Cardiac Tumors - UpToDate

Cardiac tumors - UpToDate 20/11/20 14.
09
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Cardiac tumors
Authors: William H Gaasch, MD, Thomas J Vander Salm, MD
Section Editor: Wilson S Colucci, MD
Deputy Editors: Sonali Shah, MD, Susan B Yeon, MD, JD, FACC
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Oct 2020. | This topic last updated: May 13, 2020.
INTRODUCTION
Primary cardiac tumors are extremely rare [1]. As an example, in one series of over 12,000
autopsies, only seven were identified, for an incidence of less than 0.1 percent [2]. By
comparison, metastatic involvement of the heart is over 20 times more common and has
been reported in autopsy series in up to one in five patients dying of cancer [2-5].
Cardiac tumors may be symptomatic or found incidentally during evaluation for a

seemingly unrelated problem or physical finding. In symptomatic patients, a mass can
virtually always be detected by echocardiography, magnetic resonance imaging, and/or
computed tomography. Because symptoms may mimic other cardiac conditions, the
clinical challenge is to consider the possibility of a cardiac tumor so that the appropriate
diagnostic test(s) can be conducted.
CLINICAL MANIFESTATIONS
The specific signs and symptoms of cardiac tumors generally are determined by the
location of the tumor in the heart and not by its histopathology [6].
Mechanisms of symptom production — Cardiac tumors may cause symptoms through a

variety of mechanisms:
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Cardiac tumors - UpToDate 20/11/20 14.09
● Embolization, which is usually systemic but can be pulmonic. Aortic valve and left
atrial tumors were associated with greatest risk of embolization [7].
● Obstruction of the circulation through the heart or heart valves, producing symptoms
of heart failure.
● Interference with the heart valves, causing regurgitation.
● Direct invasion of the myocardium, resulting in impaired left ventricular function,

arrhythmias, heart block, or pericardial effusion with or without tamponade.
● Invasion of the adjacent lung may cause pulmonary symptoms and may mimic
bronchogenic carcinoma [8].
● Constitutional or systemic symptoms.
Left atrial tumors — Tumors arising in the left atrium tend to grow into the atrial lumen
and cause symptoms by obstructing blood flow or creating mitral regurgitation. Left atrial
tumors thus may simulate mitral valve disease and produce heart failure and/or
secondary pulmonary hypertension. (See "Clinical manifestations and diagnosis of
rheumatic mitral stenosis" and "Treatment and prognosis of pulmonary arterial
hypertension in adults (group 1)".)
Commonly observed symptoms and signs include dyspnea, orthopnea, paroxysmal

nocturnal dyspnea, pulmonary edema, cough, hemoptysis, edema, and fatigue.
Symptoms may be worse in certain body positions due to motion of the tumor within the
atrium. On physical examination, a characteristic "tumor plop" may be heard early in
diastole. (See "Auscultation of cardiac murmurs in adults".)
In addition to interfering with the circulation, left atrial tumors may release tumor
fragments or thrombi into the systemic circulation. The most serious complications of
such embolization are neurologic. This was illustrated by a series of 74 consecutive
patients with atrial myxomas from the Mayo Clinic [9]. Central nervous system
complications were identified in nine patients (12 percent), and in seven of these the
neurologic symptoms were the initial manifestation of the myxoma. Two of these nine
patients had evidence of systemic myxomatous tumor embolization in addition to their
neurologic symptoms.
Benign myxomas are the most common tumors arising in the left atrium (see 'Myxomas'
below). However, other benign and malignant tumors can simulate myxomas and should
be considered in the differential diagnosis [10].
Right atrial tumors — Tumors arising in the right atrium grow into the atrial lumen and
obstruct blood flow, producing hemodynamic changes that are similar to those seen with
tricuspid stenosis.
Typical cardiovascular signs and symptoms are those of right heart failure (ie, fatigue,
peripheral edema, hepatomegaly, ascites, and prominent "a waves" in the jugular veins).
On physical examination, a diastolic murmur has been described, which is similar to the
"tumor plop" heard with left atrial myxomas. (See "Tricuspid stenosis".)
In addition to obstructing circulation through the right side of the heart, tumor fragments
may be released into the pulmonary circulation, causing symptoms consistent with
pulmonary emboli [11]. Right atrial hypertension can result in shunting of venous blood
into the systemic circulation if a patent foramen ovale (or atrial septal defect) is present,
resulting in hypoxemia or systemic emboli [12,13].
Myxomas are the most common tumors of the right atrium. However, sarcomas and, in
particular, angiosarcomas, have been reported to arise in the right atrium.
Right ventricular tumors — Lesions arising in the right ventricle most commonly
interfere with filling and/or outflow from the right ventricle, resulting in right-sided heart
failure.
Typical signs and symptoms may include peripheral edema, hepatomegaly, ascites,
shortness of breath, syncope, and sudden death. Tumors arising in the right ventricle can
be misdiagnosed as pulmonic stenosis, restrictive cardiomyopathy, or tricuspid
regurgitation. (See "Clinical manifestations and diagnosis of pulmonic stenosis in adults"
and "Idiopathic restrictive cardiomyopathy", section on 'Clinical presentation'.)
Left ventricular tumors — Tumors arising in the left ventricle may be intramural and
present with arrhythmias or conduction defects. Alternately, left ventricular tumors may
be intracavitary and present with systemic embolization or outflow obstruction. Syncope
or left ventricular failure may be observed. (See "Clinical manifestations and diagnosis of
aortic stenosis in adults".)
DIAGNOSTIC EVALUATION
The goals of the initial evaluation are to ascertain whether or not a cardiac tumor is
present, the location of the lesion within the heart, and, to the extent possible, whether a
tumor is benign or malignant. This information is vital in planning further evaluation and
management.
Echocardiography, cardiac magnetic resonance imaging (CMR), and cardiac computed

tomography (CCT) provide complementary information to address these questions. Most
adults having heart operations of any kind will have coronary angiography as part of the
routine preoperative evaluation. For those with epicardial tumors, coronary angiography
is specifically required to assess the location of the tumor's nutrient vessels and any that
are parasitized by the tumor.
Echocardiography — Echocardiography is widely available and provides a simple,

noninvasive technique for the initial evaluation. Echocardiography images both the
myocardium and the cardiac chambers and can usually identify the presence of a mass
and its mobility. In addition, echocardiography may provide information about any
obstruction to the circulation, as well as the likelihood that the tumor could be a source of
emboli. (See "Echocardiographic evaluation of the atria and appendages", section on 'Left
atrial masses and tumors'.)
Although transthoracic echocardiography is simpler and usually can identify a tumor,

transesophageal echocardiography (TEE) may be more informative. The superior
diagnostic utility of TEE is due to the proximity of the esophagus to the heart, the lack of
intervening lung and bone, and the ability to use high-frequency imaging transducers that
afford superior spatial resolution [14]. (See "Transesophageal echocardiography:
Indications, complications, and normal views".)
Cardiac magnetic resonance imaging and computed tomography — Although both

CMR [15-18] and CCT [19,20] provide noninvasive, high-resolution images of the heart,
CMR generally is preferred. In addition to furnishing detailed anatomic images, the T1-
and T2-weighted sequences reflect the chemical microenvironment within a tumor,
thereby offering clues as to the type of tumor that is present [6,17,18,21,22]. However, CCT
scanning is still useful when CMR is not immediately available or is contraindicated. An
excellent pictorial review of many cardiac tumors and comparison of CMR and CCT
scanning has been published [23]. (See "Clinical utility of cardiovascular magnetic
resonance imaging" and "Cardiac imaging with computed tomography and magnetic
resonance in the adult".)
The utility of information derived from echocardiography, CMR, and/or CCT is illustrated
by several examples:
● The location of myxomas within the atrial lumen facilitates their diagnosis by
echocardiography ( movie 1 and movie 2 and movie 3 and movie 4 and
movie 5 and movie 6). In most cases, TEE provides better visualization of the
tumor ( image 1A-B).
● In five series totaling approximately 2000 patients, characteristic radiographic

appearances and tissue densities accurately facilitated the diagnosis of myxomas,
other benign tumors, and sarcomas [17,24-27].
● Papillary fibroelastomas, the second most common benign cardiac tumors,

characteristically are pedunculated and mobile and flutter or prolapse with cardiac
motion when imaged by echocardiography [28,29]. (See 'Papillary fibroelastomas'
below.)
● Benign lesions such as fibromas and lipomas also have distinctive appearances on
echocardiography, CCT, and CMR [30].
● The combination of echocardiography plus CMR or CCT may be useful in

differentiating thrombus from tumor in lesions appearing to arise on a heart valve
[31].
PET scan — Positron emission tomography (PET) has been useful in identifying cardiac
involvement in patients with metastatic tumors [32-35], atrial myxoma [36], or lipomatous
septal hypertrophy [37].
Coronary angiography — Mapping the blood supply of tumors arising from the
epicardial surfaces must be performed with coronary angiography [6]. This information is
vital to the success of excising such tumors. Significant involvement of coronary arteries
with tumor may require resection and grafting of such arteries.
Transvenous biopsy — Limited data are available on the risks and benefits of
transvenous biopsy of suspected cardiac tumors. Because myxomas may embolize,

transvenous biopsy is not generally warranted if the appearance is typical on noninvasive
imaging. Biopsy is considered reasonable for other cardiac tumors if potential benefits are
deemed sufficient to outweigh potential risks. (See "Endomyocardial biopsy".)
Summary — The information obtained from echocardiography and CMR or CCT can
confirm the presence of a cardiac tumor and its location within the heart and may provide
an initial indication of the type of tumor. Transvenous biopsy may be helpful in certain
situations. In general, the information from noninvasive imaging is sufficient to make a
decision regarding the need for surgery, where a definitive histologic diagnosis can be
established.
BENIGN TUMORS
Over 75 percent of primary cardiac tumors are benign [38-43]. In adults, the majority of
benign lesions are myxomas; other common benign lesions include papillary
fibroelastomas and lipomas. In children, rhabdomyomas and fibromas are the most
common; malignant tumors are very rare [44].
Myxomas — Myxomas are the most common primary cardiac neoplasm. Histologically,
these tumors are composed of scattered cells within a mucopolysaccharide stroma. The
cells originate from a multipotent mesenchyme that is capable of neural and endothelial
differentiation [45]. Myxomas produce vascular endothelial growth factor, which probably
contributes to the induction of angiogenesis and the early stages of tumor growth [46,47].
Macroscopically, typical myxomas are pedunculated and gelatinous in consistency; the

surface may be smooth, villous, or friable. Tumors vary widely in size, ranging from 1 to 15
cm in diameter, and weigh between 15 and 180 g [48]. Approximately 35 percent of
myxomas are friable or villous, and these tend to present with emboli. Larger tumors are
more likely to have a smooth surface and to be associated with cardiovascular symptoms.
Clinical manifestations — The cardiovascular manifestations depend upon the

anatomic location of the tumor. Approximately 80 percent of myxomas originate in the left
atrium and most of the remainder are found in the right atrium [6,11,49-51]. (See 'Left
atrial tumors' above and 'Right atrial tumors' above.)
In addition to their cardiovascular effects, patients with myxomas frequently have

constitutional symptoms (eg, weight loss, fever) and laboratory abnormalities that
suggest the presence of a connective tissue disease [52]. Although the etiology of these
symptoms is not fully understood, the production of various cytokines and growth factors
by the tumor may contribute to these clinical and laboratory abnormalities [47,53].
The relative frequencies of different signs and symptoms associated with left atrial
myxomas are illustrated by a series of 112 patients, 72 of whom were women [48]:
● Cardiovascular symptoms were present in 67 percent. Most commonly, these

resembled symptoms of mitral valve obstruction and were frequently associated with
electrocardiographic evidence of left atrial hypertrophy. Although auscultatory
abnormalities were found in 64 percent, the classic "tumor plop" was identified in only
15 percent. (See 'Left atrial tumors' above.)
● Evidence of systemic embolization was present in 29 percent of patients, and 20

percent had neurologic deficits. Despite the greater frequency of myxomas in women,
men were more likely to have evidence of embolization. With myxomas, the incidence
of embolization is associated with smaller size (≤4.5 cm) and softer tumors [54].
● Constitutional symptoms (eg, fever, weight loss) were seen in 34 percent of patients.
Laboratory abnormalities (eg, anemia and elevations in the erythrocyte sedimentation
rate, C-reactive protein, or globulin level) were present in 37 percent, usually those
with systemic symptoms.
Other large series of patients with myxomas have also included a predominance of
women (60 to 70 percent) and have reported similar incidences of cardiovascular, embolic,
and constitutional symptoms [11,50,51,55,56].
Carney complex — The Carney complex is an inherited, autosomal dominant

disorder characterized by multiple tumors, including atrial and extracardiac myxomas,
schwannomas, and various endocrine tumors. The cardiac myxomas generally are
diagnosed at an earlier age than sporadic myxomas and have a higher tendency to recur
[57].
Patients with Carney complex also have a variety of pigmentation abnormalities, including
pigmented lentigines and blue nevi on the face, neck, and trunk. The Carney complex is
discussed elsewhere. (See "Cushing's syndrome due to primary pigmented nodular
adrenocortical disease", section on 'Carney complex (CNC)'.)
The Carney complex must be distinguished from other syndromes associated with Carney
with which it may be confused. These include the Carney Stratakis syndrome and the
Carney triad, neither of which include cardiac tumors [58]. (See "Epidemiology,
classification, clinical presentation, prognostic features, and diagnostic work-up of
gastrointestinal stromal tumors (GIST)", section on 'Pediatric GIST'.)
Treatment and prognosis — Once a presumptive diagnosis of myxoma has been

made on imaging studies, prompt resection is required following routine preoperative
evaluation because of the risk of embolization or cardiovascular complications, including
sudden death [50,56,59]. The results of surgical resection are generally very good, with
most series reporting an operative mortality rate under 5 percent [48,50,51,55,56,60,61].
Cardiac autotransplantation (with atrial reconstruction) or transplantation are potential
options for treatment of recurrent atrial myxoma [62,63].
Postoperative recovery is generally rapid. However, atrial arrhythmias or atrioventricular

conduction abnormalities were present postoperatively in 26 percent of patients in one
series [48]. In addition, patients are at risk for recurrence of the myxoma, which may occur
in 2 to 5 percent of cases, or the development of additional lesions [48,64]. Recurrence is
more common in patients whose primary tumor was multicentric [54]. Development of a
second primary myxoma may be more common in patients with a family history of
myxoma [60].
Papillary fibroelastomas — Papillary fibroelastomas are the second most common

primary cardiac tumor in adults [65]. Their appearance is often compared to sea
anemones, with frond-like arms emanating from a stalked central core ( picture 1).
The clinical spectrum of fibroelastomas can be illustrated by two reports combining

information from 887 patients [29,65]:
● Demographics – Fifty-five percent of patients were male. The mean age at detection
was 60 years, and 29 percent were 70 years of age or older.
● Size, location, and number – Tumors varied from 2 to 70 mm in size with a mean of 9
mm. Over 80 percent of fibroelastomas were found on the heart valves, usually on the
left side of the heart (aortic 36 percent, mitral 29 percent, tricuspid 11 percent, and
pulmonic 7 percent), while the remaining lesions were scattered throughout the atria
and ventricles. Multiple tumors were present in 9 percent of patients.
● Clinical features – Symptoms usually were caused by embolization, either of the tumor
itself or thrombus. The most common clinical presentation was stroke or transient
ischemic attack, followed by angina, myocardial infarction, sudden death, heart
failure, syncope or presyncope, and systemic or pulmonary embolic events.
Approximately 30 percent of papillary fibroelastomas were asymptomatic and diagnosed

incidentally, either by echocardiography at cardiac surgery or at autopsy [29,65].
Treatment — A presumptive diagnosis of papillary fibroelastoma can usually be made

on echocardiography (generally transesophageal echocardiography). While some
recommend surgery for all patients because of the risk of embolization and associated
morbidity [66-70], others have suggested that careful observation is an acceptable option
for asymptomatic patients as long as the tumor remains small and nonmobile [29].
Surgery is recommended for patients who have had embolic events or complications
directly related to tumor mobility (eg, coronary ostial occlusion) and those with highly
mobile or large (≥1 cm) tumors [29,65]. Recurrence of cardiac papillary fibroelastoma
following surgical resection has not been reported.
Rhabdomyomas — Rhabdomyomas develop almost exclusively in children, mostly before

the age of one year, and approximately 80 to 90 percent are associated with tuberous
sclerosis [71-73]. With increasing use of ultrasound and improvements in technique and
magnetic resonance imaging (MRI), these tumors are being detected with increasing
frequency, even in the prenatal period [71,73-76]. Rhabdomyomas are usually found in the
ventricular walls or on the atrioventricular valves. (See "Tuberous sclerosis complex:
Genetics, clinical features, and diagnosis", section on 'Clinical features'.)
Most rhabdomyomas regress spontaneously, and resection is usually not required unless
a child is symptomatic [72,76-79]. Symptoms, if present, are caused by obstruction of
blood flow through the heart or consist of rhythm disturbances such as heart block of
ventricular tachycardia [72,78].
Fibromas — Although uncommon, fibromas are nevertheless the second most common
pediatric cardiac tumor and can also occur in adults [49,80-82]. Histologically, these are
similar to fibromas arising elsewhere in the body. Fibromas usually arise in the ventricular
muscle and may become quite large. Unlike rhabdomyomas, fibromas do not regress
spontaneously. They arise approximately five times more frequently in the left ventricle
than the right ventricle [49].
Symptoms of heart failure (eg, dyspnea and fatigue) are the most common symptoms of
obstruction of blood flow or interference with valvular function. Ventricular arrhythmias
(with risk of sudden cardiac arrest) [83,84], myocardial dysfunction, and conduction
disturbances also occur. Echocardiography supplemented with cardiac magnetic
resonance imaging (CMR) or cardiac computed tomography (CCT) confirms the diagnosis.
Symptomatic tumors should be completely resected. Limited observational data suggest
that complete resection may cure the arrhythmia [84]. Complete resection of very large
tumors may require cardiac transplantation.
Teratoma — Teratomas are tumors of embryonic origin derived from two or three
germinal layers. Cardiac teratoma is a rare, generally benign tumor with most reported
cases presenting as fetal or neonatal tumors; in adults they are estimated to constitute
less than 1 percent of cardiac tumors [85]. Nearly all cardiac teratomas arise within the
pericardium, with the remainder in the myocardium [86-91].
Fetal and neonatal intrapericardial teratoma — Although these tumors are

generally benign, they tend to grow rapidly (ie, significant growth over a few weeks) and
can have serious mechanical consequences either by causing tamponade or through
direct pressure on the heart with consequent reduced cardiac output, fetal hydrops, and
death. Intrapericardial teratomas are typically located in the region of the pericardial
reflection at the junction of the ascending aorta and right atrial appendage, which leads to
compression of the right side of the heart as the teratoma grows [92]. Thus, there is a risk
of death in utero or immediately after birth.
Treatment therefore requires timely detection and resection [92]. When a small suspected
teratoma is detected, frequent serial assessment is required to detect changes in tumor
size and cardiac output prior to the onset of hydrops. An increase in tumor size and a
declining or abnormally low cardiac output are indications for tumor resection, ideally
prior to development of signs of hydrops. The tumor can be removed by fetal tumor
excision, an ex utero intrapartum therapy approach (which involves uterine hysterotomy,
continued uteroplacental support during surgical tumor excision, followed immediately by
delivery), or by early postnatal surgery, with timing dictated by tumor size and the
patient's hemodynamic condition [88,90,92,93]. Because teratomas usually have a single
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blood supply, are well encapsulated, and are not invasive, properly timed tumor surgery is
typically straightforward and successful.
By contrast, other approaches may not be therapeutic. For example, drainage of the cystic
component of a teratoma may not relieve tamponade and will not stop tumor growth.
Pericardiocentesis may not relieve tamponade if tumor mass is not removed.
Other cardiac teratomas — Scant reports are available on adult cases of benign
cardiac teratoma, which are predominantly pericardial [85,94]. In adults, cardiac teratoma
may grow slowly and may be detected as an incidental finding in an asymptomatic
patient. Symptoms such as chest pain or dyspnea may develop due to associated
pericardial effusion.
Intramyocardial teratomas have been rarely diagnosed in newborns, children, and adults.
These tumors may cause heart failure or an arrhythmia [94].
The primary treatment for benign cardiac teratoma is surgical excision [85,95].
Purkinje cell tumors/hamartomas — These tumors consist of small, flat sheets of cells
most frequently located in the left ventricle and on the endocardial and epicardial surfaces
[96,97]. As such, they are undetectable by echocardiographic or radiologic techniques.
These are usually tumors of young children and present with incessant ventricular
tachycardia [97]. Electrocardiograms often demonstrate a bundle branch pattern (right
bundle branch block when the tumor is in the left ventricle). Electrophysiologic studies can
localize the tumors, facilitating surgical excision.
Lipomas — Lipomas and fibrolipomas are characterized by a predominance of benign

fatty cells. Approximately half of these tumors occur in the subendocardial region with the
remainder evenly divided between the myocardial and subepicardial regions. They may
also occur on valves [98,99]. Although most are no more than a few centimeters in size,
lipomas as large as 4.8 kg have been reported [100].
Symptoms, when present, are generally related to local tissue encroachment

(arrhythmias, conduction block, sudden death), although valvular tumors can cause
insufficiency and symptoms of heart failure [101]. The diagnosis can be made with
echocardiography and the distinctive fat pattern seen on MRI. Because of the symptoms
they cause and their progressive growth, myocardial lipomas require resection.
Pericardial lipomas are typically an incidental finding and clinically insignificant. Rarely, a
pericardial lipoma can assume gigantic proportions and its appearance on a chest
radiograph may be mistaken for a huge pericardial effusion or massive cardiomegaly (
image 2 and image 3). Benign pericardial lipomas can infiltrate the myocardium. If
the ventricular septum is invaded, communication between the pericardial space and the
right ventricular cavity may result.
Lipomatous hypertrophy of the interatrial septum — Lipomatous hypertrophy of the

interatrial septum is an exaggerated growth of normal fat existing within the septum and
is not a true tumor. Rather, it is a developmental disorder caused by expansion of adipose
tissue trapped in the interatrial septum during embryogenesis [102]. The septal
hypertrophy may be as large as 2 cm in thickness and is seen primarily in older patients
and in those who are obese [103,104]. It has been suggested that this disorder is
associated with the presence of coronary artery disease in proportion to the degree of
atrial septal thickness [105].
Lipomatous hypertrophy of the interatrial septum is indistinguishable from lipoma except

that the former occurs in the atrial septum with a typical distribution (generally sparing
the fossa ovalis). In the absence of symptoms of atrial arrhythmias, heart block, or rare
vena caval obstruction, they do not require resection [106].
Other pseudoneoplasms — There are other pseudoneoplasms (like lipomatous

hypertrophy) as well [102]. These include inflammatory myofibroblastic tumor,
hamartoma of mature cardiac myocytes, calcified amorphous tumor [107], and
mesothelial/monocytic incidental cardiac excrescences. But unlike lipomatous
hypertrophy, these four tumors require resection to distinguish them from neoplasms or
to prevent embolization or obstruction of blood flow [102]. This is also true of the rare
intracardiac blood cyst [108].
TUMORS THAT MAY BE EITHER BENIGN OR MALIGNANT
Paragangliomas — Paragangliomas are neuroendocrine tumors that can be either

benign or malignant and can be hormonally active or inactive. In tumors not producing
catecholamines, symptoms are due to cardiac compression or tamponade. By contrast,
cardiac paragangliomas that are hormonally active primarily produce norepinephrine and
may cause systemic symptoms (eg, headache, sweating, tachycardia, hypertension) [109].
(See "Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology".)
Paragangliomas do not occur commonly in the chest, but when they do, the hormonally
inactive tumors are more frequent in the pericardium, while hormonally active tumors
(pheochromocytomas) more frequently arise elsewhere in the thorax [110].
Paragangliomas may be localized with echocardiography. Their extreme vascularity
creates a characteristic magnetic resonance imaging (MRI) appearance [93,111]. Coronary
angiography is required to plan the operative resection. (See 'Coronary angiography'
above.)
Both benign and malignant paragangliomas occurring within the pericardium parasitize
the cardiac blood supply and are, as a consequence, very difficult to excise [111-113]. All
intrapericardial paragangliomas require resection. Complete resection may be difficult but
is usually possible. Cardiopulmonary bypass and even circulatory arrest may be required
because of the high degree of vascularity or to moderate the extreme hypertension
possible from tumor manipulation or hormonally active tumors [113,114]. If complete
resection is not possible, cardiac transplantation may be required [115]. As is true of all
pheochromocytoma resections, preoperative and intraoperative adrenergic blockade
must be employed. (See "Paragangliomas: Treatment of locoregional disease" and
"Paraganglioma and pheochromocytoma: Management of malignant disease".)
Mesothelioma — Although most mesotheliomas arise in the pleura, these tumors can
also arise from the pericardium, where they are usually malignant [116-120]. Although a
causal relationship between asbestos exposure and pleural mesotheliomas is well
established, the relationship between asbestos exposure and pericardial mesothelioma is
less certain.
Mesotheliomas arising in the pericardium produce tamponade and constriction [116-119].

These tumors will be seen with echocardiography, computed tomography scan, MRI, and
sometimes by chest radiograph [116-118]. Pericardiocentesis may yield a cytologic
diagnosis [117].
More rarely, mesotheliomas may arise as benign tumors of the atrioventricular (AV) node
where they may produce heart block [121,122]. Diagnosis of the AV nodal tumors causing
heart block can be confirmed with echocardiography.
Resection is the treatment of choice for mesothelioma, but the prognosis with malignant
pericardial mesotheliomas is very poor [117,118]. The addition of radiation and/or

chemotherapy has been attempted but has not been shown to be of value.
PRIMARY MALIGNANT TUMORS
Malignant tumors constitute approximately 15 percent of primary cardiac tumors [39].

Sarcomas are the most common, although other tumor types have been reported.
Sarcomas — Virtually all types of sarcomas have been reported in the heart [6,123-128].
Cardiac sarcomas are extremely rare, and for most types, only isolated case reports have
been described.
As with benign lesions, the clinical presentation is largely determined by the location of
the tumor rather than its histopathology. The diagnostic approach relies upon
echocardiography, magnetic resonance imaging, and computed tomography to define the
presence of a tumor and its anatomic relationship to normal structures ( movie 7).
The most frequently described sarcomas include (see "Clinical presentation,

histopathology, diagnostic evaluation, and staging of soft tissue sarcoma", section on
'Histopathology'):
● Angiosarcomas – Angiosarcomas are composed of malignant cells that form vascular

channels. The pathology of angiosarcomas may overlap with Kaposi sarcoma, which
can also involve the myocardium [129]. Angiosarcomas arise predominantly in the
right atrium [129,130]. Epithelioid hemangioendothelioma, another sarcoma of
vascular origin, has also been reported [131]. Of sarcomas, 40 percent are
angiosarcomas, and 10 percent are spindle sarcomas [132].
● Rhabdomyosarcomas – Rhabdomyosarcomas constitute as many as 20 percent of all

primary cardiac sarcomas [133]. These tumors are most commonly found in adults,
although they have also been described in children. Multiple sites of myocardial
involvement are common, and there is no predominant localization within any area of
the heart.
● Fibrosarcomas and undifferentiated sarcomas – Fibrosarcomas and

undifferentiated/unclassified soft tissue sarcomas (which were formerly included in a
broad category termed malignant fibrous histiocytomas or high-grade pleomorphic
sarcomas [134]) are white, fleshy ("fish flesh") tumors that are composed of spindle
cells and may have extensive areas of necrosis and hemorrhage [135,136]. These
tumors tend to extensively infiltrate the myocardium.
● Leiomyosarcomas – Leiomyosarcomas are spindle-celled, high-grade tumors that

arise more frequently in the left atrium [137]. These sarcomas have both a high rate
of local recurrence and systemic spread.
● Other types include liposarcoma and synovial sarcoma [127,138].
Treatment and prognosis — In general, sarcomas proliferate rapidly and cause death
through widespread infiltration of the myocardium, obstruction of blood flow through the
heart, and/or distant metastases. Although complete resection is the treatment of choice,
most patients develop recurrent disease and die of their malignancy even if their tumor
can be completely resected [55,61,132,139,140]. The median survival is typically 6 to 12
months [123,127], although long-term survival has been reported with complete resection
[123,126,141-143], and patients with low-grade sarcomas may have a better prognosis
[126].
Neoadjuvant or adjuvant chemotherapy has been used in an effort to improve on the poor
results with resection alone. However, most of the published experience consists of
anecdotal case reports or retrospective reviews [39,141,143-153], and no randomized
trials have been conducted. Rhabdomyosarcomas may have a better outcome with
chemotherapy. (See "Rhabdomyosarcoma in childhood, adolescence, and adulthood:
Treatment".)
Alternative strategies such as cardiac transplantation and cardiac autotransplantation are

being explored. Radiation has been used infrequently and primarily as a treatment of
metastases [138].
Studies illustrating the prognosis of these sarcomas are discussed below:
● In a 95-patient series of malignant primary cardiac tumors, all of whom had surgical
treatment with 60 percent having preoperative adjunctive chemotherapy, only two
patients lived beyond five years [132]. In a 40-year study of over 500 primary
malignant cardiac tumors, the overall survival rates at one, three, and five years were
46, 22, and 17 percent, whereas with sarcomas the survival rates were 47, 16, and 11
percent, respectively [154].
● In a 34-patient series treated at the Mayo Clinic over a 32-year period [125], the
median survival was significantly longer when a complete surgical resection was
possible (17 versus 6 months when complete resection was not possible). Similarly,
the median survival was longer in those who did not have metastases on presentation
(15 versus 5 months in those with detectable metastases at diagnosis). Larger series
of 95 patients and over 500 patients have shown similar and very poor long-term
survival [132,154].
The poor results with surgical resection have led to occasional attempts to treat patients
with cardiac transplantation if extracardiac disease is not present [149,155-159]. Most of
these patients have undergone chemotherapy and radiation prior to transplantation. In
the largest series, results of cardiac transplantation in patients with malignant tumors
(most of which were sarcomas) were evaluated in a review of 21 cases [155]. Although
mean survival was only 12 months, seven patients were free of recurrent malignancy at a
mean follow-up of 27 months.
An alternative treatment, cardiac autotransplantation, has shown promise. In these cases,

the heart is excised, the tumor is resected ex vivo, and the heart is reconstructed before
being reimplanted. The advantage of this procedure is the increased ease with which
major resection and reconstruction can be performed, while at the same time avoiding
the need for antirejection treatment [160,161].
Another promising adjunct in operative therapy is to plan the complex operative strategy
(in a Schwannoma) by creating a three-dimensional printer reproduction of the heart and
tumor [162].
Other primary cardiac tumors — Primary lymphomas arising in the myocardium have
been reported. In a review of 40 cases identified from the literature between 1995 and
2002, the outlook was generally poor [163]. However, 38 percent of cases achieved a
complete response with systemic therapy. At least some of these responses may be
durable [163-165].
Other tumors may also arise in the heart, including paragangliomas [166,167] and
extramedullary plasmacytomas [168-170].
SECONDARY CARDIAC TUMORS
In contrast to primary malignant cardiac tumors, metastatic involvement of the heart is

relatively common. As an example, in one of the largest autopsy series of over 1900
patients dying of cancer, 8 percent had metastatic disease involving the heart [5]. Cardiac
involvement may arise from hematogenous metastases, direct invasion from the
mediastinum, or tumor growth into the vena cava and extension into the right atrium
[171].
Malignant melanomas are particularly likely to metastasize to the heart [129,168,172,173].

Other solid tumors commonly associated with cardiac involvement include lung cancer,
breast cancer, soft tissue sarcomas, renal carcinoma, esophageal cancer, hepatocellular
carcinoma, and thyroid cancer [174]. There is also a high prevalence of secondary cardiac
involvement with leukemia and lymphoma.
Cardiac or pericardial metastasis should be considered whenever a patient with known

malignancy develops a pericardial effusion; any cardiovascular symptom; or signs such as
a new or changing heart murmur, electrocardiographic conduction delay, or arrhythmia.
The development of cardiomegaly on chest radiograph should suggest pericardial
effusion. Emboli thought to originate in the heart should also raise the possibility of
cardiac involvement with tumor. Cardiac metastases rarely may be the first manifestation
of malignant disease [175].
The specific symptoms will reflect the site of cardiac involvement in a manner analogous
to primary cardiac tumors. The diagnostic evaluation is the same as that for primary
cardiac tumors and relies upon echocardiography, magnetic resonance imaging, and
computed tomography to ascertain the extent of cardiac involvement. In very carefully
selected patients, resection of cardiac metastases has been used to provide symptom
palliation and prolong life [129,176,177].
Other causes of cardiac symptoms must also be considered. In particular, metastatic

disease must be distinguished from the cardiotoxicity that may be associated with
chemotherapeutic agents, particularly anthracyclines. (See "Clinical manifestations,
monitoring, and diagnosis of anthracycline-induced cardiotoxicity".)
SUMMARY
● Tumors involving the heart may cause symptoms by obstruction of circulation,

interference with heart valves, direct invasion of the myocardium, invasion of adjacent
lung, or embolization. Constitutional or flu-like symptoms may be present. Except for
tumors with constitutional symptoms, the signs and symptoms of a cardiac tumor are
generally determined by the location of the tumor in the heart rather than by its
histopathology. (See 'Clinical manifestations' above.)
● If a cardiac tumor is suspected, imaging procedures are used to determine whether

or not a mass is present and where the tumor is located within the heart.
Echocardiography is the simplest technique for such evaluation; cardiac magnetic
resonance imaging and cardiac computed tomography provide more detailed
information. Tumors that occur from or invade the epicardial surface of the heart
require coronary angiography preoperatively to define distortion of the coronary
arteries and determine coronary blood supply of the tumor. (See 'Diagnostic
evaluation' above.)
● Over 75 percent of cardiac tumors are benign, and the majority of these are
myxomas. Approximately 80 percent of myxomas arise in the left atrium and most of
the remainder are found in the right atrium. Myxomas are managed with prompt
surgical resection because of the risk of embolization or other cardiovascular
complications. A number of other benign lesions may also occur. (See 'Myxomas'
above and 'Benign tumors' above.)
● Primary malignant tumors of the heart are rare; most of these are sarcomas. Primary
sarcomas arising in the heart generally are rapidly progressive and cause death
through infiltration of the myocardium, by obstructing circulation, or by distant
metastases. When feasible, treatment is surgical, although most of these tumors
recur relatively rapidly. (See 'Sarcomas' above.)
● Metastatic involvement of the heart is relatively frequent and may result from
hematogenous spread, direct invasion, or tumor growth through the vena cava into
the right atrium. Cardiac or pericardial metastases should be considered whenever a
patient with known malignancy develops cardiovascular symptoms. As with primary
cardiac tumors, specific signs and symptoms reflect the site of cardiac involvement.
(See 'Secondary cardiac tumors' above.)
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Topic 4881 Version 32.0
GRAPHICS
Left atrial myxoma on transthoracic and transesophageal

echocardiography
A four chamber view from a transthoracic echocardiography (panel A) suggests a well

encapsulated mass within the left atrium (arrow). The consistence is fairly uniform
although there may be a few rarefactions. Transesophageal echocardiography (panel
B) shows the details of the tumor attachment to the lower limbus of the foraminal
portion of the interatrial septum. The implied irregularity on transthoracic
echocardiography are clearly seen cysts that are also typical of these tumors.
Graphic 60232 Version 2.0
Left atrial myxoma
The long axis precordial view from a transthoracic echocardiography shows the
tumor as a vague mass just behind the aortic root (panel A). The apical four
chamber view (panel B) shows the mass attached to the interatrial septum. A
transesophageal echocardiography (panels C and D) reveals that the myxoma is
highly mobile and at surgery, the tumor had the consistency of jelly and
fragmented easily.
Papillary fibroelastoma of mitral valve
Gross and microscopic pathology of a papillary fibroelastoma of the mitral valve.

The gross specimen (left) reveals multiple frond-like structures when photographed
underwater. Low (right, top) and high power (right, bottom) microscopic features
include dense elastin (black) at the core of each frond coated with collagen (red
pink) and lined by flat endocardial cells.
Reprinted from Journal of the American College of Cardiology, volume 30, Klarich, KW, Enriquez-
Sarano, M, Gura, GM, et al. Papillary ﬁbroelastoma: Echocardiographic characteristics for
diagnosis and pathogenesis correlation, p.784, Copyright 1997, with permission from the
American College of Cardiology.
http://www.elsevier.com/locate/jacc
http://www.sciencedirect.com
Pericardial lipoma on chest x-ray
A P-A chest x-ray (A) shows the border of the left ventricle (arrowhead) surrounded by a lower density border
of a pericardial lipoma (arrow). A lateral view (B) shows the posteriorly positioned low-density lipoma of the
pericardium (arrow).
P-A: posteroanterior.
Pericardial lipoma on CT scan
A CT scan through the mid-chest shows a large low-density pericardial lipoma (arrow in panel A). A CT
reformatted in the sagittal plane shows a lipoma posterior to the heart (arrow) and pressing against the
esophagus (arrowhead).
CT: computed tomography.
Contributor Disclosures
William H Gaasch, MD Nothing to disclose Thomas J Vander Salm, MD Nothing to disclose Wilson S
Colucci, MD Grant/Research/Clinical Trial Support: Merck [Heart failure]. Sonali Shah, MD Nothing to
disclose Susan B Yeon, MD, JD, FACC Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these
are addressed by vetting through a multi-level review process, and through requirements for
references to be provided to support the content. Appropriately referenced content is required of all
authors and must conform to UpToDate standards of evidence.
Conflict of interest policy

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Cardiac tumors - UpToDate 20/11/20 14.

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Cardiac tumors may be symptomatic or found incidentally during evaluation for a

Mechanisms of symptom production — Cardiac tumors may cause symptoms through a

● Interference with the heart valves, causing regurgitation.

● Direct invasion of the myocardium, resulting in impaired left ventricular function,

● Constitutional or systemic symptoms.

Commonly observed symptoms and signs include dyspnea, orthopnea, paroxysmal

Echocardiography, cardiac magnetic resonance imaging (CMR), and cardiac computed

Echocardiography — Echocardiography is widely available and provides a simple,

Although transthoracic echocardiography is simpler and usually can identify a tumor,

Cardiac magnetic resonance imaging and computed tomography — Although both

● In five series totaling approximately 2000 patients, characteristic radiographic

● Papillary fibroelastomas, the second most common benign cardiac tumors,

● The combination of echocardiography plus CMR or CCT may be useful in

transvenous biopsy of suspected cardiac tumors. Because myxomas may embolize,

Macroscopically, typical myxomas are pedunculated and gelatinous in consistency; the

Clinical manifestations — The cardiovascular manifestations depend upon the

In addition to their cardiovascular effects, patients with myxomas frequently have

● Cardiovascular symptoms were present in 67 percent. Most commonly, these

● Evidence of systemic embolization was present in 29 percent of patients, and 20

Carney complex — The Carney complex is an inherited, autosomal dominant

adrenocortical disease", section on 'Carney complex (CNC)'.)

Treatment and prognosis — Once a presumptive diagnosis of myxoma has been

Postoperative recovery is generally rapid. However, atrial arrhythmias or atrioventricular

Papillary fibroelastomas — Papillary fibroelastomas are the second most common

The clinical spectrum of fibroelastomas can be illustrated by two reports combining

and ventricles. Multiple tumors were present in 9 percent of patients.

Approximately 30 percent of papillary fibroelastomas were asymptomatic and diagnosed

Treatment — A presumptive diagnosis of papillary fibroelastoma can usually be made

Rhabdomyomas — Rhabdomyomas develop almost exclusively in children, mostly before

Fetal and neonatal intrapericardial teratoma — Although these tumors are

Lipomas — Lipomas and fibrolipomas are characterized by a predominance of benign

Symptoms, when present, are generally related to local tissue encroachment

Lipomatous hypertrophy of the interatrial septum — Lipomatous hypertrophy of the

Lipomatous hypertrophy of the interatrial septum is indistinguishable from lipoma except

Other pseudoneoplasms — There are other pseudoneoplasms (like lipomatous

TUMORS THAT MAY BE EITHER BENIGN OR MALIGNANT

Paragangliomas — Paragangliomas are neuroendocrine tumors that can be either

(See "Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology".)

Mesotheliomas arising in the pericardium produce tamponade and constriction [116-119].

pericardial mesotheliomas is very poor [117,118]. The addition of radiation and/or

PRIMARY MALIGNANT TUMORS

Malignant tumors constitute approximately 15 percent of primary cardiac tumors [39].

The most frequently described sarcomas include (see "Clinical presentation,

● Angiosarcomas – Angiosarcomas are composed of malignant cells that form vascular

● Rhabdomyosarcomas – Rhabdomyosarcomas constitute as many as 20 percent of all

● Fibrosarcomas and undifferentiated sarcomas – Fibrosarcomas and

● Leiomyosarcomas – Leiomyosarcomas are spindle-celled, high-grade tumors that

● Other types include liposarcoma and synovial sarcoma [127,138].

Alternative strategies such as cardiac transplantation and cardiac autotransplantation are

Studies illustrating the prognosis of these sarcomas are discussed below:

An alternative treatment, cardiac autotransplantation, has shown promise. In these cases,

SECONDARY CARDIAC TUMORS

In contrast to primary malignant cardiac tumors, metastatic involvement of the heart is

Malignant melanomas are particularly likely to metastasize to the heart [129,168,172,173].

Cardiac or pericardial metastasis should be considered whenever a patient with known

Other causes of cardiac symptoms must also be considered. In particular, metastatic

● Tumors involving the heart may cause symptoms by obstruction of circulation,

● If a cardiac tumor is suspected, imaging procedures are used to determine whether

(See 'Secondary cardiac tumors' above.)

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5. Silvestri F, Bussani R, Pavletic N, Mannone T. Metastases of the heart and