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Aortic Valve Endocarditis
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In: Perspectives in Aortic Valve Disease ISBN: 978-1-53618-769-4
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Editor: Giovanni Concistrè © 2020 Nova Science Publishers, Inc.
Chapter 7
NEOPLASTIC DISORDERS INVOLVING
THE AORTIC VALVE
Angela Pucci
1,*
, Alessandra Burini
and Cristina Zucchinetti
1
Histopathology Department, University Hospital of Pisa
2
3
Sant’Anna School of Advanced Studies; Pisa, Italy
University of Pisa, Faculty of Medicine and Surgery, Pisa, Italy
ABSTRACT
Aortic valve tumors are rare and mainly represented by benign and small masses, most commonly papillary fibroelastoma followed by myxoma. Malignant primary tumors are exceptionally reported and are mainly represented by sarcomas. The aortic valve may also present tumor-like lesions and pseudo-tumors, including thrombi, Lambl’s excrescences and endocarditic vegetations. Although most aortic tumors are benign, small and often asymptomatic lesions, they may have dramatic clinical consequences, thus requiring surgical excision: embolic phenomena, outflow tract or coronary ostium occlusion. They may be incidentally discovered by echocardiography – the main diagnostic tool for cardiac masses. Computed tomography (CT) and cardiac magnetic resonance (CMR) may give additional information on tumor size, shape, location and characteristics, but definitive and differential diagnoses always require histology.
Keywords: cardiac tumors, magnetic resonance imaging, computed tomography
ABBREVIATIONS
CT computed tomography EMA epithelial membrane antigen FISH fluorescence in situ hybridisation
2,3
, Enrica Manzato
2,3
2,3
Corresponding Author’s Email: angelapucci@libero.it.
Angela Pucci, Alessandra Burini, Enrica Manzato et al.
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H&E hematoxylin/eosin IE infectious endocarditis IMT inflammatory myofibroblastic tumor LE Lambl’s excrescences CMR cardiac magnetic resonance MRI magnetic resonance imaging NBTE non-bacterial thrombotic endocarditis PAS periodic acid-Schiff PCR polymerase chain reaction PFE papillary fibroelastoma RT-PCR retro transcription-polymerase chain reaction TEE transesophageal echocardiography TTE transthoracic echocardiography
INTRODUCTION
Primary cardiac tumors are rare, with a prevalence at autopsy of 0.001-0.03% versus 1.5-
2.1% of secondary (metastatic) tumors. Valve tumors account for less than 10% of the heart tumors [1–5]. Although most cardiac valve tumors are benign and of small dimensions, they may have severe clinical sequelae and require surgical resection [1, 6, 7].
Cardiac valve tumors are more likely to occur in male (79% of cases) and adult patients,
the average age being 52 years (range, 2 to 88 years). Each of the four cardiac valves may be involved with approximately equal frequency [4, 6, 7]. They are more commonly asymptomatic, with overt symptoms in about 38% of patients, depending also upon a variable risk of embolism, and including cardiac (angina, acute myocardial infarction, heart failure, syncope and even sudden death) and neurological (transitory ischemic attack, stroke, amaurosis) symptoms, but also mesenteric, renal, splenic or limb ischemia.
The most common aortic valve tumors are benign: papillary fibroelastoma (PFE),
followed by myxoma, fibroma and hemangioma. Malignant tumors are exceptionally reported and almost exclusively represented by sarcomas and lymphomas, but both of them are more frequently found in the right side of the heart [5]. Average tumor size is 1.15 cm (range: 0.3 to 7 cm). A few cases have also been reported of secondary involvement of the aortic valve by tumors originating in different cardiac structures or other organs/tissues [1, 3, 5]. Although most tumor masses are nowadays identified in vivo by imaging techniques – first echocardiography for diagnosis, followed by computed tomography (CT) and cardiac magnetic resonance (CMR) for better definition of tumor characteristics – the definitive diagnosis of aortic valve tumors requires histology, which also represents the gold standard for the differential diagnosis of cardiac tumors [5].