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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.

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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].
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