Cardiac Tumors

Cardiac tumors - UpToDate 27/02/19 13.
45
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Cardiac tumors
Authors: William H Gaasch, MD, Thomas J Vander Salm, MD
Section Editor: Wilson S Colucci, MD
Deputy Editors: Sadhna R Vora, 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: Jan 2019. | This topic last updated: Jan 17, 2019.
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:
● Embolization, which is usually systemic but can be pulmonic. Aortic valve and left atrial
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Cardiac tumors - UpToDate 27/02/19 13.45
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
"Classification and prognosis of pulmonary hypertension in adults".)
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].
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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 (MRI), and ultrafast computed

tomography (CT) 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 MRI and CT — Although both cardiac magnetic resonance imaging (MRI) [15-18] and
ultrafast computed tomography (CT) [19,20] provide noninvasive, high-resolution images of the
heart, MRI 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, CT scanning is
still useful when MRI is not immediately available or is contraindicated. An excellent pictorial
review of many cardiac tumors and comparison of MRI and CT scanning has been published
[23]. (See "Clinical utility of cardiovascular magnetic resonance imaging" and "Noninvasive
coronary imaging with cardiac computed tomography and cardiovascular magnetic resonance".)
The utility of information derived from echocardiography, MRI, and/or CT 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 four series totaling 130 patients, characteristic radiographic appearances and tissue
densities accurately facilitated the diagnosis of myxomas, other benign tumors, and
sarcomas [17,24-26].
● 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 [27,28]. (See 'Papillary fibroelastomas' below.)
● Benign lesions such as fibromas and lipomas also have distinctive appearances on
echocardiography, CT, and MRI [29].
● The combination of echocardiography plus cardiac MRI or CT may be useful in

differentiating thrombus from tumor in lesions appearing to arise on a heart valve [30].
PET scan — Positron emission tomography (PET) has been useful in identifying cardiac
involvement in patients with metastatic tumors [31-34], atrial myxoma [35], or lipomatous septal
hypertrophy [36].
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 cardiac MRI or CT 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 [37-42]. 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 [43].
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 [44]. Myxomas produce vascular endothelial growth factor, which probably
contributes to the induction of angiogenesis and the early stages of tumor growth [45,46].
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 [47]. 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 [6,11,48-50]. (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 [51]. 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 [46,52].
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 [47]:
● 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 [53].
● 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 [11,49,50,54,55].
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 [56].
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 [57]. (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 because of the risk of embolization or
cardiovascular complications, including sudden death [49,55,58]. The results of surgical
resection are generally very good, with most series reporting an operative mortality rate under 5
percent [47,49,50,54,55,59,60]. Cardiac autotransplantation (with atrial reconstruction) or
transplantation are potential options for treatment of recurrent atrial myxoma [61,62].
Postoperative recovery is generally rapid. However, atrial arrhythmias or atrioventricular

conduction abnormalities were present postoperatively in 26 percent of patients in one series
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[47]. 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 [47,63]. Recurrence is more common
in patients whose primary tumor was multicentric [53]. Development of a second primary
myxoma may be more common in patients with a family history of myxoma [59].
Papillary fibroelastomas — Papillary fibroelastomas are the second most common primary
cardiac tumor in adults [64]. 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 [28,64]:
● 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 [28,64].
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 [65-69],
others have suggested that careful observation is an acceptable option for asymptomatic
patients as long as the tumor remains small and nonmobile [28].
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 [28,64]. 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 [70-
72]. 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 [70,72-75]. 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 [71,75-78]. 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 [71,77].
Fibromas — Although uncommon, fibromas are nevertheless the second most common
pediatric cardiac tumor and can also occur in adults [48,79-81]. 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 [48].
Heart failure is the most common symptom due to obstruction of blood flow or interference with
valvular function. Myocardial dysfunction and conduction disturbances also occur.
Echocardiography supplemented with computed tomography scans or MRI confirms the
diagnosis. Symptomatic tumors should be resected. 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 [82]. Nearly all cardiac teratomas arise within the pericardium, with the
remainder in the myocardium [83-88].
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 [89]. Thus, there is a risk of death in utero or immediately after birth.
Treatment therefore requires timely detection and resection [89]. 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 [85,87,89,90]. Because
teratomas usually have a single 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 [82,91]. 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 [91].
The primary treatment for benign cardiac teratoma is surgical excision [82,92].
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 [93,94]. As
such, they are undetectable by echocardiographic or radiologic techniques. These are usually
tumors of young children and present with incessant ventricular tachycardia [94].
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 [95,96]. Although most are no more than a few centimeters in size, lipomas as large as
4.8 kg have been reported [97].
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 [98]. 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 [99]. 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
[100,101]. 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 [102].
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 [103].
Other pseudoneoplasms — There are other pseudoneoplasms (like lipomatous hypertrophy)

as well [99]. These include inflammatory myofibroblastic tumor, hamartoma of mature cardiac
myocytes, calcified amorphous tumor [104], 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 [99].
This is also true of the rare intracardiac blood cyst [105].
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) [106]. (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 [107]. Paragangliomas
may be localized with echocardiography. Their extreme vascularity creates a characteristic
magnetic resonance imaging (MRI) appearance [90,108]. 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 [108-110]. 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 [110,111]. If complete resection is not possible,
cardiac transplantation may be required [112]. 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 [113-117]. 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 [113-116]. These
tumors will be seen with echocardiography, computed tomography scan, MRI, and sometimes
by chest radiograph [113-115]. Pericardiocentesis may yield a cytologic diagnosis [114].
More rarely, mesotheliomas may arise as benign tumors of the atrioventricular (AV) node where
they may produce heart block [118,119]. 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 [114,115]. 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 [38]. Sarcomas
are the most common, although other tumor types have been reported.
Sarcomas — Virtually all types of sarcomas have been reported in the heart [6,120-125].
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 [126]. Angiosarcomas arise predominantly in the right atrium
[126,127]. Epithelioid hemangioendothelioma, another sarcoma of vascular origin, has also
been reported [128]. Of sarcomas, 40 percent are angiosarcomas, and 10 percent are
spindle sarcomas [129].
● Rhabdomyosarcomas – Rhabdomyosarcomas constitute as many as 20 percent of all

primary cardiac sarcomas [130]. 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
[131]) are white, fleshy ("fish flesh") tumors that are composed of spindle cells and may
have extensive areas of necrosis and hemorrhage [132,133]. These tumors tend to
extensively infiltrate the myocardium.
● Leiomyosarcomas – Leiomyosarcomas are spindle-celled, high-grade tumors that arise

more frequently in the left atrium [134]. These sarcomas have both a high rate of local
recurrence and systemic spread.
● Other types include liposarcoma and synovial sarcoma [124,135].
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 [54,60,129,136,137]. The median survival is typically 6 to 12 months
[120,124], although long-term survival has been reported with complete resection
[120,123,138,139], and patients with low-grade sarcomas may have a better prognosis [123].
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 [38,138,140-149], 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
[135].
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 [129]. 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 [150].
● In a 34-patient series treated at the Mayo Clinic over a 32-year period [122], 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 [129,150].
The poor results with surgical resection have led to occasional attempts to treat patients with
cardiac transplantation if extracardiac disease is not present [145,151-155]. 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 [151]. 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 [156,157].
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
[158].
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 [159]. However, 38 percent of cases achieved a complete response
with systemic therapy. At least some of these responses may be durable [159-161].
Other tumors may also arise in the heart, including paragangliomas [162,163] and
extramedullary plasmacytomas [164-166].
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 [167].
Malignant melanomas are particularly likely to metastasize to the heart [126,164,168,169].

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 [170]. 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 [171].
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
[126,172,173].
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 ultrafast
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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REFERENCES
1. Reynen K. Frequency of primary tumors of the heart. Am J Cardiol 1996; 77:107.
2. Lam KY, Dickens P, Chan AC. Tumors of the heart. A 20-year experience with a review of
12,485 consecutive autopsies. Arch Pathol Lab Med 1993; 117:1027.
3. Tumors of the cardiovascular system. In: Atlas of Tumor Pathology, Second, Armed Force
s Institute of Pathology, Washington, DC 1978. Vol Fascicle 15.
4. Salcedo EE, Cohen GI, White RD, Davison MB. Cardiac tumors: diagnosis and
management. Curr Probl Cardiol 1992; 17:73.
5. Silvestri F, Bussani R, Pavletic N, Mannone T. Metastases of the heart and pericardium. G

Ital Cardiol 1997; 27:1252.
6. Vander Salm TJ. Unusual primary tumors of the heart. Semin Thorac Cardiovasc Surg
2000; 12:89.
7. Elbardissi AW, Dearani JA, Daly RC, et al. Embolic potential of cardiac tumors and
outcome after resection: a case-control study. Stroke 2009; 40:156.
8. Sheu CC, Lin SF, Chiu CC, et al. Left atrial sarcoma mimicking obstructive pulmonary
disease. J Clin Oncol 2007; 25:1277.
9. Lee VH, Connolly HM, Brown RD Jr. Central nervous system manifestations of cardiac
myxoma. Arch Neurol 2007; 64:1115.
10. Kumar S, Chaudhry MA, Khan I, et al. Metastatic left atrial synovial sarcoma mimicking a
myxoma. J Thorac Cardiovasc Surg 2004; 128:756.
11. Kuon E, Kreplin M, Weiss W, Dahm JB. The challenge presented by right atrial myxoma.
Herz 2004; 29:702.
12. Savino JS, Weiss SJ. Images in clinical medicine. Right atrial tumor. N Engl J Med 1995;
333:1608.
13. Diaz Castro O, Bueno H, Nebreda LA. Acute myocardial infarction caused by paradoxical
tumorous embolism as a manifestation of hepatocarcinoma. Heart 2004; 90:e29.
14. Engberding R, Daniel WG, Erbel R, et al. Diagnosis of heart tumours by transoesophageal
echocardiography: a multicentre study in 154 patients. European Cooperative Study
Group. Eur Heart J 1993; 14:1223.
15. Constantine G, Shan K, Flamm SD, Sivananthan MU. Role of MRI in clinical cardiology.
Lancet 2004; 363:2162.
16. Gilkeson RC, Chiles C. MR evaluation of cardiac and pericardial malignancy. Magn Reson
Imaging Clin N Am 2003; 11:173.
17. Kaminaga T, Takeshita T, Kimura I. Role of magnetic resonance imaging for evaluation of
tumors in the cardiac region. Eur Radiol 2003; 13 Suppl 4:L1.
18. Gulati G, Sharma S, Kothari SS, et al. Comparison of echo and MRI in the imaging
evaluation of intracardiac masses. Cardiovasc Intervent Radiol 2004; 27:459.
19. Bleiweis MS, Georgiou D, Brundage BH. Detection of intracardiac masses by ultrafast
computed tomography. Am J Card Imaging 1994; 8:63.
20. de Lucas EM, Pagola MA, Fernández F, et al. Primary cardiac lymphoma: helical CT
findings and radiopathologic correlation. Cardiovasc Intervent Radiol 2004; 27:190.
21. Mousavi N, Cheezum MK, Aghayev A, et al. Assessment of Cardiac Masses by Cardiac
Magnetic Resonance Imaging: Histological Correlation and Clinical Outcomes. J Am Heart
Assoc 2019; 8:e007829.
22. Araoz PA, Eklund HE, Welch TJ, Breen JF. CT and MR imaging of primary cardiac
malignancies. Radiographics 1999; 19:1421.
23. Hoey ET, Mankad K, Puppala S, et al. MRI and CT appearances of cardiac tumours in
adults. Clin Radiol 2009; 64:1214.
24. Sommer T, Vahlhaus C, Hofer U, et al. [MRI diagnosis of cardiac myxomas: sequence
evaluation and differential diagnosis]. Rofo 1999; 170:156.
25. Siripornpitak S, Higgins CB. MRI of primary malignant cardiovascular tumors. J Comput
Assist Tomogr 1997; 21:462.
26. Grebenc ML, Rosado-de-Christenson ML, Green CE, et al. Cardiac myxoma: imaging
features in 83 patients. Radiographics 2002; 22:673.
27. Klarich KW, Enriquez-Sarano M, Gura GM, et al. Papillary fibroelastoma:

echocardiographic characteristics for diagnosis and pathologic correlation. J Am Coll
Cardiol 1997; 30:784.
28. Sun JP, Asher CR, Yang XS, et al. Clinical and echocardiographic characteristics of
papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation
2001; 103:2687.
29. Araoz PA, Mulvagh SL, Tazelaar HD, et al. CT and MR imaging of benign primary cardiac
neoplasms with echocardiographic correlation. Radiographics 2000; 20:1303.
30. Wintersperger BJ, Becker CR, Gulbins H, et al. Tumors of the cardiac valves: imaging
findings in magnetic resonance imaging, electron beam computed tomography, and
echocardiography. Eur Radiol 2000; 10:443.
31. García JR, Simo M, Huguet M, et al. Usefulness of 18-fluorodeoxyglucose positron

emission tomography in the evaluation of tumor cardiac thrombus from renal cell
carcinoma. Clin Transl Oncol 2006; 8:124.
32. Gates GF, Aronsky A, Ozgur H. Intracardiac extension of lung cancer demonstrated on
PET scanning. Clin Nucl Med 2006; 31:68.
33. Kim JH, Jung JY, Park Yl, et al. Non-small cell lung cancer initially presenting with
intracardiac metastasis. Korean J Intern Med 2005; 20:86.
34. Buchmann I, Wandt H, Wahl A, Reske SN. FDG PET for imaging pericardial
manifestations of Hodgkin lymphoma. Clin Nucl Med 2003; 28:760.
35. Agostini D, Babatasi G, Galateau F, et al. Detection of cardiac myxoma by F-18 FDG PET.
Clin Nucl Med 1999; 24:159.
36. Fan CM, Fischman AJ, Kwek BH, et al. Lipomatous hypertrophy of the interatrial septum:
increased uptake on FDG PET. AJR Am J Roentgenol 2005; 184:339.
37. Shi L, Wu L, Fang H, et al. Identification and clinical course of 166 pediatric cardiac
tumors. Eur J Pediatr 2017; 176:253.
38. Molina JE, Edwards JE, Ward HB. Primary cardiac tumors: experience at the University of
Minnesota. Thorac Cardiovasc Surg 1990; 38 Suppl 2:183.
39. Tazelaar HD, Locke TJ, McGregor CG. Pathology of surgically excised primary cardiac
tumors. Mayo Clin Proc 1992; 67:957.
https://www.uptodate.com/contents/cardiac-tumors/print?search=fe…rch_result&selectedTitle=1~150&usage_type=default&display_rank=1 Page 20 of 37
40. Larrieu AJ, Jamieson WR, Tyers GF, et al. Primary cardiac tumors: experience with 25
cases. J Thorac Cardiovasc Surg 1982; 83:339.
41. Odim J, Reehal V, Laks H, et al. Surgical pathology of cardiac tumors. Two decades at an
urban institution. Cardiovasc Pathol 2003; 12:267.
42. Kamiya H, Yasuda T, Nagamine H, et al. Surgical treatment of primary cardiac tumors: 28
years' experience in Kanazawa University Hospital. Jpn Circ J 2001; 65:315.
43. Ying L, Lin R, Gao Z, et al. Primary cardiac tumors in children: a center's experience. J
Cardiothorac Surg 2016; 11:52.
44. Pucci A, Gagliardotto P, Zanini C, et al. Histopathologic and clinical characterization of

cardiac myxoma: review of 53 cases from a single institution. Am Heart J 2000; 140:134.
45. Kono T, Koide N, Hama Y, et al. Expression of vascular endothelial growth factor and
angiogenesis in cardiac myxoma: a study of fifteen patients. J Thorac Cardiovasc Surg
2000; 119:101.
46. Sakamoto H, Sakamaki T, Kanda T, et al. Vascular endothelial growth factor is an

autocrine growth factor for cardiac myxoma cells. Circ J 2004; 68:488.
47. Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of
112 consecutive cases. Medicine (Baltimore) 2001; 80:159.
48. ElBardissi AW, Dearani JA, Daly RC, et al. Analysis of benign ventricular tumors: long-
term outcome after resection. J Thorac Cardiovasc Surg 2008; 135:1061.
49. Keeling IM, Oberwalder P, Anelli-Monti M, et al. Cardiac myxomas: 24 years of experience
in 49 patients. Eur J Cardiothorac Surg 2002; 22:971.
50. Jelic J, Milicić D, Alfirević I, et al. Cardiac myxoma: diagnostic approach, surgical
treatment and follow-up. A twenty years experience. J Cardiovasc Surg (Torino) 1996;
37:113.
51. Maisch B. Immunology of cardiac tumors. Thorac Cardiovasc Surg 1990; 38 Suppl 2:157.
52. Seino Y, Ikeda U, Shimada K. Increased expression of interleukin 6 mRNA in cardiac

myxomas. Br Heart J 1993; 69:565.
53. Wang Z, Chen S, Zhu M, et al. Risk prediction for emboli and recurrence of primary
cardiac myxomas after resection. J Cardiothorac Surg 2016; 11:22.
54. Centofanti P, Di Rosa E, Deorsola L, et al. Primary cardiac tumors: early and late results
of surgical treatment in 91 patients. Ann Thorac Surg 1999; 68:1236.
55. Selkane C, Amahzoune B, Chavanis N, et al. Changing management of cardiac myxoma

based on a series of 40 cases with long-term follow-up. Ann Thorac Surg 2003; 76:1935.
56. Vidaillet HJ Jr, Seward JB, Fyke FE 3rd, et al. "Syndrome myxoma": a subset of patients
with cardiac myxoma associated with pigmented skin lesions and peripheral and
endocrine neoplasms. Br Heart J 1987; 57:247.
57. Alrashdi I, Bano G, Maher ER, Hodgson SV. Carney triad versus Carney Stratakis
syndrome: two cases which illustrate the difficulty in distinguishing between these
conditions in individual patients. Fam Cancer 2010; 9:443.
58. Cina SJ, Smialek JE, Burke AP, et al. Primary cardiac tumors causing sudden death: a
review of the literature. Am J Forensic Med Pathol 1996; 17:271.
59. Bhan A, Mehrotra R, Choudhary SK, et al. Surgical experience with intracardiac
myxomas: long-term follow-up. Ann Thorac Surg 1998; 66:810.
60. Bakaeen FG, Reardon MJ, Coselli JS, et al. Surgical outcome in 85 patients with primary
cardiac tumors. Am J Surg 2003; 186:641.
61. Gammie JS, Abrishamchian AR, Griffith BP. Cardiac autotransplantation and radical bi-
atrial resection for recurrent atrial myxoma. Ann Thorac Surg 2007; 83:1545.
62. Goldstein DJ, Oz MC, Michler RE. Radical excisional therapy and total cardiac
transplantation for recurrent atrial myxoma. Ann Thorac Surg 1995; 60:1105.
63. D'Alfonso A, Catania S, Pierri MD, et al. Atrial myxoma: a 25-year single-institutional
follow-up study. J Cardiovasc Med (Hagerstown) 2008; 9:178.
64. Gowda RM, Khan IA, Nair CK, et al. Cardiac papillary fibroelastoma: a comprehensive
analysis of 725 cases. Am Heart J 2003; 146:404.
65. Giannesini C, Kubis N, N'Guyen A, et al. Cardiac papillary fibroelastoma: aA rare cause of
ischemic stroke in the young. Cerebrovasc Dis 1999; 9:45.
66. Colucci V, Alberti A, Bonacina E, Gordini V. Papillary fibroelastoma of the mitral valve. A
rare cause of embolic events. Tex Heart Inst J 1995; 22:327.
67. Grinda JM, Couetil JP, Chauvaud S, et al. Cardiac valve papillary fibroelastoma: surgical
excision for revealed or potential embolization. J Thorac Cardiovasc Surg 1999; 117:106.
68. Ni Y, von Segesser LK, Dirsch O, et al. Cardiac papillary fibroelastoma. Thorac
Cardiovasc Surg 1996; 44:257.
69. Shahian DM, Labib SB, Chang G. Cardiac papillary fibroelastoma. Ann Thorac Surg 1995;
59:538.
70. Beghetti M, Gow RM, Haney I, et al. Pediatric primary benign cardiac tumors: a 15-year
review. Am Heart J 1997; 134:1107.
71. Bosi G, Lintermans JP, Pellegrino PA, et al. The natural history of cardiac rhabdomyoma
with and without tuberous sclerosis. Acta Paediatr 1996; 85:928.
72. Kocabaş A, Ekici F, Cetin Iİ, et al. Cardiac rhabdomyomas associated with tuberous
sclerosis complex in 11 children: presentation to outcome. Pediatr Hematol Oncol 2013;
30:71.
73. Stiller B, Hetzer R, Meyer R, et al. Primary cardiac tumours: when is surgery necessary?
Eur J Cardiothorac Surg 2001; 20:1002.
74. Isaacs H Jr. Fetal and neonatal cardiac tumors. Pediatr Cardiol 2004; 25:252.
75. Smythe JF, Dyck JD, Smallhorn JF, Freedom RM. Natural history of cardiac
rhabdomyoma in infancy and childhood. Am J Cardiol 1990; 66:1247.
76. Becker AE. Primary heart tumors in the pediatric age group: a review of salient pathologic
features relevant for clinicians. Pediatr Cardiol 2000; 21:317.
77. Jacobs JP, Konstantakos AK, Holland FW 2nd, et al. Surgical treatment for cardiac
rhabdomyomas in children. Ann Thorac Surg 1994; 58:1552.
78. Jóźwiak S, Kawalec W, Dłuzewska J, et al. Cardiac tumours in tuberous sclerosis: their
incidence and course. Eur J Pediatr 1994; 153:155.
79. Valente M, Cocco P, Thiene G, et al. Cardiac fibroma and heart transplantation. J Thorac
80. Bapat VN, Varma GG, Hordikar AA, et al. Right-ventricular fibroma presenting as tricuspid
stenosis--a case report. Thorac Cardiovasc Surg 1996; 44:152.
81. Burke AP, Rosado-de-Christenson M, Templeton PA, Virmani R. Cardiac fibroma:

clinicopathologic correlates and surgical treatment. J Thorac Cardiovasc Surg 1994;
108:862.
82. Cohen R, Mirrer B, Loarte P, Navarro V. Intrapericardial mature cystic teratoma in an

adult: case presentation. Clin Cardiol 2013; 36:6.
83. Brabham KR, Roberts WC. Cardiac-compressing intrapericardial teratoma at birth. Am J

Cardiol 1989; 63:386.
84. Campagne G, Quereda F, Merino G, et al. Benign intracardiac teratoma detected

prenatally. Case report and review of the literature. Eur J Obstet Gynecol Reprod Biol
1998; 80:105.
85. Paw PT, Jamieson SW. Surgical management of intrapericardial teratoma diagnosed in
utero. Ann Thorac Surg 1997; 64:552.
86. Sklansky M, Greenberg M, Lucas V, Gruslin-Giroux A. Intrapericardial teratoma in a twin

fetus: diagnosis and management. Obstet Gynecol 1997; 89:807.
87. Tollens T, Casselman F, Devlieger H, et al. Fetal cardiac tamponade due to an

intrapericardial teratoma. Ann Thorac Surg 1998; 66:559.
88. Agozzino L, Vosa C, Arciprete P, et al. Intrapericardial teratoma in the newborn. Int J
Cardiol 1984; 5:21.
89. Rychik J, Khalek N, Gaynor JW, et al. Fetal intrapericardial teratoma: natural history and
management including successful in utero surgery. Am J Obstet Gynecol 2016;
215:780.e1.
90. Riskin-Mashiah S, Moise KJ Jr, Wilkins I, et al. In utero diagnosis of intrapericardial

teratoma: a case for in utero open fetal surgery. Prenat Diagn 1998; 18:1328.
91. Pathology and genetics of tumours of the lung, pleura, thymus, and heart. In: World Healt
h Organization classification of tumours, Travis WD, Brambilla E, Muller-Hermelink HK, Ha
rris CC (Eds), IARC Press, Lyon 2004. p.287.
92. Mori M, Hosoba S, Iturra S, et al. Large primary right ventricular teratoma in an adult. Ann
Thorac Surg 2015; 99:1799.
93. Cooley DA. Surgical treatment of cardiac neoplasms: 32-year experience. Thorac
Cardiovasc Surg 1990; 38 Suppl 2:176.
94. Garson A Jr, Smith RT Jr, Moak JP, et al. Incessant ventricular tachycardia in infants:
myocardial hamartomas and surgical cure. J Am Coll Cardiol 1987; 10:619.
95. Benvenuti LA, Mansur AJ, Lopes DO, Campos RV. Primary lipomatous tumors of the
cardiac valves. South Med J 1996; 89:1018.
96. Hananouchi GI, Goff WB 2nd. Cardiac lipoma: six-year follow-up with MRI characteristics,
and a review of the literature. Magn Reson Imaging 1990; 8:825.
97. Lang-Lazdunski L, Oroudji M, Pansard Y, et al. Successful resection of giant

intrapericardial lipoma. Ann Thorac Surg 1994; 58:238.
98. Caralps JM, Martí V, Ferrés P, et al. Mitral valve repair after excision of a fibrolipoma. Ann
Thorac Surg 1998; 66:1808.
99. Miller DV, Tazelaar HD. Cardiovascular pseudoneoplasms. Arch Pathol Lab Med 2010;
134:362.
100. Basu S, Folliguet T, Anselmo M, et al. Lipomatous hypertrophy of the interatrial septum.
101. Zeebregts CJ, Hensens AG, Timmermans J, et al. Lipomatous hypertrophy of the
interatrial septum: indication for surgery? Eur J Cardiothorac Surg 1997; 11:785.
102. Chaowalit N, Somers VK, Pellikka PA, et al. Adipose tissue of atrial septum as a marker of
coronary artery disease. Chest 2007; 132:817.
103. Calé R, Andrade MJ, Canada M, et al. Lipomatous hypertrophy of the interatrial septum:
report of two cases where histological examination and surgical intervention were
unavoidable. Eur J Echocardiogr 2009; 10:876.
104. Rehman A, Heng EE, Cheema FH. Calcified amorphous tumour of right ventricle. Lancet
2014; 383:815.
105. Dumantepe M, Ak K, Mungan U, et al. Blood cyst of the right ventricle presenting as
recurrent high fever and chills in an adult. Ann Thorac Surg 2009; 87:638.
106. Biology and treatment of thoracic tumors of neural crest origin. In: Thoracic Oncology, WB
Saunders, Philadelphia 1989. p.520.
107. Tanaka F, Kitano M, Tatsumi A, et al. Paraganglioma of the posterior mediastinum: value
of magnetic resonance imaging. Ann Thorac Surg 1992; 53:517.
108. Flickinger FW, Yuh WT, Behrendt DM. Magnetic resonance imaging of mediastinal
paraganglioma. Chest 1988; 94:652.
109. Jebara VA, Uva MS, Farge A, et al. Cardiac pheochromocytomas. Ann Thorac Surg 1992;
53:356.
110. Vander Salm, TJ. Mediastinal pheochromocytoma. Worcester, University of Massachusett

s Medical Center. Society of Thoracic Surgeons, Surgical Motion Pictures 1994.
111. Chang CH, Lin PJ, Chang JP, et al. Intrapericardial pheochromocytoma. Ann Thorac Surg
1991; 51:661.
112. Jeevanandam V, Oz MC, Shapiro B, et al. Surgical management of cardiac

pheochromocytoma. Resection versus transplantation. Ann Surg 1995; 221:415.
113. Naramoto A, Itoh N, Nakano M, Shigematsu H. An autopsy case of tuberous sclerosis

associated with primary pericardial mesothelioma. Acta Pathol Jpn 1989; 39:400.
114. Kaul TK, Fields BL, Kahn DR. Primary malignant pericardial mesothelioma: a case report
and review. J Cardiovasc Surg (Torino) 1994; 35:261.
115. Thomason R, Schlegel W, Lucca M, et al. Primary malignant mesothelioma of the

pericardium. Case report and literature review. Tex Heart Inst J 1994; 21:170.
116. Ohmori T, Arita N, Okada K, et al. Pericardial malignant mesothelioma: case report and
discussion of immunohistochemical and histochemical findings. Pathol Int 1995; 45:622.
117. Vigneswaran WT, Stefanacci PR. Pericardial mesothelioma. Curr Treat Options Oncol
2000; 1:299.
118. Balasundaram S, Halees SA, Duran C. Mesothelioma of the atrioventricular node: first
successful follow-up after excision. Eur Heart J 1992; 13:718.
119. Kawano H, Okada R, Kawano Y, et al. Mesothelioma in the atrioventricular node. Case
report. Jpn Heart J 1994; 35:255.
120. Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992; 69:387.
121. Donsbeck AV, Ranchere D, Coindre JM, et al. Primary cardiac sarcomas: an
immunohistochemical and grading study with long-term follow-up of 24 cases.
Histopathology 1999; 34:295.
122. Simpson L, Kumar SK, Okuno SH, et al. Malignant primary cardiac tumors: review of a
single institution experience. Cancer 2008; 112:2440.
123. Zhang PJ, Brooks JS, Goldblum JR, et al. Primary cardiac sarcomas: a clinicopathologic
analysis of a series with follow-up information in 17 patients and emphasis on long-term
survival. Hum Pathol 2008; 39:1385.
124. Truong PT, Jones SO, Martens B, et al. Treatment and outcomes in adult patients with
primary cardiac sarcoma: the British Columbia Cancer Agency experience. Ann Surg
Oncol 2009; 16:3358.
125. Orlandi A, Ferlosio A, Roselli M, et al. Cardiac sarcomas: an update. J Thorac Oncol
2010; 5:1483.
126. Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas. A review and a case report.
Cancer 1986; 57:852.
127. Herrmann MA, Shankerman RA, Edwards WD, et al. Primary cardiac angiosarcoma: a
clinicopathologic study of six cases. J Thorac Cardiovasc Surg 1992; 103:655.
128. Lisy M, Beierlein W, Müller H, et al. Left atrial epithelioid hemangioendothelioma. J Thorac
129. Ramlawi B, Leja MJ, Abu Saleh WK, et al. Surgical Treatment of Primary Cardiac
Sarcomas: Review of a Single-Institution Experience. Ann Thorac Surg 2016; 101:698.
130. Castorino F, Masiello P, Quattrocchi E, Di Benedetto G. Primary cardiac

rhabdomyosarcoma of the left atrium: an unusual presentation. Tex Heart Inst J 2000;
27:206.
131. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. World Health Organization Classi
fication of tumours of soft tissue and bone, 4th ed, IARC Press, Lyon 2013.
132. Schena S, Caniglia A, Agnino A, et al. Survival following treatment of a cardiac malignant
fibrous histiocytoma. Chest 2000; 118:271.
133. Okamoto K, Kato S, Katsuki S, et al. Malignant fibrous histiocytoma of the heart: case
report and review of 46 cases in the literature. Intern Med 2001; 40:1222.
134. Pins MR, Ferrell MA, Madsen JC, et al. Epithelioid and spindle-celled leiomyosarcoma of
the heart. Report of 2 cases and review of the literature. Arch Pathol Lab Med 1999;
123:782.
135. Bakaeen FG, Jaroszewski DE, Rice DC, et al. Outcomes after surgical resection of
cardiac sarcoma in the multimodality treatment era. J Thorac Cardiovasc Surg 2009;
137:1454.
136. Kosuga T, Fukunaga S, Kawara T, et al. Surgery for primary cardiac tumors. Clinical
experience and surgical results in 60 patients. J Cardiovasc Surg (Torino) 2002; 43:581.
137. Raaf HN, Raaf JH. Sarcomas related to the heart and vasculature. Semin Surg Oncol
1994; 10:374.
138. Putnam JB Jr, Sweeney MS, Colon R, et al. Primary cardiac sarcomas. Ann Thorac Surg
1991; 51:906.
139. Shapira OM, Korach A, Izhar U, et al. Radical multidisciplinary approach to primary
cardiac sarcomas. Eur J Cardiothorac Surg 2013; 44:330.
140. Llombart-Cussac A, Pivot X, Contesso G, et al. Adjuvant chemotherapy for primary

cardiac sarcomas: the IGR experience. Br J Cancer 1998; 78:1624.
141. Antunes MJ, Vanderdonck KM, Andrade CM, Rebelo LS. Primary cardiac
leiomyosarcomas. Ann Thorac Surg 1991; 51:999.
142. Kakizaki S, Takagi H, Hosaka Y. Cardiac angiosarcoma responding to multidisciplinary

treatment. Int J Cardiol 1997; 62:273.
143. Pessotto R, Silvestre G, Luciani GB, et al. Primary cardiac leiomyosarcoma: seven-year
survival with combined surgical and adjuvant therapy. Int J Cardiol 1997; 60:91.
144. Landolsi-Ben Ammou A, Ben Fatma L, Kallel L, et al. [Primary cardiac sarcoma: report of
3 cases and review of the literature]. Ann Cardiol Angeiol (Paris) 2003; 52:370.
145. Fahn W, Schlemmer M, Issels R, et al. [Leiomyosarcoma of the heart--interdisciplinary

therapeutic approach of systemic chemotherapy and subsequent heart transplantation].
Dtsch Med Wochenschr 2003; 128:2005.
146. Mery GM, Reardon MJ, Haas J, et al. A combined modality approach to recurrent cardiac
sarcoma resulting in a prolonged remission: a case report. Chest 2003; 123:1766.
147. Nakamichi T, Fukuda T, Suzuki T, et al. Primary cardiac angiosarcoma: 53 months'

survival after multidisciplinary therapy. Ann Thorac Surg 1997; 63:1160.
148. Mayer F, Aebert H, Rudert M, et al. Primary malignant sarcomas of the heart and great
vessels in adult patients--a single-center experience. Oncologist 2007; 12:1134.
149. Abu Saleh WK, Ramlawi B, Shapira OM, et al. Improved Outcomes With the Evolution of
a Neoadjuvant Chemotherapy Approach to Right Heart Sarcoma. Ann Thorac Surg 2017;
104:90.
150. Oliveira GH, Al-Kindi SG, Hoimes C, Park SJ. Characteristics and Survival of Malignant
Cardiac Tumors: A 40-Year Analysis of >500 Patients. Circulation 2015; 132:2395.
151. Gowdamarajan A, Michler RE. Therapy for primary cardiac tumors: is there a role for heart
transplantation? Curr Opin Cardiol 2000; 15:121.
152. Uberfuhr P, Meiser B, Fuchs A, et al. Heart transplantation: an approach to treating

primary cardiac sarcoma? J Heart Lung Transplant 2002; 21:1135.
153. Talbot SM, Taub RN, Keohan ML, et al. Combined heart and lung transplantation for
unresectable primary cardiac sarcoma. J Thorac Cardiovasc Surg 2002; 124:1145.
154. Baay P, Karwande SV, Kushner JP, et al. Successful treatment of a cardiac angiosarcoma
with combined modality therapy. J Heart Lung Transplant 1994; 13:923.
155. Grandmougin D, Fayad G, Decoene C, et al. Total orthotopic heart transplantation for
primary cardiac rhabdomyosarcoma: factors influencing long-term survival. Ann Thorac
Surg 2001; 71:1438.
156. Reardon MJ, Malaisrie SC, Walkes JC, et al. Cardiac autotransplantation for primary
cardiac tumors. Ann Thorac Surg 2006; 82:645.
157. Blackmon SH, Reardon MJ. Surgical treatment of primary cardiac sarcomas. Tex Heart
Inst J 2009; 36:451.
158. Son KH, Kim KW, Ahn CB, et al. Surgical Planning by 3D Printing for Primary Cardiac
Schwannoma Resection. Yonsei Med J 2015; 56:1735.
159. Ikeda H, Nakamura S, Nishimaki H, et al. Primary lymphoma of the heart: case report and
literature review. Pathol Int 2004; 54:187.
160. Anghel G, Zoli V, Petti N, et al. Primary cardiac lymphoma: report of two cases occurring
in immunocompetent subjects. Leuk Lymphoma 2004; 45:781.
161. Nakagawa Y, Ikeda U, Hirose M, et al. Successful treatment of primary cardiac lymphoma
with monoclonal CD20 antibody (rituximab). Circ J 2004; 68:172.
162. Moorjani N, Kuo J, Wilkins D. Left atrial phaeochromocytoma. Heart 2004; 90:e64.
163. Lupinski RW, Shankar S, Agasthian T, et al. Primary cardiac paraganglioma. Ann Thorac
Surg 2004; 78:e43.
164. Keung YK, Lau S, Gill P. Extramedullary plasmacytoma of the heart presenting as cardiac
emergency. Review of literature. Am J Clin Oncol 1994; 17:427.
165. Khankirawatana B, Ginete WL. Primary extramedullary plasmacytoma of the heart. Clin
Cardiol 2004; 27:368.
166. Fernandez LA, Couban S, Sy R, Miller R. An unusual presentation of extramedullary

plasmacytoma occurring sequentially in the testis, subcutaneous tissue, and heart. Am J
Hematol 2001; 67:194.
167. Longo R, Mocini D, Santini M, et al. Unusual sites of metastatic malignancy: case 1.
Cardiac metastasis in hepatocellular carcinoma. J Clin Oncol 2004; 22:5012.
168. Savoia P, Fierro MT, Zaccagna A, Bernengo MG. Metastatic melanoma of the heart. J
Surg Oncol 2000; 75:203.
169. Reynen K, Köckeritz U, Strasser RH. Metastases to the heart. Ann Oncol 2004; 15:375.
170. Goldberg AD, Blankstein R, Padera RF. Tumors metastatic to the heart. Circulation 2013;
128:1790.
171. Sosinska-Mielcarek K, Senkus-Konefka E, Jassem J, et al. Cardiac involvement at

presentation of non-small-cell lung cancer. J Clin Oncol 2008; 26:1010.
172. Messner G, Harting MT, Russo P, et al. Surgical management of metastatic melanoma to
the ventricle. Tex Heart Inst J 2003; 30:218.
173. Labib SB, Schick EC Jr, Isner JM. Obstruction of right ventricular outflow tract caused by
intracavitary metastatic disease: analysis of 14 cases. J Am Coll Cardiol 1992; 19:1664.
Topic 4881 Version 26.0
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 Consultant/Advisory Boards: Novartis [Heart failure (Valsartan, sacubitril/valsartan)], Merck
[Heart failure (Enalapril)]. Sadhna R Vora, 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

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Cardiac tumors - UpToDate 27/02/19 13.

Official reprint from UpToDate®

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

tumors were associated with greatest risk of embolization [7].

● Interference with the heart valves, causing regurgitation.

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

● Constitutional or systemic symptoms.

Echocardiography, cardiac magnetic resonance imaging (MRI), and ultrafast computed

Echocardiography — Echocardiography is widely available and provides a simple,

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

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

● The combination of echocardiography plus cardiac MRI or CT may be useful in

Clinical manifestations — The cardiovascular manifestations depend upon the anatomic

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

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

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

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

Approximately 30 percent of papillary fibroelastomas were asymptomatic and diagnosed

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

resection has not been reported.

Rhabdomyomas — Rhabdomyomas develop almost exclusively in children, mostly before the

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

Lipomatous hypertrophy of the interatrial septum — Lipomatous hypertrophy of the

Other pseudoneoplasms — There are other pseudoneoplasms (like lipomatous hypertrophy)

TUMORS THAT MAY BE EITHER BENIGN OR MALIGNANT

Paragangliomas — Paragangliomas are neuroendocrine tumors that can be either benign or

PRIMARY MALIGNANT TUMORS

● 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 arise

● Other types include liposarcoma and synovial sarcoma [124,135].

Studies illustrating the prognosis of these sarcomas are discussed below:

SECONDARY CARDIAC TUMORS

Malignant melanomas are particularly likely to metastasize to the heart [126,164,168,169].

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

'Clinical manifestations' above.)

Use of UpToDate is subject to the Subscription and License Agreement.

1. Reynen K. Frequency of primary tumors of the heart. Am J Cardiol 1996; 77:107.

12,485 consecutive autopsies. Arch Pathol Lab Med 1993; 117:1027.

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

Lancet 2004; 363:2162.

27. Klarich KW, Enriquez-Sarano M, Gura GM, et al. Papillary fibroelastoma:

31. García JR, Simo M, Huguet M, et al. Usefulness of 18-fluorodeoxyglucose positron

44. Pucci A, Gagliardotto P, Zanini C, et al. Histopathologic and clinical characterization of

46. Sakamoto H, Sakamaki T, Kanda T, et al. Vascular endothelial growth factor is an

52. Seino Y, Ikeda U, Shimada K. Increased expression of interleukin 6 mRNA in cardiac

55. Selkane C, Amahzoune B, Chavanis N, et al. Changing management of cardiac myxoma

ischemic stroke in the young. Cerebrovasc Dis 1999; 9:45.

81. Burke AP, Rosado-de-Christenson M, Templeton PA, Virmani R. Cardiac fibroma:

82. Cohen R, Mirrer B, Loarte P, Navarro V. Intrapericardial mature cystic teratoma in an

83. Brabham KR, Roberts WC. Cardiac-compressing intrapericardial teratoma at birth. Am J

84. Campagne G, Quereda F, Merino G, et al. Benign intracardiac teratoma detected

86. Sklansky M, Greenberg M, Lucas V, Gruslin-Giroux A. Intrapericardial teratoma in a twin

87. Tollens T, Casselman F, Devlieger H, et al. Fetal cardiac tamponade due to an

90. Riskin-Mashiah S, Moise KJ Jr, Wilkins I, et al. In utero diagnosis of intrapericardial

97. Lang-Lazdunski L, Oroudji M, Pansard Y, et al. Successful resection of giant

unavoidable. Eur J Echocardiogr 2009; 10:876.

110. Vander Salm, TJ. Mediastinal pheochromocytoma. Worcester, University of Massachusett

112. Jeevanandam V, Oz MC, Shapiro B, et al. Surgical management of cardiac

113. Naramoto A, Itoh N, Nakano M, Shigematsu H. An autopsy case of tuberous sclerosis

115. Thomason R, Schlegel W, Lucca M, et al. Primary malignant mesothelioma of the

Sarcomas: Review of a Single-Institution Experience. Ann Thorac Surg 2016; 101:698.

130. Castorino F, Masiello P, Quattrocchi E, Di Benedetto G. Primary cardiac