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State of the Art

Surgery for Tumors of the Heart

D1X XBobby Yanagawa, D2X XMD, PhD,* D3X XAmine Mazine, D4X XMD, MSc,† D5X XEdward Y. Chan, D6X XMD,‡
D7X XColin M. Barker, D8X XMD,§ D9X XMichael Gritti, D10X XMD,† D1X XRoss M. Reul, D12X XMD,║ D13X XVinod Ravi, D14X XMD,¶
D15X XSergio Ibarra, D16X XMD,║ D17X XOz M. Shapira, D18X XMD,# D19X XRobert J. Cusimano, D20X XMD,† and D21X XMichael J. Reardon, D2X XMD║

Most surgeons will encounter only a handful of primary cardiac tumors out-
side of myxomas. Approximately 3 quarters of primary cardiac tumors are
benign and 1 quarter is malignant. In most cases, cardiac tumors are silent
but when symptoms do occur, they are primarily determined by tumor size
and anatomical location, not by histopathology. The diagnosis and preoper-
ative imaging relies heavily on multimodal imaging including echocardiogra-
phy, computed tomography, magnetic resonance imaging, and coronary
angiography. Surgical resection is the most common treatment for most
simple primary cardiac tumors and for some complex benign tumors. Surgi-
cal resection of primary cardiac tumors frequently involves the need for
complex cardiac reconstruction, particularly when malignant. Secondary
tumors to the heart are 30 times more frequent than primary cardiac tumors,
and their incidence is increasing, largely as a result of advances in cancer
diagnosis and therapy. Surgical resection is feasible in only a small fraction
of highly-selected patients with secondary tumors to the heart. For complex
benign tumors—such as paraganglioma or large fibromas—and all primary
and secondary malignant tumors, a multidisciplinary cardiac tumor team
review in experienced centers of excellence is recommended.
Intraoperative image of a large left ventricular
Semin Thoracic Surg 30:385
397 © 2018 Elsevier Inc. All rights reserved. melanoma.

Keywords: cardiac tumor, malignant tumor, sarcoma, tumor team Central Message

Cardiac tumors can be conceptualized as pri-

mary simple, primary complex, and secondary.
Surgical resection is the most common treat-
INTRODUCTION
ment for most simple primary cardiac tumors
Primary cardiac tumors were first recognized in an autopsy and some complex benign tumors. Secondary
report by Dr. Realdo Colombus in 1559.1 In 1820, Dr. William tumors to the heart are more frequent, but their
Norris first reported on a “melanotic heart” with secondary prognosis remains dismal. Patients with com-
plex benign, malignant, and secondary tumors
*Division of Cardiac Surgery, Department of Surgery, St Michael's Hos- should be managed by a multidisciplinary car-
pital, University of Toronto, Toronto, Ontario, Canada diac tumor team in a center of excellence.
y
Division of Cardiac Surgery, Department of Surgery, Peter Munk Car-
diac Centre, Toronto General Hospital and University of Toronto, Tor-
onto, Ontario, Canada
z
metastasis to the heart.2 In 1953, Bhanson et al attempted sur-
Department of Surgery, Houston Methodist Hospital, Houston, Texas gical resection of a large right atrial myxoma via anterior thora-
x
Department of Cardiology, Houston Methodist DeBakey Heart & Vas-
cular Center, Houston Methodist Hospital, Houston, Texas
cotomy using a short period of caval inflow occlusion, but the
║
Department of Cardiovascular Surgery, Houston Methodist DeBakey patient died 24 days later.3 The emergence of echocardiogra-
Heart & Vascular Center, Houston Methodist Hospital, Houston, Texas phy has greatly increased the number of primary cardiac
{
Department of Oncology, MD Anderson Cancer Center, Houston, tumors diagnosed. The introduction of cardiopulmonary
Texas bypass ushered in a new era for surgical management of these
#
Department of Cardiothoracic Surgery, Hebrew University, Hadassah
Medical Center, Jerusalem, Israel
cases. In 1954, Crawford et al performed the first successful
removal of a primary intracardiac myxoma.4
The authors have no disclosures for this paper
Primary cardiac tumors are rare. The estimated incidence ranges
Address reprint requests to Michael J. Reardon MD, Department of
Cardiovascular Surgery, Houston Methodist DeBakey Heart and Vas-
from 0.001% to 0.3% in autopsy studies.5 Approximately 3 quar-
cular Center, Houston Methodist Hospital, 6550 Fannin Street, Suite ters of primary cardiac tumors are benign and 1 quarter is malig-
1401, Houston, TX 77030. E-mail: mreardon@houstonmethodist.org nant.6 Of the malignant tumors, 3 quarters are sarcomas and

1043-0679/$
see front matter © 2018 Elsevier Inc. All rights reserved. 385
https://doi.org/10.1053/j.semtcvs.2018.09.001
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SURGERY FOR TUMORS OF THE HEART
1 quarter comprised of other tumors such as lymphomas and
others. Myxomas represent the most common benign primary car-
diac tumors, accounting for nearly half of all benign tumors.5 Other
less common benign primary cardiac tumors include lipoma,
hemagioma, fibroma, and paraganglioma. The majority of primary
malignant cardiac tumors are sarcomas, with undifferentiated sarco-
mas and angiosarcomas being the most common, followed by leio-
myosarcomas and rhabodmyosarcomas.5 Lymphomas represent
only 1
5% of primary malignant cardiac tumors.5 While surgery
for primary simple benign tumor is relatively straightforward, man-
agement of complex primary tumors can be a challenge.
Secondary cardiac tumors are 30 times more common than
primary cardiac tumor.7,8 The incidence ranges from 2% to
18% in patients with metastatic cancer and almost every type
of malignant tumor has been known to reach the heart.7,8 In
autopsy studies of patients with known malignancies 9.1%
have cardiac metastases, which increase to 14.2% in patients
with multiple distant metastases.7,8 The most common primary
sites of metastatic cardiac tumors are lung adenocarcinoma and
squamous cell carcinoma (36
39% of all secondary cardiac
tumors), leukemia and lymphoma (10
21%), and breast carci-
noma (10
12%) (Fig. 1).8 Up to 50% of patients with leuke-
mia develop cardiac lesions. Melanoma is also common and
28
56% of patients with metastatic melanoma have some car-
diac involvement.8 The overall prognosis of patients with can-
cer and secondary lesions to the heart remains very poor.9
Overall survival ranges from 7 months to 2 years from the time
of diagnosis, with worse prognosis once malignant pericardial
effusions occur.9
While most cases of primary cardiac tumors are sporadic, an
increasing number of cases with a genetic basis are being
Figure 1. Most common primary sites of secondary metastatic cardiac tumors.
386 Seminars in Thoracic and Cardiovascular Surgery  Volume 30, Number 4
reported. Approximately 3
10% of cardiac myxomas occur as
part of the Carney complex, a familial syndrome associated
with mutation of the tumor-suppressor gene PRKAR1A.10
Most cardiac paragangliomas occur sporadically, but recent
evidence points to a genetic basis in 25
50% of cases.11
Although 17 genes have been linked to extra-adrenal paragan-
glioma, only succinate dehydrogenase mutations were found
to be associated with cardiac paragangliomas.11 We suggest
consideration of genetic testing in patients diagnosed with car-
diac paraganglioma. Cases of cardiac sarcomas with a genetic
basis have also been recently identified and are of current inter-
est to our groups.12
CLASSIFICATION OF PRIMARY CARDIAC TUMORS
The fourth edition of the World Health Organization classi-
fication of tumors published in 2015, divides primary cardiac
tumors into benign, malignant, and intermediate tumors of
uncertain behavior.13 Benign tumors can be further subdivided
into congenital and childhood tumors (eg, histiocytoid cardio-
myopathy, rhabdomyoma, hamartoma, and cardiac fibroma),
non-neoplastic tumors of adults (eg, papillary fibroelastoma
(PFE) and lipomatous hypertrophy of the atrial septum), and
neoplastic benign tumors of adults (eg, cardiac myxoma, car-
diac paraganglioma, and cardiac lipoma). The most recent edi-
tion has simplified the classification of sarcomas with a
predilection for the left atrium: lower-grade sarcomas previ-
ously called fibrosarcoma and myxosarcoma have now been
combined in the term myxofibrosarcoma. High-grade sarco-
mas formerly called malignant fibrous histiocytoma are now
classified as undifferentiated pleomorphic sarcoma.

PATHOGENESIS AND CLINICAL MANIFESTATIONS
Primary Tumors
Primary tumors of the heart can present with a variety of non-
specific symptoms.5 In addition, a significant proportion of pri-
mary cardiac tumors is asymptomatic and is detected incidentally
during cardiovascular assessment or at autopsy. When symptoms
do occur, they can be grouped into 5 broad categories:
1) Constitutional or systemic manifestations such as weight
loss, malaise, fatigue, anemia, arthralgia, fever, polycythe-
mia, leukocytosis, and thrombocytosis.
2) Obstructive cardiac symptoms including dyspnea, orthopnea,
hemoptysis, chest pain, dizziness syncope, and sudden car-
diac death. Left atrial tumors cause symptoms by interfering
with blood flow or inducing mitral valve incompetence
resulting in heart failure or presyncope and/or syncope.
Right atrial tumors can also impair blood flow and cause
hemodynamic changes analogous to those observed with
tricuspid stenosis. Typical manifestations of right atrial
tumors include signs and symptoms of right heart failure
and symptoms secondary to pulmonary embolism. Pulmo-
nary hypertension may beget shunting through a patent
foramen ovale or atrial septal defect, resulting in hypoxemia
or paradoxical emboli. Right ventricular tumors may cause
signs and symptoms of right ventricular outflow tract
obstruction. Left ventricular intracavitary tumors may pres-
ent with left ventricular outflow obstruction.
3) Other cardiac manifestations including atrial or ventricular
tachyarrhythmia, conduction abnormalities, pericardial
effusion, and cardiac tamponade.
4) Embolic manifestations including stroke, transient ischemic
attack, myocardial infarction, peripheral arterial embo-
lism, and pulmonary embolism. In addition to impeding
blood flow, left atrial tumors can be a source of tumor
fragments or thrombi, which may detach and embolize to
the systemic circulation. The most common manifesta-
tion of such embolization is neurologic, and central ner-
vous system complications have been reported in up to
12% of patient with left atrial myxomas.14
5) Manifestations due to local invasion and metastases.
Importantly, the specific signs and symptoms of cardiac
tumors are primarily determined by tumor size and anatomical
location, not by histopathology.
Secondary Tumors
Patients with cardiac metastases are often asymptomatic as they
present late in the course of malignancy.9,15 Malignant tumors
can spread to the heart by lymphatic, hematogenous, transve-
nous, and direct invasion, which ultimately determines the type
of cardiac involvement (Table 1).7,8 Lymphatic extension of carci-
nomas from lung, breast, and esophagus often deposit in the peri-
cardium, myocardium, and epicardium.8 Hematogenous spread
leads to endocardial involvement of tumors such as melanoma,
Seminars in Thoracic and Cardiovascular Surgery  Volume 30, Number 4
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SURGERY FOR TUMORS OF THE HEART
Table 1. Clinical Manifestations of Secondary Cardiac Tumors
Pericardial Metastasis (64
69%*)
 Pericarditis
 Pericardial effusion / Tamponade
 Constrictive pericarditis
Epicardial and Myocardial Metastasis (25
34%)
 Atrial and ventricular arrhythmias and conduction
disturbances
 Systolic or diastolic dysfunction
 Myocardial ischemia or infarction
 Cardiac rupture
Endocardial and Intracavitary Metastasis (3
5%)
 Intracavitary obstruction
 Valvular regurgitation and stenosis
 Left heart failure
 Right heart failure
 Cardiogenic shock
 Pulmonary embolism
 Systemic thromboembolism
 Sudden cardiac death
Transvenous Metastasis ( < 1%)
 Superior vena cava syndrome
 Inferior vena cava syndrome
*The approximate percentage of secondary cardiac tumor involvement.4
sarcoma, lymphoma (hematogenous and lymphatic spread), and
leukemia. Hematogenous spread can also result in obstructive
coronary artery disease. Metastatic lung tumors can extend from
the lung to the pulmonary veins to enter the left atrium. Direct
extension to the heart can also occur via the superior vena cava
(SVC) and inferior vena cava (IVC). Subdiaphragmatic tumors are
frequently renal carcinomas, although hepatic, adrenal, and uter-
ine tumors occasionally have exhibited this behavior. Up to 10%
of renal cell carcinomas invade the IVC, and nearly 40% of these
reach the right atrium.8,15 Transvenous SVC involvement may
occur with lung and breast cancers, lymphoma, thymoma, and
germ cell tumors. These tumors may be associated with SVC
syndrome.8,15
Secondary cardiac tumors can be classified anatomically as
pericardial, epicardial and/or myocardial, and endocardial and/
or intracavitary.
1) Pericardial metastasis
The pericardium is the most common site of cardiac metas-
tases representing approximately two-thirds of cases.7 Most
commonly, pericardial metastases result in serosanguineous
malignant pericardial fluid but may also appear as a fibrionohe-
morrhagic pericarditis or pericardial infiltrate. Patients may
present with shortness of breath, manifest anterior or pleuritic
chest pain and/or peripheral edema secondary to pericardial
effusion. In a patient with a known primary malignancy, the
presence of signs or symptoms suggestive of pericardial effu-
sion or pericarditis is highly suggestive of metastatic pericardial
disease and should prompt a cardiac workup.
387
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SURGERY FOR TUMORS OF THE HEART
2) Epicardial and myocardial metastases
Metastatic epicardial and myocardial involvement, together,
constitute about one-third of cardiac metastatic cases.7 Palpita-
tions from arrhythmias are the most common sign of meta-
static myocardial disease. Infiltration of the conduction system
can trigger complete heart block or even ventricular fibrilla-
tion, depending on the size and location of the tumor in rela-
tion to the conduction system.16 While uncommon,
myocardial metastases may cause left or right heart failure.
3) Endocardial metastases
Endocardial and intracavitary metastases are rare forms of
cardiac metastases. While mostly asymptomatic, metastatic
endocardial and/or intracavitary lesions may lead to obstruc-
tion and embolization. Transvenous metastasis is a subset of
endocardial metastases where the tumor spread by direct vena
caval extension. Tumors may lead to venous obstruction and
pulmonary embolization.
Benign Metastatic Spread
There are 2 different clinical settings for cardiac metastatic
spread of benign smooth muscle tumors: intravenous cardiac
extension of pelvic leiomyomas, and benign metastasizing leio-
myomas from the uterus, the latter being very rare.7,8 Benign
metastasizing leiomyoma is an extra-uterine smooth muscle
cell tumor that can occur in patients with a history of uterine
leiomyoma.9 Benign metastasizing leiomyomas may form
pedunculated masses to the interventricular septum, right ven-
tricular papillary muscle, and multiple pedunculated lesions
Figure 2. Right ventricular renal cell carcinoma. CT (top right,
arrows) and MRI (top left, arrows) showing an anterior right
ventricular free-wall mass. Bottom: coronary angiography
demonstrating tumor (arrows) and blood supply from the acute
marginal coronary artery.
388 Seminars in Thoracic and Cardiovascular Surgery  Volume 30, Number 4
attached to the tricuspid valve. Immunohistochemical evalua-
tion can be performed for uterine smooth muscle estrogen and
progesterone receptors.
DIAGNOSTIC IMAGING
The goals of diagnostic evaluation are to confirm the pres-
ence of a cardiac tumor, to describe its size, location, and
extension and to rule out malignancy. The main imaging
modalities are echocardiography, magnetic resonance imaging
(MRI), and ultrafast computed tomography (CT) (Fig. 2). A
multimodality approach is desirable. In select patients, adjunc-
tive diagnostic modalities such as coronary angiography, posi-
tron emission tomography (PET), and transvenous biopsy may
be useful.
Echocardiography: Echocardiography is fast and noninva-
sive—it is the primary diagnostic modality for the assessment
of cardiac tumors. It describes the size, location, shape, attach-
ment and mobility of a tumor, as well as its relation to other
anatomical structures.5 Features suggestive of malignancy
include, location outside of the left atrium, involvement of
more than one cardiac chamber, broad base attachment, exten-
sion to the mediastinum or great vessels, presence of multiple
masses, pericardial effusion, and high enhancement after con-
trast-medium injection.17 Three-dimensional echocardiogra-
phy is an emerging modality with better spatial visualization of
intracardiac masses.18
MRI and CT: Cardiac MRI19 and ultrafast CT20 both provide
high-resolution cardiac imaging. Cardiac MRI is generally pre-
ferred due to its higher sensitivity and specificity.19 It also
allows for tissue characterization, providing insight into the
tumor type.21 While no single feature is pathognomonic of
malignancy, MRI diagnosis allows for superior correlation with
histopathologic diagnosis compared with echocardiography.22
Ultrafast CT remains a useful diagnostic tool, especially
when MRI is contraindicated or not readily available. Further-
more, CT is often the first modality to be used to screen for
metastatic disease in newly diagnosed cancers. It is an excellent
imaging modality for cardiac metastases, as well as for tumors
with direct extension to the heart. Both cardiac MRI and CT
may be useful in differentiating tumor from thrombus.23 In
addition, benign lesions such as fibromas, lipomas, and certain
sarcomas have distinctive appearances on MRI and on CT.24
Coronary angiography: Coronary angiography is required to
characterize the tumor blood supply and assess potential
involvement of the coronary arteries (Fig. 2). These data are
critical for preoperative surgical planning, as involvement of
the coronary arteries may warrant coronary artery excision and
grafting.5
PET: Florodeoxyglucose-positron emission tomography is a
functional study of glucose uptake. Especially when combined
with CT (PET-CT), it becomes an important adjunct to deter-
mine presence and localization of any metastatic deposits
when other modalities are inconclusive in determining if
abnormalities represent innocent or sinister changes.25 In addi-
tion to its utility in identifying secondary cardiac involvement
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Figure 3. Flow diagram for management of primary cardiac tumors.

Seminars in Thoracic and Cardiovascular Surgery  Volume 30, Number 4 389

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SURGERY FOR TUMORS OF THE HEART

in patients with metastatic tumors,26 PET has been useful in
diagnosing atrial myxoma27 and lipomatous septal hypertro-
phy.28 With primary malignant cardiac tumors, the search for
extracardiac metastatic disease is also important.
3D printing: This emerging technology offers a model with
segment individual anatomical components printed in distinct
colors allows for enhanced insight into the anatomical and
structural disposition of tissues can facilitate preoperative
planning.29
MANAGEMENT
Our overall treatment approach for primary benign, primary
malignant, and secondary cardiac tumors is outlined inFig-
ures 3 and4. Generally, the first decision upon finding a new
cardiac mass is to decide if it represents a tissue mass versus
nontissue such as clot, calcium, vegetation, or foreign body.
Tissue should show perfusion whereas lack of perfusion sug-
gests another diagnosis such as thrombus. If tumor is sus-
pected, then the next decision is to attempt to classify the
tumor as benign or malignant. We further subdivide tumors
into simple versus complex benign tumors and primary versus
secondary malignant tumors. The misdiagnosis of malignant
primary tumors as benign and lack of recognition for the com-
plexity of benign tumors such as paraganglioma and large
fibroma can still lead to inappropriate decisions on care. We
suggest that multidisciplinary cardiac tumor heart teams be
considered for complex primary benign and primary malignant
cases.
The management of secondary cardiac tumors is variable
and contingent on the comorbidities, clinical presentation, and
overall prognosis, which is largely based on the extent of tumor
spread and the type of cancer. Since cardiac metastases often

Figure 4. Flow diagram for management of secondary cardiac tumors.

390 Seminars in Thoracic and Cardiovascular Surgery  Volume 30, Number 4


represent one of many locations of metastatic deposits, the
main goals of therapy are the general treatment of the patients'
overall cancer, not the cardiac lesion alone. The treatment
options for patients with secondary cardiac tumors are adju-
vant or neo-adjuvant chemotherapy, radiation, surgical resec-
tion, and palliation. Most often, a combination of the
aforementioned strategies is employed.
SIMPLE BENIGN TUMORS
Myxoma
Myxomas account for 50% of all benign cardiac tumors in
adults.5 Cardiac myxomas arise from pluripotent mesenchymal
stem cells. They occur most frequently in women between the
third and sixth decade of life. The vast majority of these tumors
are sporadic, but approximately 7% occur in the context of a
large syndrome called the Carney complex.30 When they arise
in the setting of the Carney complex, cardiac myxomas show
no sex predilection and can occur at any age.
The majority of cardiac myxomas are solitary. They arise
from the endocardium and protrude into a cardiac chamber.
Cardiac myxomas show a predilection for the left atrium
(75%), followed by the right atrium (15%), with the remaining
cases equally distributed between the right ventricle and the
left ventricle. Myxomas are most frequently pedunculated and
attached to the interatrial septum with a mean diameter of 5
cm.31 They can be soft, gelatinous, or firm with a smooth vari-
colored surface that may be covered with thrombus.
Because of the risk of embolization and complications, surgi-
cal resection of myxomas is recommended in the absence of
physiologic contraindications to surgery. Myxomas can be
approached via a median sternotomy or mini-thoracotomy.
Outcomes in these cases are excellent. Our current experience
at the Houston Methodist DeBakey Heart & Vascular Center is
135 myxomas with no deaths and 2 recurrences. At the Tor-
onto General Hospital (1990
2016) we have operated on 139
myxomas with 2 deaths and 2 recurrences, both of whom had
Carney's Syndrome.
Papillary Fibroelastoma
PFEs are the second most common adult cardiac tumor
occurring in about 10% of primary cardiac tumor cases.32
They have been described as neoplasms, hamartomas, organiz-
ing thrombi, or post-traumatic tumors. They occur most com-
monly on the valvular endocardium with the non-coronary
cusp being the most prevalent but can occur anywhere on the
cardiac endocardium.33 They are usually single but can be
multiple with as many as 40 PFE reported in a single patient.34
Surgery is recommended for left-sided lesions because of the
risk of embolism. Treatment recommendations for right-sided
lesions are more problematic since the rate and risk of emboli-
zation is unclear. Given the low operative risk, most right-sided
PFE larger than 1 cm are generally removed surgically. Our
previous experience with PFE has shown 50% of the tumors
arising from the left side and most (13 of 14) involving cardiac
Seminars in Thoracic and Cardiovascular Surgery  Volume 30, Number 4
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valves. There were no operative deaths and no valves required
resection or had insufficiency after tumor excision.35
Lipoma
Cardiac lipomas are well-encapsulated homogenous tumors
composed of mature adipocytes.36 They can occur anywhere
in the heart, but the most typical locations are the endocar-
dium of the right atrium or left ventricle.37 Finding of a
low-attenuation mass with density similar to fat on CT is
pathognomonic.38 Intramyocardial lipomas can cause arrhyth-
mias and conduction abnormalities, whereas subendocardial
lipomas can embolize or cause intracardiac obstruction and/or
valve dysfunction.39 Large, symptomatic tumors should be
resected.40 Cardiac lipomas are slow growing tumors that do
not typically recur.
Lipomatous Hypertrophy of the Interatrial Septum
Lipomatous hypertrophy is the nonencapusalted collection
of mature adipocytes, with associated mature intervening
islands of myocardial or nonlipomatous cells. It is commonly
found between the right and left atria.41 It is more common
than lipomas described above and commonly occurs in the
typical location in elderly, obese females and is often found
incidentally on testing for other reasons. Cardiac MR has a typ-
ical fat signal. Although arrhythmia has been associated with
this condition, it is usually asymptomatic. There is no evidence
that prognosis is improved by resection in asymptomatic or
symptomatic patients.
Hemangioma
Cardiac hemangiomas are rare representing about 2% of pri-
mary cardiac tumors.42 These benign tumors may involve the
endocardium, myocardium, or epicardium, and they can
develop anywhere in the heart (atrial hemangiomas may be
mistaken for myxomas). Cardiac hemangiomas occur most fre-
quently in children and adolescents.43 They are classified into:
cavernous type composed of multiple, thin-walled, dilated ves-
sels, capillary type composed of smaller capillary-like vessels,
and arteriovenous type composed of dysplastic arteries and
veins.44 The cavernous and capillary types are the most com-
mon in the heart.
Most cardiac hemangiomas are discovered incidentally.44
When present, symptoms include congestive heart failure,
right ventricular outflow tract obstruction,45 coronary obstruc-
tion,46 pericardial effusion, arrhythmias,47 and sudden
death.48 The diagnosis of cardiac hemangiomas is challenging.
Characteristic findings on MRI include hyperintense signal on
T2-weighted images with gradual centripetal contrast enhance-
ment, demonstrating the hypervascular nature of hemangnio-
mas.49 Coronary angiography is also useful in mapping the
blood supply to the tumor and demonstrates a characteristic
“vascular blush” in 80% of cases.43 The natural history of car-
diac hemangiomas is unpredictable. Spontaneous regression of
these tumors is rare.
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Patients with a resectable tumor usually have a good progno-
sis and surgery is recommended.42 When complete resection is
not feasible, partial resections are thought to produce long-
term benefits.43 Patients with unresectable tumors may have a
poor prognosis due to the continued hazard of malignant ven-
tricular arrhythmias, sudden death, and tumor progression. At
the Houston Methodist DeBakey Heart & Vascular Center, we
have successfully operated on 5 cardiac hemangiomas that
were causing obstructive symptoms. We also have 3 patients
with tumors that met the characteristics of hemangioma that
are in anatomically difficult areas to resect safely. We have
been following these with patients with no changes for over
3 years.
COMPLEX BENIGN TUMORS
Fibroma
Cardiac fibromas are rare benign tumors occurring predomi-
nantly in children and adolescents.50 These tumors are solitary
and occur almost exclusively within the ventricular myocar-
dium, usually in the left ventricle free wall or septum.51 They
arise sporadically but an association with Gorlin-Goltz syn-
drome has been suggested.52 Cardiac fibromas are firm, nodu-
lar, and gray
white tumors grossly resembling uterine
leiomyomas. These tumors are generally well circumscribed
and noninfiltrating. Patients who undergo surgery tend to have
large, bulky tumors.
Clinical presentation is variable, and approximately 70% of
fibromas are symptomatic. Most cardiac fibromas produce
symptoms through arrhythmia and conduction disturbances,
intracardiac blood flow obstruction or interference with nor-
mal valvular and/or ventricular function.50,51 The natural his-
tory of cardiac fibromas is unknown. These tumors tend to
grow rapidly and have a low tendency for spontaneous regres-
sion. As such, surgical intervention should be considered.
Surgery may involve extensive resection requiring ventricu-
lar reconstruction.53 Even large left ventricular fibromas can
generally be resected and the left ventricle reconstructed since
these tumors tend to displace the left ventricular muscle away
rather than replacing it.53 Occasionally, mitral valve repair or
replacement is needed.51,53 Successful complete resection of
the tumor is curative. When the tumor cannot be removed
completely, partial resection provides good palliation.51 In
selected patients with unresectable tumors, cardiac transplanta-
tion may be considered.54 The largest published series of surgi-
cally treated cardiac fibromas includes 18 patients who
underwent surgery over a 38-year period, and were followed
for up to 34 years.51 There was one operative death and no late
deaths. There was no tumor recurrence or change in size of the
residual tumor in the one patient who had subtotal resection.
Paraganglioma
Chromaffin tumors arising from the neural crest cells origi-
nating from the sympathetic or parasympathetic chains are
classified as paragangliomas. Only about 1
2% occurs in the
392 Seminars in Thoracic and Cardiovascular Surgery  Volume 30, Number 4
chest and most of these arise in the posterior mediastinum.55
They occur most commonly in the roof of the left atrium or
the base of the pulmonary artery and aorta. These tumors can
be hormonally actively. The term pheochromocytoma is
restricted to hormonally active paragangliomas of the adrenal
gland. Most cardiac paragangliomas occur sporadically but
recent evidence points to a genetic basis in 25
50% of cases.56
About 10% of cardiac paragangliomas are malignant.57 Patients
presenting with hypertension or suspicion of tumor hormonal
activity require testing and blockade before surgery.
Complete surgical excision is the only effective treatment for
paraganglioma. Surgery is complicated by the fact that these
tumors do not have a capsule and require complete excision
for success. This lack of encapsulation and proximity to
anatomically important structure can make the surgical
approach to these highly vascular tumors very complex.58
PRIMARY MALIGNANT TUMORS
Primary Cardiac Sarcoma
Primary cardiac sarcomas are rare. Survival without surgical
resection is generally measured in months. Surgical resection
has proven the only effective therapy to date to prevent local
complications and offer some limited survival benefit.59 The
Mayo Clinic published an experience with 34 patients operated
on for primary cardiac sarcoma over a 32-year period.60 The
median survival was 12 months although this extended to 17
months if a R0 resection was achieved. We recently reported
our updated experience with 95 surgical resections for primary
cardiac sarcoma out of 131 patients seen.61 The 1-year survival
in this group was 65%. Our current experience includes 105
surgical resections for primary cardiac sarcoma. We have used
this experience combined with the literature to define our cur-
rent approach to primary cardiac sarcoma.
We classify primary cardiac sarcoma by its location, rather
than histologically, because we find that this determines the
presentation, urgency of intervention, and surgical options
available.6,62 Our classification divides primary cardiac sarco-
mas into right heart, left heart, and pulmonary artery sarco-
mas.
Right Heart Sarcoma
The majority of right heart sarcomas are angiosarcomas.61
These tumors are 2
3 times more common in men than in
women. They may occur in the right atrium or ventricle but
show a strong predilection for the right atrium. Right heart sar-
comas tend to be bulkier, more infiltrative, and metastasize
earlier.63 These tumors tend to aggressively invade adjacent
structures, including the great veins, tricuspid valve, right ven-
tricular free wall, interventricular septum, and right coronary
artery.
When feasible, complete surgical resection is the mainstay of
therapy, and it is the only treatment modality that has been
shown to improve survival.64 However, complete surgical
resection is not always possible due to the bulky infiltrative

nature of right heart sarcomas and the high incidence of meta-
static disease at presentation. Our approach to the treatment of
right heart sarcoma is for neoadjuvant chemotherapy following
biopsy diagnosis.65 Doxorubicin and ifosfamide are our stan-
dard chemotherapy regimens, which are continued until unre-
sponsive. Then candidacy for surgical resection is evaluated.
Patients with even limited metastatic disease who did not
respond well to chemotherapy and those who developed new
metastatic disease while on treatment are not considered candi-
dates for surgery. Patients whose primary tumor does not
respond well to this regimen tend to have very poor survival
even with surgery. Patients with widely metastatic disease are
also not considered candidates, unless palliative surgery is
undertaken to relieve severe symptoms. In our experience, the
introduction of this standardized multimodality treatment pro-
tocol, largely centered on the use of neoadjuvant chemother-
apy, led to a significant increase in the rate of microscopically
complete resection (ie, with R0 margins) from 24% to 61% (P
= 0.03). Neoadjuvant chemotherapy was also associated with a
doubling of median survival (20 vs 9.5 months).65
Due to their infiltrative nature, right heart sarcomas require
extensive resection to achieve microscopically negative mar-
gins. The right atrium can be completely resected and patch
reconstructed.66 Surgical resection of the right atrium, the right
coronary artery, tricuspid valve and up to a third of the right
ventricular wall can performed. Total cardiac replacement with
biventricular assist device implantation67 or total artificial heart
implantation68 have been described in cases where surgical
resection is so extensive that it precludes reconstruction. Rates
of local recurrence are low in patients undergoing radical resec-
tion, and the leading cause of death in these patients is distant
metastatic disease. The primary reason for local failure is
incomplete resection.
Left Heart Sarcoma
Primary sarcomas of the left heart are usually found in the
left atrium and are most commonly solid, without the degree
of infiltration seen in right heart sarcomas. They are less likely
to have metastasized at the time of presentation.62 They often
present with acute-onset heart failure secondary to obstruction
of forward flow so neoadjuvant chemotherapy is rarely consid-
ered possible. Left heart sarcomas are frequently misdiagnosed
as cardiac myxomas. In these cases of misdiagnosis, initial sur-
gical resection often results in a positive margin followed by
rapid recurrence. We recommend chemotherapy for these
cases if the tumor has been completely removed grossly and
there is no tumor mass to image. If the tumor recurs, repeat
resection with specialized techniques such as cardiac auto-
transplantation to allow complete resection will need to be
considered.
Left heart sarcomas present a surgical challenge in terms of
achieving complete resection. Orthotopic heart transplantation
has been considered, although limited donor supply, immuno-
suppression, and limited survival benefit have all dampened
enthusiasm for this approach. Cardiac autotransplantation is a
Seminars in Thoracic and Cardiovascular Surgery  Volume 30, Number 4
ADULT
SURGERY FOR TUMORS OF THE HEART
technique of cardiac explantation, ex vivo tumor resection,
reconstruction, and reimplantation.69 Our group first used this
technique to successfully treat a large left atrial sarcoma not
resectable by standard methods.70 We have since used cardiac
autotransplantation for large intracavitary left ventricular
tumors to avoid ventriculotomy71 and for left atrial sarcomas.72
Incomplete resection of primary cardiac sarcoma is universally
associated with rapid recurrence and very poor outcomes.
The largest published series includes 34 patients, of whom
26 had primary cardiac sarcomas. The 1- and 2-year survival
for primary cardiac sarcoma in this series was 46% and 28%,
respectively. These large tumors often involve extensive cardiac
and lung involvement. Of those requiring cardiac autotrans-
plant for complete resection, hospital mortality was 11%. For
the cases requiring cardiac autotransplant and pneumonec-
tomy, hospital mortality was 43%. Death in the latter cases
requiring pneumonectomy was from bleeding into the
pneumonectomy space from the site of chest wall adhesion
takedown. We have recently approached this complex combi-
nation by separating the surgery into 2 stages: (1) cardiac resec-
tion first then (2) standard pneumonectomy after the metabolic
disturbance of cardiopulmonary bypass has abated. We refer to
this approach, which has been successful in addressing this
problem, as the Texas Two Step. (JTCVS, In Press)
Pulmonary Artery Sarcoma
Pulmonary artery sarcoma is unusual with fewer than 250
cases reported as of 2009.62 Patients usually present with
symptoms of dyspnea, cough, hemoptysis, or chest pain, often
resulting is a misdiagnosis of pulmonary embolism.62,73 Che-
motherapy alone has shown poor results.74 Treatment
approaches have included palliative pulmonary artery stenting,
pneumonectomy, debulking, tumor endarterectomy, and wide
surgical excision. The Mayo Clinic has reported a series of 9
patients having surgical resection collected over a 19-year
period.75 Our group reported a series of 8 pulmonary artery
sarcoma resections with a mean survival of 24.7 months com-
pared to 8.0 months found in the literature.62 We have shown
that wide excision that often includes the pulmonary trunk can
be safely done and improve survival.62
Systemic Therapy in the Management of Malignant
Primary Cardiac Tumors
Malignant primary cardiac tumors have a propensity for
early metastatic dissemination and systemic neoadjuvant ther-
apy should be strongly considered in hemodynamically stable
patients with localized disease as it allows for: (a) easier resec-
tion by reducing the tumor volume and (b) reduction in the
risk of systemic relapse. These advantages exist only in cases
where response to therapy is demonstrated. A combination of
doxorubicin and ifosfamide is used in the neoadjuvant therapy
of most sarcomas of the right and left heart. At our center,
most patients receive 6 cycles of therapy with imaging every 2
cycles to confirm response to therapy. Other regimens includ-
ing gemcitabine and/or docetaxel combination and single agent
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SURGERY FOR TUMORS OF THE HEART

paclitaxel (for patients with angiosarcoma) are available for

these patients. Patients are seen by both medical and surgical
oncology and a joint decision-making process is in place
regarding ongoing medical therapy and the timing of surgical
intervention.
In patients with metastatic disease, systemic therapy forms
the mainstay of treatment. In addition to cytotoxic chemother-
apy, patients also have targeted therapy and immunotherapy
options that have not been investigated in the adjuvant and/or
neoadjuvant setting are available. Prominent targeted therapy
options include antiangiogenic agents such as pazopanib76 and
sorafenib.77 Emerging evidence also suggests the utility of
immunotherapy in these patients but large studies are currently
unavailable to support their use in standard of care. Undiffer-
entiated pleomorphic sarcomas tend to respond well to PD-1
inhibition using pembrolizumab and response rates as high as
40% have been reported in a small subset of 10 patients.78

SECONDARY TUMORS
For symptomatic or large pericardial effusions (or tampo-
nade), pericardiocentesis for both diagnosis and treatment
should be considered. Malignant recurrent effusions may
require the use of a sclerosing agent or drainage with a subxi-
phisternal windows or percutaneous approach. The complica-
tion rate for open compared to a percutaneous approach is
higher.79,80 Surgical therapy is directed at providing symptom-
atic palliation with minimal patient discomfort and hospital
stay. This is most readily accomplished via subxiphoid pericar-
diotomy, which can be accomplished under local anesthesia if
necessary with reliable relief of symptoms, relatively low recur-
rence rate and little mortality. Alternatively, a large pericardial
window in the left pleural space can be created using thoraco-
scopy, but we recommend this only under unusual circum-
stances. The latter can be accomplished with minimal patient
discomfort but does require general anesthesia with single-
lung ventilation and may be poorly tolerated by patients with
hemodynamic deterioration secondary to large effusions.
Patients with atrial or ventricular arrhythmias can be
managed with oral antiarrhythmic medication and/or radio-
frequency ablation. Generally arrhythmias secondary to
metastatic invasion of conduction tissues are difficult to con-
trol. Symptomatic heart block in the individual with a
reasonable prognosis may be treated with permanent pace-
maker but there is little if any role for implantable defibrilla-
tors in patients with advanced cancer.
Solid cardiac tumors can be treated with radiation. However,
doses are limited due to cardiotoxicity, and while effect has
been shown, its role in metastatic cardiac lesions is limited.81
Surgical Resection
Surgical tumor resection may be considered in highly
selected malignant cases (Fig. 5). Ideally, individuals have iso-
lated cardiac disease with a controlled primary tumor. The
lesion should be resectable while preserving sufficient cardiac
function to sustain life. Surgical resection should be given
Figure 5. Right atrial and left ventricular melanoma. Left: CT
shows right atrial (arrowhead) and left ventricular tumor masses
(arrows). Right top: the left ventriculotomy and ventricular api-
cal tumor. Right bottom: resected left ventricular tumor with
ventricular freewall.
particular consideration in individuals with endocardial metas-
tases to prevent or treat acute and complete obstruction. While
preliminary biopsy and tissue diagnosis are preferable, the
tumor is not always accessible. If imaging studies and history
suggest metastatic disease, consideration for excisional biopsy
is considered. These resections are often larger than anticipated
and should likely be undertaken in a center of excellence for
cardiac tumors—both for the decision to operate and for opti-
mal operative and postoperative care. It is unknown if resec-
tion of isolated cardiac metastatic deposits prolongs life or
disease-free survival, although there are instances with pro-
longed survival. While technically achievable, surgery for
debulking plays little to no role especially without adjuvant
therapy due to early local recurrence of disease.
When a patient presents with both a peripheral and isolated
cardiac lesion, biopsy of the peripheral lesion is paramount for
tumor characterization and to determine response to therapy.
PET-CT can often help to delineate if the 2 lesions are biologi-
cally similar. If the response to chemotherapy is poor, and the
cardiac lesion grows on chemotherapy, resection should not be
undertaken. If the cardiac tumor responds completely and is
no longer visible even on MRI, operation is delayed until or if
the lesion reappears. If one or more tumors grow, despite
response of others, the overall prognosis is poor and operation
is not appropriate. Surgery is undertaken if and/or when the
tumor stabilizes after an initial decline in size. This response to

394 Seminars in Thoracic and Cardiovascular Surgery  Volume 30, Number 4


systemic agents offers the best opportunity for complete resec-
tion, although not necessarily for overall prognosis. Any meta-
static lesions found at operation that were not appreciated on
preoperative imaging portends poor prognosis, especially if
viable tumor is found.
Renal Cell Tumors
For direct invasive renal carcinomas, radiation and chemo-
therapy are not effective in relieving the obstruction of blood
flow and surgical resection should be considered. If the kidney
can be fully removed, as well as the tail of the tumor thrombus
in the IVC, survival can approach 75% at 5 years.82
Renal cell tumors with atrial extension are typically resected
with abdominal dissection to ensure resectability of the renal
tumor. Initially, we performed a concomitant median sternot-
omy and used cardiopulmonary bypass with hypothermic cir-
culatory arrest. However, we have changed our approach and
now work closely with our liver transplant surgeons who have
extensive experience in operating on the retro-hepatic vena
cava. We expose the vena cava to the right atrium through an
abdominal incision. With ligation of the arterial inflow, the
tumor tail often shrinks below the diaphragm, and in almost
all circumstances, this can be removed without the use of car-
diopulmonary bypass (CPB). Occasionally venovenous bypass
(as used in hepatic transplantation) may be necessary to
occlude inflow through the IVC. If the tumor is too complex
for this maneuver, then a median sternotomy is performed,
and CPB with hypothermic circulatory arrest can be used to
remove the tumor from the cardiac chambers down into the
IVC. Other subdiaphragmatic tumors with atrial extension that
have been resected in a similar manner including hepatic and
adrenal carcinoma, as well gynecologic tumors.82,83
Lung Tumors
We advocate for surgical treatment in selected patients with
advanced carcinoma of the lung with intracardiac involvement.
A partial atrial resection may be achieved by opening the peri-
cardium and freeing the posterior left atrium from its pericar-
dial attachments, a technique used routinely in lung
transplantation. This approach makes it easy to apply an atrial
vascular clamp. When the atrial clamping technique is not pos-
sible, cardiopulmonary bypass can be used to facilitate com-
plete resection. Total cardiopulmonary bypass is used with
either cardioplegia or fine ventricular fibrillation. These techni-
ques may improve the chances of survival for patients in
whom advanced carcinoma of the lung has spread to the peri-
cardial or cardiac structures.
Our group has also reported surgical resection of direct
spread of advanced nonsmall cell lung carcinoma, which was
incorporated into the inferior pulmonary vein and extended
into the left atrium, right atrium, and the interatrial septum.84
The tumor and the pulmonary specimen were excised success-
fully en bloc, the atrial margins were closed primarily, and the
pericardial defect was closed with a patch. Several groups have
Seminars in Thoracic and Cardiovascular Surgery  Volume 30, Number 4
ADULT
SURGERY FOR TUMORS OF THE HEART
similarly described resection of lung carcinoma with direct
atrial invasion with reasonable midterm success.85
HEART TEAM
We began a multidisciplinary cardiac tumor team that
included a cardiac surgeon, cardiologist, cardio-oncologist,
oncologist, radiologist, and palliative care specialists in 2002 at
the Houston Methodist Hospital and MD Anderson Cancer
Center. Since then we have extended this cardiac tumor heart
team to an international effort. We believe that complex benign
and all malignant cardiac tumors should be referred to an
experienced, multidisciplinary team. Realistic goals and expect-
ations are discussed with the patient. While the patient is cen-
tral, family support, care and understanding is very important
to the overall treatment plan and should not be forgotten.
CONCLUSION
Here we provide an overview of cardiac tumors for the heart
surgeon. Simple, benign primary cardiac tumors such as myx-
oma, fibroelastoma, lipoma, and hemangioma are generally
safely treated with excellent outcomes at most cardiac centers.
Complex benign such as paraganglioma and fibroma and pri-
mary malignant cardiac tumors, commonly sarcomas, are less
uncommon and more complex. Secondary cardiac tumors are
more common but have poor overall outcomes. Medical man-
agement of secondary cardiac tumors varies widely but may
include radiation and chemotherapy. Surgical management
may include percutaneous or open pericardial drainage for
effusion and open surgical resection in highly select patients.
Few surgeons or even institutions gain much experience with
these complex cardiac tumor entities and we recommend care
by an established multidisciplinary cardiac tumor team in a
highly-experienced center of excellence. As always, individual-
ized clinical assessment is of utmost importance for decision-
making.
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