IMAGE
Heart, Lung and Circulation (2015) 24, e63–e64 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2014.11.017
Obstructive Pulmonary Artery Sarcoma Anna Nowacka, MD a*, Olivier Muller, MD, PhD b, Michel Hurni, MD a, Rene´ Preˆtre, MD, PhD, Prof a, Lars Niclauss, MD a a
Department of Cardiac Surgery, University Hospital, Lausanne Switzerland Department of Cardiology, University Hospital, Lausanne Switzerland
b
Received 21 October 2014; accepted 20 November 2014; online published-ahead-of-print 28 November 2014
Keywords
Adult Primary cardiac tumor Undifferentiated pleomorphic sarcoma Pulmonary valve Pulmonary artery
A 68 year-old woman presented with increasing dyspnoea (NYHA II) and systolic murmur at auscultation. Echocardiography showed thickened pulmonary valve leaflets, a systolic prolapsing mass provoking severe pulmonary stenosis (peak systolic pulmonary pressure: 42 mmHg), no regurgitation, minimal right ventricular dilatation but normal ventricular function. CT scan showed a dense structure extending from the right ventricular outflow tract (RVOT) up to the pulmonary bifurcation infiltrating the pulmonary valve (PV) (Fig. 1 a,b). MRI confirmed a mass of 203034 mm, a thickened pulmonary arterial wall (of 6 mm) and contrast enhancement of the anterior wall of the pulmonary trunk and right ventricle, indicating an infiltrating tumour (Fig. 1 c). A PET scan did not indicate further tumour localisations. After sternotomy, pulmonary arteriotomy was realised (under cardiopulmonary bypass, aortic bi-caval cannulation,
cardiac arrest). Intra-operative status showed a tense whitish mass, infiltrating two-thirds of the PV circumference (Fig. 2 a,b). The rudimentary valve together with the attached mass and anterior circumference of the pulmonary trunk were excised and replaced by a homograft. Complete resection was impracticable due to advanced tumour infiltration with extension to the RVOT and pulmonary bifurcation. Histological examination revealed an undifferentiated pleomorphic sarcoma. Primary pulmonary artery sarcomas are rare with PV involvement of 30%. Palliative tumour resection is recommended to improve survival, ideally in combination with neo-adjuvant therapy (not realised due to rapid aggravation of dyspnoea and unknown tumour origin before surgery). Prognosis is poor with reported mean survival duration of 11 3 months after palliative surgery [1].
*Corresponding author at: Department of cardiac surgery, Rue du Bugnon 46/CH-1011, Email: [email protected] © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
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A. Nowacka et al.
Figure 1 Axial (a) and sagittal (b) view of the CT scan shows an isodense structure (x) at the level of the pulmonary valve extending to RVOT and bifurcation. In the MRI (T1 –weighted) (c) contrast enhancement of the antero-lateral wall of the pulmonary trunk and right ventricle indicate advanced tumour infiltration (arrows).
Figure 2 Macroscopic view of neoplasm adherent to the pulmonary trunk (a) and of the tumour after resection (b).
Reference [1] Blackmon SH, Rice DC, Correa AM, Mehran R, Putnam JB, Smythe WR, et al. Management of primary pulmonary artery sarcomas. Ann Thorac Surg 2009;87:977–84.
Heart, Lung and Circulation (2015) 24, e63–e64 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2014.11.017
Obstructive Pulmonary Artery Sarcoma Anna Nowacka, MD a*, Olivier Muller, MD, PhD b, Michel Hurni, MD a, Rene´ Preˆtre, MD, PhD, Prof a, Lars Niclauss, MD a a
Department of Cardiac Surgery, University Hospital, Lausanne Switzerland Department of Cardiology, University Hospital, Lausanne Switzerland
b
Received 21 October 2014; accepted 20 November 2014; online published-ahead-of-print 28 November 2014
Keywords
Adult Primary cardiac tumor Undifferentiated pleomorphic sarcoma Pulmonary valve Pulmonary artery
A 68 year-old woman presented with increasing dyspnoea (NYHA II) and systolic murmur at auscultation. Echocardiography showed thickened pulmonary valve leaflets, a systolic prolapsing mass provoking severe pulmonary stenosis (peak systolic pulmonary pressure: 42 mmHg), no regurgitation, minimal right ventricular dilatation but normal ventricular function. CT scan showed a dense structure extending from the right ventricular outflow tract (RVOT) up to the pulmonary bifurcation infiltrating the pulmonary valve (PV) (Fig. 1 a,b). MRI confirmed a mass of 203034 mm, a thickened pulmonary arterial wall (of 6 mm) and contrast enhancement of the anterior wall of the pulmonary trunk and right ventricle, indicating an infiltrating tumour (Fig. 1 c). A PET scan did not indicate further tumour localisations. After sternotomy, pulmonary arteriotomy was realised (under cardiopulmonary bypass, aortic bi-caval cannulation,
cardiac arrest). Intra-operative status showed a tense whitish mass, infiltrating two-thirds of the PV circumference (Fig. 2 a,b). The rudimentary valve together with the attached mass and anterior circumference of the pulmonary trunk were excised and replaced by a homograft. Complete resection was impracticable due to advanced tumour infiltration with extension to the RVOT and pulmonary bifurcation. Histological examination revealed an undifferentiated pleomorphic sarcoma. Primary pulmonary artery sarcomas are rare with PV involvement of 30%. Palliative tumour resection is recommended to improve survival, ideally in combination with neo-adjuvant therapy (not realised due to rapid aggravation of dyspnoea and unknown tumour origin before surgery). Prognosis is poor with reported mean survival duration of 11 3 months after palliative surgery [1].
*Corresponding author at: Department of cardiac surgery, Rue du Bugnon 46/CH-1011, Email: [email protected] © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
e64
A. Nowacka et al.
Figure 1 Axial (a) and sagittal (b) view of the CT scan shows an isodense structure (x) at the level of the pulmonary valve extending to RVOT and bifurcation. In the MRI (T1 –weighted) (c) contrast enhancement of the antero-lateral wall of the pulmonary trunk and right ventricle indicate advanced tumour infiltration (arrows).
Figure 2 Macroscopic view of neoplasm adherent to the pulmonary trunk (a) and of the tumour after resection (b).
Reference [1] Blackmon SH, Rice DC, Correa AM, Mehran R, Putnam JB, Smythe WR, et al. Management of primary pulmonary artery sarcomas. Ann Thorac Surg 2009;87:977–84.
