Cardiac tumours

INTRODUCTION
 Primary tumors of the heart are rare across all
age groups, with a reported prevalence of
0.001% to 0.03% in autopsy series.
 Secondary involvement of the heart by
extracardiac tumors is 20 to 40 times more
common than by primary cardiac tumors.
 In the fetus, there is a higher proportion of
germ cell tumors.

 About 75% of all primary cardiac tumors are
regarded as benign neoplasms and cardiac
myxoma accounts for at least half of them.
 By virtue of their anatomic locations, primary
cardiac tumors are capable of producing
myriad cardiac, embolic, and systemic
symptoms

 Of the remaining 25% (malignant neoplasms)
the majority are sarcomas; lymphomas are the
next most common.

 The diagnosis of primary cardiac tumors is
frequently challenging.
 The symptoms associated with most primary
cardiac tumors are nonspecific, and this
elusiveness often results in a delay in the
diagnosis of disease.

Extra Cardiac Tumors can involve the heart by
direct invasion from adjacent malignancies
(lung, breast), by lymphatic spread, or by
metastatic spread of distant disease (lymphoma,
melanoma).

Nonprimary cardiac tumors can affect the heart
by:
Invasion of the pericardium, epicardium,
myocardium, or endocardium
Production of biologically active substances
Toxic effects of treatment on the heart (e.g.,
radiation or chemotherapy)

 Myocardial involvement by metastatic disease
is less common than pericardial involvement,
but does occur, particularly with lymphoma or
melanoma.
 Intramyocardial masses can project into or
compress cardiac chambers, resulting in
hemodynamic compromise.
 Endocardial involvement is rarely seen.

EPIDIMEOLOGY
 The types of tumors predominantly seen in
adults (>16 years) are different from that seen
in children (<16 years)
 85% of benign tumors in adults are myxomas,
lipomatous tumors, and papillary
fibroelastomas
 82% of benign tumors in children are
rhabdomyomas, teratomas, and
fibromas.

 The most common malignant tumors in adults
are angiosarcomas, rhabdomyosarcomas,
mesotheliomas, and fibrosarcomas
 In children they are rhabdomyosarcomas,
fibrosarcomas, malignant teratomas, and
neurogenic sarcomas.

CLINICAL MANIFESTATIONS
 The clinical manifestations can be divided into
four general mechanistic categories:
1. Systemic manifestations
2. Embolic manifestations
3. Cardiac manifestations
4. Phenomena secondary to metastatic diseases

SYSTEMIC MANIFESTATIONS
 Constitutional symptoms of fever, chills,
fatigue, malaise, and weight loss.
 In addition, myalgia, arthralgia, muscle
weakness, and Raynaud phenomenon.
 Routine laboratory tests may reveal
leukocytosis, polycythemia, anemia,
thrombocytosis, thrombocytopenia,
hypergammaglobulinemia, and increased
ESR.

 These systemic manifestations are believed to
be produced by secretory products released
by the tumor or by tumor necrosis.
Most commonly seen in : Cardiac Myxoma
Caused by : Increased Serum IL-6 levels.

EMBOLIC PHENOMENON
 Systemic or pulmonary embolism via tumor
emboli or thromboemboli from or formed on
the surface of the tumor, respectively.
 The propensity to embolize depends on the
origin of the tumor (intramural or intracavitary),
the type of the tumor, and the friability of the
intraluminal tumor surface.

Brain emboli most commonly causes TIA or
ischemic stroke but rarely ICH can be the
presentation.
Neurological manifestations depend upon the
area affected.

 Coronary Artery Embolism can be seen rarely
and mimicks as M.I.
 Pulmonary embolization is also rare, more
with Right sided tumors and Left sided tumors
in presence of Intracardiac L-R shunt.

CARDIAC MANIFESTATIONS
Mechanisms
1. Direct mechanical interference with
myocardial or valvular function
2. Interruption of coronary blood flow
3. Interference with electrophysiologic
conduction
4. Accumulation of pericardial fluid.

 PCT that are completely intramural or
myocardial are typically asymptomatic,
especially if the sizes are small.
 Large intramural tumors that are within or
pressing on major cardiac conduction
pathways may, however, cause a wide variety
of arrhythmias, including complete heart block
and asystole in more severe cases.

 In addition, large intramural tumors may also
compress the cardiac cavities, obstruct the
ventricular outflow tract, or contribute to
insufficiency of the mitral valve.

 Intracavitary component tend to cause more
symptoms for patients specially if
pedunculated and mobile because of their
tendency to interfere with valvular and
myocardial function.

 For left atrial tumors, intracavitary lesions that
are pedunculated and mobile can interfere
with the mitral valve and produce clinical
findings of mitral regurgitation that include
fatigue, dyspnea, orthopnea, paroxysmal
nocturnal dyspnea, cough, hemoptysis, chest
pain, pulmonary edema, and peripheral
edema.

 However, findings atypical for mitral
regurgitation, such as the aforementioned
systemic and constitutional symptoms, should
prompt further investigation.
 These symptoms can be sudden in onset,
intermittent, and positional.

 Physical examination may reveal signs of
pulmonary congestion with an S3 and loud
and widely split S1, a holosystolic murmur
most prominent at the apex with radiation to
the axilla, a diastolic murmur from turbulent
blood flow through the mitral orifice, and a
tumor “plop.”

TUMOR PLOP
The ultrafast computed tomographic (UFTC) and
Echo studies show the tumor obstructing the mitral
orifice with a piston-like action, first pushing a bolus of
blood into the ventricle and then coming to rest in the
mitral orifice.
This sudden bolus of blood plus tumor in the ventricle
causes a third sound, called a "tumor plop," while
relieving the left atrium of that volume and causing an
abrupt y-descent in the left atrial pressure.
Sound is evanescnent and positional

 It occurs later than an OS but earlier than an
S3.
 For intracavitary tumors located in the right
atrium, findings of right-sided heart failure that
include fatigue, peripheral edema,
ascites,hepatosplenomegaly, and elevated
JVP with a prominent a wave are the most
common cardiac presentations.

 Because of the right atrial location, the
diagnosis is often delayed, with an average
time interval from presentation to the correct
diagnosis of 3 years.

 Patients frequently present with rapidly
progressive right-sided heart failure and also
new-onset heart murmurs because of
mechanical interference with the tricuspid
valve.

 In patients with a PFO, the buildup of right
atrial pressure can produce right-to-left
intracardiac shunting with resulting systemic
hypoxia, cyanosis, clubbing, and
polycythemia.

 On occasion, patients may also present with
superior vena cava syndrome caused by a
large right atrial tumor.

SVC SYNDROME
 In the midthird of the mediastinum, the left and
right brachiocephalic veins join to form the
SVC.
 The SVC then extends caudally, courses
anterior to the right main bronchus, and
terminates in the superior aspect of the right
atrium.
 The SVC is joined posteriorly by the azygos
vein and runs posterior to and to the right of
the ascending aorta.

 During its course the SVC is adjacent to the
right paratracheal, azygous, right hilar, and
subcarinal lymph node groups.
 Blood flow in the venous system is under low
pressure and the vessel itself is thin walled.
 Any inflammatory process in the mediastinum
or enlargement of the lymph nodes or
ascending aorta can cause the SVC to be
compressed

 First described by William Hunter in 1757 in a
patient with a syphilitic aneurysm of the
ascending aorta.
 Now the most common cause of SVC
syndrome is malignancy, of which lung
carcinoma is the most frequent, followed by
lymphoma and metastatic cancer.

 The severity of the syndrome depends on the
rapidity of onset of the obstruction and its
location.
 The more rapid the onset, the more severe the
symptoms because collateral veins do not
have time to distend and accommodate the
increased blood flow.
 A typical manifestation consist of facial
edema, dyspnea, and cough.

 Facial edema is seen most frequently; it is
worse in the morning and gets better during
the day as the patient ambulates.
 Other symptoms that occur less frequently
include stridor, headache, syncope, dizziness,
hoarseness, and confusion.
 Common findings on examination include
facial edema, distended neck and chest veins,
arm edema, and facial plethora

 Physical examination may reveal findings of
peripheral edema, hepatosplenomegaly,
ascites, elevated JVP with prominent a wave
and steep y descent, and an early diastolic
murmur or holosystolic murmur that exhibits
significant respiratory or positional variation.

 LV tumors can obstruct the LVOT and produce
findings of left-sided heart failure and syncope
as well as atypical chest pain from obstruction
of a coronary artery by either direct tumor
involvement or tumor emboli.
 Physical examination may reveal evidence
of pulmonary edema, low blood pressure, and
systolic murmurs that mimic the findings of
aortic or subaortic stenosis.

 The murmurs and blood pressure may display
considerable positional variation.
 In the case of malignant primary cardiac
tumors, such as angiosarcomas and primary
cardiac lymphomas, malignant hemorrhagic
pericardial effusion may be present.
 Life-threatening cardiac tamponade and
cardiac rupture leading to sudden death may
also occur

METASTATIC DISEASES
 Truly metastatic diseases are by definition
features of malignant primary cardiac tumors.
 Common sites of metastases :lung, brain,
and bone,although metastases to the liver,
lymph node, adrenal gland, spleen, and skin
are also seen.

DIAGNOSTIC APPROACH
 The key to proper and timely diagnosis of a
primary cardiac tumor lies in the consideration
of primary cardiac tumor in the differential
diagnosis especially when atypical features
are present.

 When cardiac tumor is considered in the
differential diagnosis, the most ideal initial
method of evaluation is echocardiography
either transthoracic or transesophageal,
depending on the clinical circumstances.

 TEE is especially advantageous in evaluating
right atrial tumors.
 3D echocardiography can assess the size of
cardiac masses and describe complex
anatomy of the heart.
 If a cardiac lesion is identified, chest CT with
contrast enhancement and CMR with contrast
are superior modalities for delineation of the
extent of tumor involvement

CT and CMR are particularly good at depicting
the pericardium and great vessels and
evaluating the extent of disease, and CT can
also detect calcification, which is important in the
differential diagnosis.

 Features suggestive of malignant cardiac
tumors include a large broad-based lesion
occupying most of the affected cardiac
chamber, hilar lymphadenopathy, extensive
pericardial involvement, and hemorrhagic
pericardial effusions.
 Depending on the clinical settings, tissue
diagnosis may be made with less invasive
methods such as cytologic evaluation of
pericardial or pleural fluids.

 However, a negative finding on biopsy
performed through these less invasive
methods does not rule out a diagnosis of
malignancy because the false-negative rate of
these methods can be significant.

 More invasive methods of tumor biopsy
through mediastinoscopy or even thoracotomy
may be necessary to obtain a definitive
diagnosis.
 Of note, the great majority of malignant
cardiac tumors represent local or distant
metastases by an extracardiac tumor, and
most malignant primary cardiac tumors are
diagnosed at an advanced stage with distant
metastases already present.

ECHO EVALUATION
A cardiac mass is defined as an abnormal
structure within or immediately adjacent to the
heart. There are three basic types of cardiac
masses:
Tumor
Thrombus
Vegetation

The first step in assessing a possible cardiac
mass is to ensure it is not an artifact caused by
electrical interference, characteristics of the
ultrasound transducer/system, or various
physical factors influencing image formation
from the reflected ultrasound signals.

 Appropriate transducer selection, scanning
technique, and evaluation from multiple
examining windows will help to distinguish
artifacts from actual anatomic structures

DIFFIRENTIAL DIAGNOSIS OF
MASS

 A specific type of cardiac involvement by tumor
that should be recognized by the
echocardiographer is the extension of renal cell
carcinoma up the inferior vena cava.
 A “fingerlike” projection of a tumor may
protrude into the right atrium (RA) from the
inferior vena cava, and the tumor can be
followed retrograde (from a subcostal
approach) back to the kidney.

 Correlation with other wide-view imaging
techniques is needed for full delineation of the
tumor extent. Uterine tumors also occasionally
present in this fashion.

BENIGN TUMORS
 MYXOMAS
1. Most common type of primary cardiac
tumor
2. 30% to 50% of all primary tumors
3. Annual incidence of 0.5 per million
population
4. Most commonly presents in adults 30 to
50 years and 65% in Women

5. 4.5% to 10% myxomas are familial
6. Arises from multipotent mesenchymal cells
7. Typically benign but embolic tumor fragments
can be deposited along arterial circulation.

 The pathogenesis of cardiac myxoma is poorly
understood, especially for those tumors that
occur sporadically.
 Studies have, however, shed more light on
the pathogenesis of familial cases of cardiac
myxomas.
Carney syndrome accounts for the majority of
familial cases of cardiac myxoma and for 7%
of all cardiac myxomas.

CARNEY SYNDROME
 Autosomal Dominant syndrome
 Myxoma in cardiac and several extracardiac
locations
 Spotty skin pigmentation
 Endocrine hyperactivity
 Other tumors (such as testicular Sertoli cell
tumor, pituitary adenoma, and thyroid tumors).

 Carney syndrome show no age or gender
predilection, can be single or multiple, can
occur in any intracardiac location, and tend to
recur with a rate of 20% despite adequate
surgical excision.
 In contrast, sporadic cases of cardiac myxoma
tend to occur in women of middle age and as
isolated lesions in the left atrial aspect of
interatrial septum.

 Sporadic cases have a lower recurrence rate
(roughly 3%) than those mentioned previously.
 Mutations in PRKAR1A, a phosphorylated
perinatal myosin isoform.

 Majority of the patients will present with at least one
of the classic triad of obstructive cardiac, embolic,
and constitutional or systemic signs.
Obstructive cardiac findings
 Dizziness
 dyspnea
 Cough
 pulmonary edema
 heart failure.

Result of mechanical interference of mitral valve
by the tumor and account for the most common
presenting findings in the triad.
Tumor embolism
Most common cardiac tumor to embolise and
virtually to every organ
Symptoms depend on the location of the tumor
and the patency of the foramen ovale.

INVESTIGATIONS
2/3rd
have abnormal ECGs mainly showing LA
enlargement while atrial arrythmias and conduction
disorders are rare
2/3rd
pts CXR shows evidence of elevated LA
pressure, such as LA enlargement, vascular
redistribution, prominent pulmonary trunk
Pulmonary edema, is found in 53% of patients with
LA myxoma.

 Cardiomegaly is seen in 37% and 50% of LA
and RA myxomas, respectively.
 Intracardiac tumor calcification is a rare finding
in LA myxomas but is in 56% of patients with
right atrial myxoma.
 The typical imaging modalities used for
diagnostic and preoperative assessment
purposes include CT, CMR, and
echocardiography.

 Most cardiac myxomas appear as spherical or
ovoid masses with lobular contour on CT and
CMR scans.
 Two thirds of myxomas are heterogeneous,
whereas one third appear homogeneous on
CT.

 Contrast-enhanced CT reveals that most
myxomas have an overall attenuation lower
than that of myocardium
 Echocardiography is the most commonly used
modality for diagnostic purposes, and TEE is
the preffered echo modality.

 83% in LA and 12.7% occur in RA and 1.3% are
biatrial.
 Only 1.7% and 0.6% of myxomas occur in the LV
and RV respectively.
 Usually pedunculated tumors with a fibrovascular
stalk attaching to the subendothelial base of IAS in
the region of the fossa ovalis.
 Rarely, cardiac myxoma can involve heart valves
directly.

 Size usually 4 to 8 cm in diameter with
maximum reported as 16cms.
 The mean weight is 37gm.
 Half of the cardiac myxomas have a smooth
compact surface, and half have a villous
surface.
 Myxomas with a villous surface are more likely
to embolize.

 The treatment is prompt surgical resection
 Complete excision is the goal, although this
may not be possible in all instances.
 Postoperative mortality in most series ranges
from 0% to 7.5%.

 Recurrence rate is 3% which can be local or in
extracardiac locations, such as the brain, lung,
skeletal muscle, bone, kidney, gastrointestinal
tract, skin, and other soft tissue sites.

 Myxomas may be sessile or pedunculated,
spheric or polypoid.
 “Syndrome myxoma” refers to one of several
genetic disorders which should be suspected
in patients< 40 years with multiple cardiac
myxomas.

Myxoma syndromes include LAMB
(lentigines, atrial myxomas, mucocutaneous
myxomas, and blue nevi),
NAME (nevi, atrial myxomas, myxoid
neurofibroma, and ephelides)
Carney syndrome (atrial, cutaneous and
mammary myxomas, lentigines, blue nevi,
endocrine disorders and testicular tumours).

LIPOMAS AND LIPOMATOUS
HYPERTROPHY
 Benign lipomatous tumor is the second most
common primary neoplasm of the heart and
can be divided into two major groups primarily
on the basis of the degree of encapsulation
 Lipoma
 Lipomatous hypertrophy of the atrial septum
(LHAS).

 Sporadic with equal occurences in both
genders.
 Clinically, mostly asymptomatic and typically
are incidental findings.
 Can occur at any atrial or ventricular surface
most commonly in the subepicardial and
subendocardial locations, although
intramyocardial lesions have also been
reported.

 Subendocardial lipomas with a prominent
intracavitary component can result in
symptoms of heart failure.
 Subepicardial tumors are usually
asymptomatic, but large lesions may cause
compression of the heart and produce
pericardial effusion.
 Intramyocardial lipoma may interfere with
electrical conduction in the heart and cause
arrhythmias.

 TEE followed by CT is the modality of choice
as they display a low attenuation signal similar
to subcutaneous or mediastinal fat.
 Size typically ranges from 1 to 8 cm in
diameter.
 The treatment of symptomatic cardiac lipomas
is surgical resection, and the postoperative
prognosis is excellent.

LHAS
 LHAS are massive fatty deposits of the atrial
septum, is a nonencapsulated excessive
accumulation of fat in the atrial septum at the
level of the fossa ovalis that is more than 2 cm
in thickness and typically occurs in elderly,
obese patients.
 Mean age of diagnosis - 70yrs.

 Clinically LHAS does not cause any
symptoms.
 Can result in rhythm disturbances and even
SCD due to fatty tissue infiltration into atrial
myocyte tissue, which alters the architecture
and function of the myocytes.
 In rare instances in which the tumor protrudes
into the right atrium and the superior vena
cava, patients can present with symptoms
related to blood flow obstruction.

 CT and CMR are the most desirable as they
are superior to ECHO in differentiating
between fat and connective tissue.
 Atrial septum is thickened to up to 7 cm,
whereas normally it is less than 1 cm but this
thickening always avoids the fossa ovalis,
giving the atrial septum a dumbbell or
hourglass shape.
 LHAS with symptomatic arrhythmias can be
managed medically

 Surgical management should be restricted to
hemodynamically compromised patients.

PAPILLARY TUMORS OF
HEART VALVES
 Papillary fibroelastoma is the third most
common primary cardiac tumor
 INCIDENCE 0.33%
 Fibroelastic growth distinct from lambl
excrescences and does not spontaneously
regress which may have focal calcification
 Clinically mostly asymptomatic.

 A series showed 50% pts having
TIA,stroke,angina, myocardial infarction, and
dyspnea.
 Cerebral embolic symptoms are present
in>50% of the symptomatic patients.
 Rarely, patients present with SABE and PE
and sudden death have also been reported.
 Firmly attached to the valvular or mural
endocardium.

 TEE is the recommended imaging modality for
the diagnosis for lesions measuring 2 cm or
more.
 Generally appears as a round, oval or irregular
lesion well demarcated
 80% to 90% are found on the valvular
endocardium most commonly on Aortic Valve.

 44% have a 1-to 3-mm stalk, and this mobile
type of papillary fibroelastoma appears more
likely to give rise to embolism.
 The treatment of papillary fibroelastoma is
surgical excision or tumor shaving from the
valvular leaflets with either reconstitution or,
less commonly, replacement of the valve.
 Asymptomatic patients with small, left-sided,
nonmobile-type can be observed and excision
for large >1cm mobile symptomatic cases.

RHABDOMYOMA
 Most frequent PCT in infants and children.
 80% in <1 year but adult presentation also
seen while rare.
 Arrhythmias represent the most common
presentation in adults
 Imaging: well-circumscribed masses with
hyperintense T1- and T2-weighted spin-echo
images and hypointense images after the
administration of gadolinium.

 70% atrial origin and 30% ventricular
 In symptomatic patients with arrhythmias,
antiarrhythmic drugs may be used
 Surgical excision is indicated if the drugs fail to
control symptoms.
 No recurrence
 Prognosis of this disease is excellent

FIBROMAS AND
HAMARTOMAS
 Cardiac fibroma: 2nd
most common PCT in
infants and children.
 90% in children <1 year
 No sex predilection
 Majority appear to be nonfamilial.
 Majority of cardiac fibromas behave like
hamartomas with no tendency to recur or to
grow aggressively.

 Usually cause arrhythmogenic disorders as
VT,AF, and RBBB and LBBB.
 They typically appear as subendocardial
yellow-tan nodules or plaques.
 The first line of treatment is antiarrhythmic
drugs, allowing the regression of lesions.
 Other treatments include surgical excision,
electrophysiologic mapping and cryoablation,
or direct-vision cryoablation of nodular tumors.

 Clinically, heart failure, arrhythmias, and
syncope are presenting findings while less
common presenting findings include sudden
death and atypical chest pain.
 1/3rd
of patients with cardiac fibroma can
remain asymptomatic.
 ECG may show evidence of LVH,RVH, BBB,
AV blocks and VTs

 CXR may reveal cardiomegaly with or without
a focal bulge, and calcification is visible in
15% of cases.
 ECHO : cardiac fibroma usually appears as a
solitary homogeneous echogenic lesion
 For preoperative assessment, CT and CMR
are more desirable for evaluating the
“resectability” of the lesion which can delineate
heterogeneity of the mural mass, the degree
of calcification, and its three-dimensional
characteristics.

 The average size of the tumor is 5 cm
 Generally single lesions, and they are
commonly found in the ventricular septum or
the left ventricular free wall, with occurrence in
the right ventricle or the atria in less than 10%
of cases.

 The differential feature favoring fibroma over
rhabdomyoma is calcification, which occurs in
fibromas but not in rhabdomyomas
 Complete surgical resection in symptomatic
cases is recommended.
 The postoperative prognosis is typically good,
 For large, unresectable tumors, cardiac
transplantation may be considered if
symptoms such as arrhythmias persist.

PRIMARY VASCULAR
TUMORS
 HEMANGIOMA
 LYMPHANGIOMA
 HEMOENDOTHELIOMA

 Extremely rare
 Benign with tendency to recur
 No age gender prediliction
 Ususally asymptomatic but can present with
palpitations, arrhythmias, heart failure,
pericardial effusion, ventricular outflow tract
obstruction, pseudoangina, cerebral
embolism, and, in more extreme cases,
sudden death.

 Kasabach-Merritt syndrome: Thrombosis,
consumative coagulopathy and
thrombocytopenia involving giant coronary
hemangioma.
 ECHO is a sensitive, noninvasive method for
detection of the tumor, with cardiac
hemangioma appearing typically as a
hyperechoic lesion.
 TUMOR BLUSH : Typically seen on
coronary angiogram.

 No layer specific.
 Ventricles>Atrial
 Treatment is surgical resection.

MALIGNANT TUMOURS
 1/4TH
of PCT are malignant, and most common
of them are sarcomas followed by lymphomas.
 Mets are 20-40 times more common than PCT
and mostly come from lung and breast cancer.
 Hodgkin disease, non-Hodgkin lymphomas,
malignant melanoma,numerous primary
gastrointestinal malignant neoplasms, and
various types of sarcomas arising from
extracardiac locations can also secondarily
involve the heart.

The more common malignant PCT are
1.Angiosarcomas
2.Leiomyosarcomas
3.Rhabdomyosarcomas
4.Malignant fibrous histiocytomas
5.Undifferentiated sarcomas
6.Fibrosarcomas
7.Malignant lymphomas

 Sarcomas showing high mitotic activity (>5
mitotic figures/10 high-power fields), extensive
tumor necrosis, and poor cellular
differentiation have a worse prognosis than
sarcomas without these features.
 The presence of metastases also confers a
poorer prognosis.

 On CT or CMR : large, heterogeneous, broad-
based masses that frequently occupy most of
the affected cardiac chambers.
 CMR is usually the method of choice for
imaging of sarcomas.
 The tumors may also show evidence of
extension into other cardiac chambers and the
pericardium, and there may also be
associated pericardial effusions and hilar
lymphadenopathy.

ANGIOSARCOMA
 Most common primary cardiac sarcoma in
adults,
 In adults 30 to 50 years old
 A slight male predilection exists
 occur sporadically.
 typically present with advanced disease, with
66% to 89% of patients already demonstrating
evidence of metastatic disease at initial
presentation.

 Initial findings may include dyspnea, chest
pain, constitutional symptoms, arrhythmias,
and evidence of heart failure.
 pericardial effusion and cardiac tamponade
may also be the presentation.
 Hemorrhagic pericardial tamponade usually
indicates tumor infiltration through the
myocardium.

 Less commonly stroke-like neurologic
symptoms secondary to cerebral metastases,
are the initial presentation in patients with
cardiac angiosarcoma.
 ECG reveals nonspecific ST changes,
arrhythmias, and AV block.
 CXR may show nonspecific changes like
cardiomegaly, widened mediastinum, hilar
lymphadenopathy, and pleural effusion.
 TEE is the initial imaging modality of choice

 CT and CMR for a better characterization of
the tumor growth and involvement.
 appear as low-attenuation, invasive, irregular
nodular masses showing heterogeneous
enhancement with the administration of
contrast media on CT and heterogeneous
mass on CMR with frequent pericardial
involvement and hemorrhagic pericardial
effusion
 90% in RA and involvement of structures such
as the tricuspid valve, pulmonary valve, and

 Immunohistochemical analysis is done for final
diagnosis
 aggressive neoplasms with a poor prognosis
and mean survival of 9 to 10 months.

 Common sites of metastases : lung, liver,
brain, and bone
 A multidisciplinary approach to the treatment
of cardiac angiosarcoma is advocated
including a combination of surgery, irradiation,
adjuvant or neoadjuvant chemotherapy, and
immunotherapy with interleukin-12 (IL-12).

 Chemotherapy : doxorubicin, an anthracycline.
 Surgery for complete tumor resection.
 Neoadjuvant chemotherapy may be
administered to reduce the tumor mass and to
facilitate surgical excision. This includes
combined doxorubicin and ifosfamide and a
combination of docetaxel and gemcitabine.
 The use of heart transplantation remains
controversial in this setting.

 For advanced-stage unresectable disease,
palliative treatment including the use of
metallic stents for superior vena cava
syndrome and for severe right ventricular
outflow tract obstruction may help improve the
patient’s short-term quality of life.

USE OF MDM2 drugs
 Novel MDM2 drugs which genetically reduce
the expresion of p53 in the neoplastic cells are
being probed to reduce the neo-angiogenesis
in tumour progression.

RHABDOMYOSARCOMA
 most common primary sarcoma of the heart in
children.
 The average age at disease presentation is in
the second decade of life with slight male
preponderance.


 Heart failure, arrhythmias, cardiac murmurs,
and constitutional symptoms are common
manifestations of the disease.
 Occasional cases are also associated with
hypereosinophilia, hypertrophic
osteoarthropathy, and polyarthritis.
 Nonspecific ECG and CXR findings are often
present.

 ECHO followed by CT/CMR to delineate the
extent of tumour.
 In contrast to angiosarcomas, cardiac
rhabdomyosarcomas show no predilection for
a specific cavity, and multiple lesions are
frequently present
 aggressive neoplasms with a tendency to
metastasize most commonly to the lung and
lymph nodes, with survival <1 year.

 The primary aim of treatment is complete
surgical resection
 Poor response to radiotherapy and
chemotherapy.
 In selected cases, heart transplantation may
be considered if no obvious distant
metastases are present

LEIOMYOSARCOMA
 Leiomyosarcomas are malignant
mesenchymal tumors with histologic and
immunophenotypic evidence of smooth
muscle differentiation.
 Mean age : fourth decade, and there is no
apparent sex predilection.
 The common clinical presentations include
dyspnea, pericardial effusions, chest pain,
atrial arrhythmias, and heart failure.
 70-80% arise from the left atrium, and they
tend to extend into the pulmonary trunk.

 Multiple origin in 30% of patients.
 ECHO followed by CT/CMR for diagnosis.
 rapidly growing tumors with a high rate of local
recurrence and distant metastases
 prognosis is poor, with a mean survival of 6
months.
 Effective treatment of this progressively lethal
disease is unknown.

LYMPHOMAS
 1.3% to 2% of all primary cardiac tumors.
 In both immunocompetent and
immunocompromised more in latter
 Commonly associated with HIV, Post
transplant lymphoproliferative disorder and
EBV infections.
 The average age : 62 to 67 with male
predominance.

 The common clinical presentations include
chest pain, heart failure, pericardial effusion,
palpitation, and arrhythmias.
 Less common presentations of primary
cardiac lymphomas are cardiac tamponade,
pulmonary and systemic embolism.
 ECG findings are nonspecific
 ECHO followed by CT/CMR.

 Right side involvement more common.
 Size range from 3 to 12 cm in size with a
mean of 7 cm.
 Treatment : Early implementation of
anthracycline-based chemotherapy with or
without radiation therapy has become the
mainstay for treatment of primary cardiac
lymphomas.
 Radical surgical excision is generally
discouraged.

 Rituximab, a monoclonal antibody targeted
against CD20, in combination with
conventional chemotherapy,
 60% of patients die of the disease within 2
months after the initial diagnosis

SECONDARY CARDIAC
TUMOURS
 INCIDENCE-1.7% to 14% in cancer patients
and from 0.7% to 3.5% in the general
population.
 Cardiac metastases can occur either by direct
extension, via the bloodstream or lymphatics,
or by intracavitary diffusion through the inferior
vena cava (IVC).
 Pericardial metastasis (69%) is the most
common, followed by epicardial (34%),
myocardial (32%), and endocardial
metastases (5%).

 The pericardium is most often involved
because of direct invasion by thoracic cancer,
including breast and lung cancer.
 Abdominal and pelvic tumors may reach the
right atrium through the IVC. The most
common tumor exhibiting this tendency is
renal cell carcinoma.

 In men: lung cancer followed by esophageal
cancer and lymphoma
 In women: lung cancer followed by lymphoma
and breast cancer.

Clinical Manifestations of SCT
 peripheral edema are common clinical
findings.
 Heart failure, cardiac arrhythmias, heart
blocks, acute myocardial infarction, myocardial
rupture, systemic embolization, and superior
vena cava (SVC) syndrome
 ECG : ST-T wave changes (mimicking
myocardial ischemia or injury), new atrial
fibrillation or flutter, and low voltages, with
electrical alternans indicating a significant
pericardial effusion

COMPLICATIONS OF
NEOPLASIA
 Pericardial Effusion
Causes: malignant effusion,
radiation- or drug-induced pericarditis,
idiopathic pericarditis
infectious causes (TB Bacterial)
iatrogenic secondary to procedures.
Drug-induced pericarditis is typically seen after
high-dose anthracycline or cyclophosphamide
therapy.

CARDIAC TAMPONADE
Usual presentation of pericardial effusion.
Immediate treatment is directed towards fluid
drainage.
Cytologic examination 85% of malignancy
patients.
Risk for recurrence is reduced by extended
catheter drainage (3 ± 2 days; 11.5%
recurrence) as opposed to simple
pericardiocentesis.

Some have used intrapericardial instillation of
chemotherapeutic agents or sclerosing agents,
but it is not clear which approach is better.
Occasionally, percutaneous balloon
pericardiotomy or pericardiectomy may be
required, but patients with malignant effusions
have such a poor prognosis
Therapy is directed at the underlying tumor

CARCINOID
 Tumors also can affect the cardiac structures
indirectly, as is seen in carcinoid heart disease.
 Metastatic carcinoid tissue in the liver
produces biologically active substances,
including serotonin, which cause abnormalities
of the right-sided cardiac valves and
endocardium.

 Typical changes include thickening, retraction,
and increased rigidity of the tricuspid and
pulmonic valve leaflets, resulting in valvular
regurgitation or, less often, valvular stenosis.
 Left-sided valvular involvement is rarely seen,
possibly because of a lower concentration of
the active molecules after passage through
the lungs.

 While metastatic carcinoid disease is rare, the
echocardiographic findings are
pathognomonic and may lead to the diagnosis
in a patient in whom it was not considered
previously.

Cardiac tumours

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