Primary malignant peripheral nerve sheath tumour in the right atrium

Primary malignant peripheral nerve sheath tumour in the right atrium A 35-year-old, previously healthy woman presented with a 1-month history of palpitations, dyspnoea, cough, and facial and lower limb oedema. Transthoracic echocardiography showed a right atrial mass (71 × 33 mm) in close relationship with the tricuspid valve, causing slight increase in forward flow (Vmax 1.1 m/s, Panel A). It was also found to be close to the opening of the superior vena cava on transoesophageal echocardiography, without interference on venous return (Panel B). Magnetic resonance imaging showed moderate pericardial and pleural effusion, with normal myocardial viability and perfusion (Panels C and D). 18Fluorine 18 fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) demonstrated abnormal 18F-FDG uptake in the mass (SUVmax 16.4), the root of the ascending aorta, one mediastinal node, C6 and the left 4th rib, suggesting metastasis (Panels E and F). Our initial plan was to excise the tumour. Perioperatively, we found the tumour encasing the root of the ascending aorta and the right atrial appendage, dramatically increasing the difficulty of establishing the extracorporeal circulation and subsequent excision. Consequently, a needle biopsy was taken instead through the anterior right atrial wall. Immunohistochemical analysis showed positivity for S100 (Panel G), NSE (Panel H), SMA (Panel I), CD57, TLE-1 and 20% positivity for MIB-1, whereas staining for PCK, EMA, WT-1, CK5/6, desmin, and myogenin yielded negative results. This suggested the diagnosis of a malignant peripheral nerve sheath tumour (MPNST). Primary cardiac MPNST is very rare. Surgical excision seems to be the gold standard, with median survival ranging from 3 months to 1 year due to high recurrence rate and metastasis. When surgical excision is impossible, radiotherapy can be considered. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2018. For permissions, please email: journals.permissions@oup.com. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices) http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Heart Journal Oxford University Press

Primary malignant peripheral nerve sheath tumour in the right atrium

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Publisher
Oxford University Press
Copyright
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2018. For permissions, please email: journals.permissions@oup.com.
ISSN
0195-668X
eISSN
1522-9645
D.O.I.
10.1093/eurheartj/ehy302
Publisher site
See Article on Publisher Site

Abstract

A 35-year-old, previously healthy woman presented with a 1-month history of palpitations, dyspnoea, cough, and facial and lower limb oedema. Transthoracic echocardiography showed a right atrial mass (71 × 33 mm) in close relationship with the tricuspid valve, causing slight increase in forward flow (Vmax 1.1 m/s, Panel A). It was also found to be close to the opening of the superior vena cava on transoesophageal echocardiography, without interference on venous return (Panel B). Magnetic resonance imaging showed moderate pericardial and pleural effusion, with normal myocardial viability and perfusion (Panels C and D). 18Fluorine 18 fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) demonstrated abnormal 18F-FDG uptake in the mass (SUVmax 16.4), the root of the ascending aorta, one mediastinal node, C6 and the left 4th rib, suggesting metastasis (Panels E and F). Our initial plan was to excise the tumour. Perioperatively, we found the tumour encasing the root of the ascending aorta and the right atrial appendage, dramatically increasing the difficulty of establishing the extracorporeal circulation and subsequent excision. Consequently, a needle biopsy was taken instead through the anterior right atrial wall. Immunohistochemical analysis showed positivity for S100 (Panel G), NSE (Panel H), SMA (Panel I), CD57, TLE-1 and 20% positivity for MIB-1, whereas staining for PCK, EMA, WT-1, CK5/6, desmin, and myogenin yielded negative results. This suggested the diagnosis of a malignant peripheral nerve sheath tumour (MPNST). Primary cardiac MPNST is very rare. Surgical excision seems to be the gold standard, with median survival ranging from 3 months to 1 year due to high recurrence rate and metastasis. When surgical excision is impossible, radiotherapy can be considered. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2018. For permissions, please email: journals.permissions@oup.com. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)

Journal

European Heart JournalOxford University Press

Published: May 26, 2018

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