Pulmonary artery sarcomas are uncommon intracardiac tumours with poor prognosis. We report the case of a 69-year-old woman in good health presenting with rapidly progressive dyspnoea and an unfamiliar systolic murmur. Echocardiography revealed pulmonary valve stenosis due to an obstructing mobile mass. Imaging studies confirmed the presence of a contrast-enhancing lesion adherent to the valve, extending into the pulmonary trunk and right ventricular outflow tract, and suggestive of malignancy.
Endovascular biopsy was attempted with no success. Surgical resection with autologous graft valve replacement and pulmonary artery reconstruction was performed. Postoperative histological examination
confirmed the diagnosis of an undifferentiated pleomorphic sarcoma. Pulmonary artery sarcoma should be considered as a rare differential diagnosis in patients presenting with dyspnoea and a crescendo-decrescendo systolic murmur increasing with inspiration. Echocardiography is a useful first diagnostic approach but multi-imaging assessment is almost always necessary for definite diagnosis. Our case provides insights into the challenges met by cardiologists, radiologists and cardiac surgeons in the management of such cases

Antiochos, P.

Descloux, E.

Hurni, M.

Monney, P.

Muller, O.

Niclauss, L.

Qanadli, SD.

Rotman, S.

Schlueter, L.

Serveur académique lausannois

CASE REPORT 186The importance of early referral for multidisciplinary managementPulmonary valve stenosis due  to undifferentiated pleomorphic sarcomaPanagiotis Antiochosa, Pierre Monneya, Salah Dine Qanadlic, Leopold Schluetere, Samuel Rotmanb,  Emilienne Desclouxb, Lars Niclaussd, Michel Hurnid, Olivier Mullera a Division of cardiology, Department of internal medicine, University Hospital of Lausanne (CHUV), Switzerland; b Institute of pathology, University Hospital of Lausanne (CHUV), Switzerland; c Department of radiology, University Hospital of Lausanne (CHUV), Switzerland; d Division of cardiovascular surgery, Department of surgery, University Hospital of Lausanne (CHUV), Switzerland; e The Cecil Clinic, Hirslanden, Lausanne, SwitzerlandSummaryPulmonary artery sarcomas are uncommon intracardiac tumours with poor prognosis. We report the case of a 69-year-old woman in good health presenting with rapidly progressive dyspnoea and an unfamiliar systolic murmur. Echocardiography revealed pulmonary valve stenosis due to an obstructing mobile mass. Imaging studies confirmed the presence of a contrast-enhancing lesion adherent to the valve, extending into the pulmo-nary trunk and right ventricular outflow tract, and suggestive of malig-nancy. Endovascular biopsy was attempted with no success. Surgical re-section with autologous graft valve replacement and pulmonary artery reconstruction was performed. Postoperative histological examination confirmed the diagnosis of an undifferentiated pleomorphic sarcoma. Pul-monary artery sarcoma should be considered as a rare differential diagno-sis in patients presenting with dyspnoea and a crescendo–decrescendo systolic murmur increasing with inspiration. Echocardiography is a useful first diagnostic approach but multi-imaging assessment is almost always necessary for definite diagnosis. Our case provides insights into the chal-lenges met by cardiologists, radiologists and cardiac surgeons in the man-agement of such cases.Key words: pulmonary artery sarcoma; undifferentiated pleomorphic cardiac sarcoma; pulmonary valve stenosis; right ventricular outflow tract Case descriptionA 69-year-old Caucasian woman presented to her general practitioner with new onset fatigue and rap-idly progressive dyspnoea on exertion. Upon physi-cal examination the patient was afebrile, blood pres-sure was 116/72 mm Hg, pulse rate 76/min and respiration rate 12/min. Heart sounds were normal, but a new, 3/6, crescendo–decrescendo systolic mur-mur, which increased during deep inspiration and was maximal along the left sternal border, was noted. Laboratory studies of electrolytes, renal function, liver and cardiac enzymes, acute phase reactants and complete blood count were within normal limits, as were a 12-lead ECG and a chest radiograph. Transthoracic echocardiography (TTE) revealed a mod erate pulmonary valve stenosis caused by a glob-ular mass protruding into the right ventricular out-flow tract (RVOT) during diastole (fig. 1). The maxi-mum pulmonary pressure gradient was measured at 42 mm Hg (mean 30 mm Hg). Left as well as right ven-tricle size and function were normal.The patient was promptly referred to our institution where cardiac computed tomography (CT) revealed a 25 × 35 × 24 mm mass attached to the RVOT and extending into the pulmonary trunk through the pulmonary valve (fig. 2). After intravenous injection of 60–80 ml iodinated contrast material, late image acquisition showed inhomogeneous enhancement of the mass and mural enhancement of the pulmonary trunk suggestive of infiltration. This technique in the context of cardiac CT consists of acquiring images 60 seconds after CT angiography in order to optimise tissue enhancement. Fludeoxyglucose (18F) positron emission tomography-CT (18F-FDG PET-CT) revealed mild metabolic activity of the mass, with no signs of metastasis (fig. 3). Finally, cardiac magnetic reso-nance imaging (MRI) confirmed proximal extension of the valve mass, 4 cm towards the pulmonary trunk, without apparent invasion of the bifurcation (fig. 4). A hyperintense signal in T2-weighted images indicated oedema, whereas moderate signal-en-hance ment during resting perfusion suggested rich vascularisation. Intense signals on gadolinium en-CARDIOVASCULAR MEDICINE – KARDIOVASKULÄRE MEDIZIN – MÉDECINE CARDIOVASCULAIRE 2015;18(6):186–190CASE REPORT 187Figure 1: (A) TTE parasternal long axis view of the pulmonary artery. The tumour is recognised as a large round mass invading the pulmonary valve. (B) Transoesophageal echocardiography (TOE) in transgastric inflow–outflow view of the right ventricle. The tricuspid valve and the right ventricular apex are seen at the top of the picture. The pulmonary valve is replaced by a ball-like tumour (*) obstructing the RVOT. Ao = aorta; PA = pulmonary artery; RV = right ventricle; RVOT = right ventricle outflow tract; TV = tricuspid valve.Figure 2: Cardiac computed tomography, 2d multi-planar reformation showing the voluminous mass (*) attached to the pulmonary valve (A) and protruding in the pulmonary trunk during systole (B).Ao = aorta; LV = left ventricle; PA = pulmonary artery; RVOT = right ventricle outflow tract.hancement sequences suggested rich extracellular content (fibrosis or extracellular matrix), but no sig-nificant necrosis (fig. 5). A provisory diagnosis of intracardiac malignancy was made on clinical (rapid installation of symp-toms) and imaging (vascular infiltrative mass) grounds, and endovascular biopsy was attempted. The procedure was uneventful, but yielded limited material for histology. The case was discussed by a multidisciplinary panel involving cardiologists, ra-diologists and a cardiothoracic surgeon, and surgery was decided on. The time delay from first diagnosis CARDIOVASCULAR MEDICINE – KARDIOVASKULÄRE MEDIZIN – MÉDECINE CARDIOVASCULAIRE 2015;18(6):186–190CASE REPORT 188on TTE to the operation was 22 days. During the pro-cedure, a bulky, white mass, densely adherent and in-filtrating the pulmonary valve and all of the pulmo-nary infundibulum was identified and removed, along with the deformed pulmonary valve, part of the RVOT, as well as most of the pulmonary trunk as proximally as possible to the bifurcation. An autolo-gous graft was used to replace the valve; the right ventricle was then anastomosed to the distal part of the pulmonary artery. Postoperative histology re-vealed a mesenchymal tumour consisting of highly atypical spindle cells without specific differentiation (fig. 6) [1]. Given the retrograde extension of the mass and the possibility of residual fragments very low in-side the RVOT, the patient was referred for adjuvant chemotherapy and radiotherapy after six weeks of convalescence in a cardiac rehabilitation centre. Discussion Our case highlights the major impediments encoun-tered during management of these rare and often Figure 4: (A) Cardiac magnetic resonance imaging (MRI) steady-state free precession (SSFP) cine acquisition in inflow–outflow view of the right ventricle. The tumour is seen as a massive thickening of the pulmonary valve (*). Note also the thickening  of the anterior wall of the pulmonary trunk (arrows). (B) Corresponding acquisition with a phase-sensitive inversion-recovery sequence after contrast injection. There is homogenous late gadolinium enhancement of the tumour (*) and of the anterior  wall of the pulmonary trunk (arrows).Ao = aorta; PA = pulmonary artery; RPA = right pulmonary artery; RA = right atrium; RV = right ventricle.Figure 3: Fludeoxyglucose (18F) positron emission tomography-computed tomography (18F-FDG PET-CT) showing discrete metabolic activity of the mass (arrow), with no evidence of other lesion.Ao = aorta; PA = pulmonary artery; RV = right ventricle.CARDIOVASCULAR MEDICINE – KARDIOVASKULÄRE MEDIZIN – MÉDECINE CARDIOVASCULAIRE 2015;18(6):186–190CASE REPORT 189fatal intracardiac tumours; from timely and accurate diagnosis to surgical resection and adjuvant care, pulmonary artery sarcomas (PAS) represent a diag-nostic and therapeutic challenge for cardiologists, radiologists, cardiac surgeons and oncologists.Typical onset age is 45–55 years with a female to male ratio of 2:1. The tumour typically arises within the pulmonary trunk and spreads into the pulmonary arteries. Embryologically, it originates from the bul-bus cordis, a structure that later gives birth to both the RVOT and pulmonary trunk, which could, in our case, explain valve involvement and RVOT extension. As with the majority of intracardiac tumours, pre-sentation is non-specific, with dyspnoea on exertion, chest or back pain, cough and haemoptysis being common symptoms. In many cases, the clinical pre-sentation mimics chronic thromboembolic pulmo-nary hypertension, and in advanced stages constitu-tional symptoms appear. Physical examination remains unremarkable until late. Our case is unique in the sense that it presented as a new unfamiliar murmur that alarmed the primary physician. Such a finding is extremely rare and highlights the para-mount importance of good auscultation skills and a clinical acumen. A new harsh systolic murmur that increases with inspiration indicates pulmonary ste-nosis and warrants further investigation with echo-cardiography. TTE may show an increased gradient across a narrowed RVOT [2]. The presence of a pedun-culated lesion arising from the RVOT, or pulmonary valve or trunk should always raise suspicion of PAS [3] and prompt a thoracic CT or a cardiac MRI.Figure 5: (A) T1-weighted image; the mass (arrow) has a signal iso-intense to the myocardium. (B) T2-weighted image; the mass has a signal hyper-intense to the myocardium indicating presence of tissue oedema. (C) Late gadolinium enhancement sequen ce; the mass shows bright enhancement indicating rich vascularisation. (D to F) Perfusion imaging; (D) contrast in the right cardiac chambers; (E) contrast in the left cardiac chambers; (F) contrast enhancing the myocardium: the mass shows  moderate first-pass enhancement with peak signal intensity lower than that of the myocardium. CARDIOVASCULAR MEDICINE – KARDIOVASKULÄRE MEDIZIN – MÉDECINE CARDIOVASCULAIRE 2015;18(6):186–190CASE REPORT 190CT findings can be misleading in over 50% of cases, as PAS is confused with the far more common condition of central pulmonary embolism. Both entities pre-sent with a filling defect in the pulmonary arteries. Suggestive of PAS are an inhomogeneous, soft tissue density, with the filling defect occupying the entire lumen of the pulmonary trunk, along with delayed contrast enhancement on angiography [4]. Once sus-picion is raised, PET-CT and MRI are helpful to differ-entiate fully the mass. Preoperative histological diagnosis is generally not possible although it should be attempted through CT-guided transthoracic aspi-ration, transvenous catheter biopsy or transbron-chial biopsy. In most cases definitive diagnosis is made only during surgery or at autopsy. Surgery  remains the cornerstone of management, offering definite diagnosis, clinical improvement and the best prognosis [5]. Even if curative resection is not possi-ble, palliative resection can provide symptom relief by restoring haemodynamics. Reported interven-tions range from bilateral pneumonectomy, pulmo-nary endarterectomy (PEA) with or without pneumo-nectomy and with or without reconstruction of the pulmonary artery to debulking and palliative stent-ing. Regarding adjuvant therapy, combined chemo- and radiotherapy should be adopted in nearly every case to extend palliation. Overall, PAS represents a fat al disease, with a mean survival time with out surgi-cal intervention of 1.5 months. Survival of 36.5 ± 20.2 months has been reported for patients benefiting from a “curative” resection while 11 ± 3 months for those undergoing “incomplete” resection [5]. In conclusion, it is important that physicians recog-nise the clinical features associated with PAS and in-clude it as a differential diagnosis in the appropriate setting. Early referral for multidisciplinary manage-ment in a tertiary centre offers the only chance for prolonged survival and improved quality of life.DisclosuresNo financial support and no other potential conflict of interest relevant to this article was reported.References A full list of references is available in the online version of this article.Correspondence: Panagiotis Antiochos, MD University Hospital  of Lausanne (CHUV) 46, rue du Bugnon CH-1011 Lausanne Switzerland panagiotis.antiochos [at]chuv.chFigure 6: (A) (Haematoxylin and eosin; × 40) Postoperative histology revealed a poorly differentiated tumour composed  of a proliferation of spindle cells with fibroblastic differentia-tion (black arrows). Some areas have a fibromyxoid pattern (*). (B) (haematoxylin and eosin; × 200); Highly atypical giant spindle cell (white arrows). (C) Macroscopic aspect of the mass.ABCCARDIOVASCULAR MEDICINE – KARDIOVASKULÄRE MEDIZIN – MÉDECINE CARDIOVASCULAIRE 2015;18(6):186–190

Pulmonary valve stenosis due to undifferentiated pleomorphic sarcoma

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Pulmonary valve stenosis due to undifferentiated pleomorphic sarcoma

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