(IVS). Some lungparenquimal

pearance (Fig. 6) was reported. Lung nodes were also related aspulmonary metastasis, the biggest measuring 23 mm.

Given the unsuccessful surgery, the will of the patient for a lessinvasive diagnostic method and the infiltrative aspect of the mass,right ventricle catheterization with endomyocardial biopsy wasscheduled. A 23 mm Hg systolic gradient trough the outflow tractwas registered. Ventriculography showed a small right ventricularcavity, the body and outflow tract being occupied with the mass(Fig. 7). The biopsy of 6 myocardial fragments revealed only a probablemyocarditis in regression.

As the diagnosis was still obscure and the mass and the metastasiswere growing fast, biopsy of the lung nodules was then carried outthrough lateral thoracotomy. Finally the histopathological studyreported a clear cell sarcoma. With the information collected retro-spectively we know now that the patient had had a primary lesionof this tumor in her left hallux, which was removed in 2006. Howevershe was lost in follow up and never had an appointment after surgery.The information of the patient about the lesion was erroneousregarding the malignancy and the tissue affected.

Clear cell sarcomas are very rare slow-growing tumors with high-grade malignancy, being also known as soft tissue melanoma. Theprimary lesion of this tumor occurs generally in the extremities, mostlyknee, ankle and foot. Typically late metastasis and tumor recurrenceare frequent. Given the rarity of this tumor there are no randomized ther-apeutic trials and the response to chemotherapy is rare.

The patient died 7 months after the initial diagnosis.

Conflict of interest

The authors report no relationships that could be construed as aconflict of interest.

ST elevated acute myocardial infarction, Int J Cardiol (2014), http://

Page 2: Rare cardiac tumor simulating ST elevated acute myocardial infarction

Fig. 1. Electrocardiogram showing ST elevation in V1–V5 without secondary ST depression in inferior leads.

Fig. 2. Echocardiography in PLAXview. LV: left ventricle; LA: left atrium; IVS: interventricularseptum; RV: right ventricle; PE: pericardial effusion.

Fig. 3. Echocardiography in PSAX-MV view. LV: left ventricle; IVS: interventricularseptum; RV: right ventricle; PE: pericardial effusion; Arrow: mass.

e2 B. Marmelo et al. / International Journal of Cardiology xxx (2014) xxx–xxx

Please cite this article as: Marmelo B, et al, Rare cardiac tumor simulating ST elevated acute myocardial infarction, Int J Cardiol (2014), http://dx.doi.org/10.1016/j.ijcard.2014.07.168

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Fig. 4. Contrast echocardiography enhancing the heterogeneous aspect of the mass.LV: left ventricle; RV: right ventricle; Arrow: mass.

Fig. 5. Thoracic angioCT. LV: left ventricle; RV: right ventricle; Arrow: mass.

Fig. 6. Cardiac magnetic resonance. LV: left ventricle; RV: right ventricle; Arrow: mass.

Fig. 7. Right ventriculography showing the mass occupying part of the body and outflowtract of the right ventricle.

e3B. Marmelo et al. / International Journal of Cardiology xxx (2014) xxx–xxx

Please cite this article as: Marmelo B, et al, Rare cardiac tumor simulating ST elevated acute myocardial infarction, Int J Cardiol (2014), http://dx.doi.org/10.1016/j.ijcard.2014.07.168

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Fig. 8. 3D echocardiography showing tumor growth.

e4 B. Marmelo et al. / International Journal of Cardiology xxx (2014) xxx–xxx

Please cite this article as: Marmelo B, et al, Rare cardiac tumor simulating ST elevated acute myocardial infarction, Int J Cardiol (2014), http://dx.doi.org/10.1016/j.ijcard.2014.07.168