Download Cardiac tumors

Dr Shreetal Rajan Nair
Senior Resident, Department of Cardiology,
Calicut Medical College
History
 1559 : Columbus , a noted pathologist – first to report
 1934 : first report of a primary sarcoma of the heart
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diagnosed in a living patient by Barnes
1942 : surgical resection of intrapericardial teratoma by
Beck
1951 : The first successful excision of a cardiac tumor
performed by Maurer
1952 : Goldberg diegnosed intracavitary myxoma by
angiocardiography
1968 : Shattenberg used echo to diagnose cardiac tumors
Classification
Primary benign (75%)
malignant (25%)
secondary
Breast
Myxoma – 50%
Rhabdomyoma – 20%
Fibroma
Papillary fibroelastoma
Lipoma
Angiosarcoma
Rhabdomyosarcoma
Leiomyosarcoma
Lymphomas
lungs
Incidence
 All age groups - 0.002% to 0.03%
 Pediatric
 Adults
- 0.027% to 0.08%
- upto 0.02%
 By comparison, metastatic involvement is 20 times
more common
Incidence of benign tumors
Shapiro LM. Cardiac tumours: diagnosis and management. Heart 2001; 85: 218 - 22.
 LITERATURE
Tumors of the heart Constitutional or systemic
2. Embolic
3. Cardiac
4. Phenomena secondary to metastatic diseases.
1.
Specific signs and symptoms generally are determined
by the location of the tumor, size, friability ,
mobility in the heart and depends least on the
histopathology
Tumors of the heartclinical presentations
 Primary tumors most
commonly involve the
endocardium, followed
by myocardium and then
epicardium
 Endocardium-
obstruction to flow of
blood through heart
 Myocardium - rhythm
abnormalities
 Metastatic involvement
of heart is reverse that of
primary tumors
 Epicardium –
pericardial effusion,
cardiac tamponade
Approach to a cardiac tumor
Differential diagnosis
 PUO
 CONDUCTION
 IE/CONNECTIVE TISSUE
ABNORMALITIES
 EMBOLISM
 SYNCOPE
 PERICARDIAL EFFUSION
DISEASES
 VALVULAR LESIONS
 CCF
Diagnostic evaluation
 Confirm the diagnosis
 locate the lesion within the heart
 Whether benign or malignant
Echocardiography
 Simple, noninvasive technique for initial evaluation.
 Images myocardium and the cardiac chambers
Identify the presence of a mass and its mobility.
 Information about any obstruction to the circulation
& whether the tumor could be a source of emboli
Echocardiography - TEE vs TTE
Transthoracic echocardiography – the initial diagnostic tool
Transesophageal echocardiography (TEE)more informative.
This 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
CARDIAC MRI VS CT
 Both provide noninvasive, high resolution images of
the heart, but MRI generally is preferred.
 cMRI -detailed anatomic images
T1- and T2-weighted sequences help to identify
the chemical microenvironment within a tumor
 CT -useful when calcification is present and MRI is not
available or is contraindicated.
CORONARY ANGIOGRAPHY
 Mapping the blood supply of tumors arising from the
epicardial surfaces
 vital to the success of excising such tumors.
 Significant involvement of coronary arteries -
resection and grafting of such arteries
Role of Transvenous biopsy
 Limited data are available on the risks and benefits
 considered reasonable if potential benefits outweigh
risks and there is diagnostic dilemma
Benign tumors
 Around 75 percent of primary cardiac tumors are
benign
 In adults, most common- myxomas (50%)
 Other common benign lesions-
papillary fibroelastomas and lipomas.
 In children, the most common-
rhabdomyomas and fibromas
Myxomas
 Most common primary cardiac neoplasm.
 Histology- scattered cells within a mucopolysaccharide
stroma which originate from multipotent mesenchymal
cells capable of neural and endothelial differentiation .
Hallmark histological feature is myxoma (lepidic) cell
 Produce VEGF-induction of angiogenesis and the early
stages of tumor growth
&
IL 6 -responsible for the constitutional features.
Myxoma ( lepidic ) cells
Myxomas
 Typically pedunculated and gelatinous in consistency;
smooth, villous, or friable.
 Calcification , necrosis and cystic changes seen
 Mostly (90%) solitary
 About 35 % of are friable or villous, these tend to
present with emboli.
 Larger tumors -smooth surface and associated with
cardiovascular symptoms.
Myxomas
 The cardiovascular manifestations depend upon the
anatomic location of the tumor.
 Mostly seen in females
 40-60 years of age
 80% -originate in the left atrium,
most of the remainder is found in the right atrium
 constitutional symptoms (eg, weight loss, fever) and
laboratory abnormalities
Syndrome Myxoma
Characteristics
Syndromic associations
 Younger than 40 years
 LAMB (lentigines, atrial
 Biatrial ,ventricular , valves
 all first degree relatives
should be screened
 Recurrent – 30%
 No gender predilection
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)
Myxoma
Carney syndrome
Histological features
Mutations in :
 PAS
 PRKAR1A (protein kinase A
 Vimentin
regulatory subunit 1A )
 S100 and NSE
 MYH8 - myosin isoform
- POSITIVITY
Treatment
 Prompt resection-due to the risk of embolization or
cardiovascular complications, including sudden death
 Result of surgical resection is good ( <5% mortality)
 Cardiac autotransplantation
Papillary fibroelastomas
 Second most common
primary cardiac tumor in
adults
 Their appearance is
compared to pom-pom
or sea anemones
Clinical features
 Symptoms -caused by embolization of the
tumor/thrombus.
 most common clinical presentation- stroke /TIA.
 Commonly attached to the aortic valve
 30%- incidentally diagnosed by echocardiography, at
cardiac surgery, or at autopsy
TREATMENT
 SURGERY –
1. patients who have had embolic events or
complications directly related to tumor mobility
(eg, coronary ostial occlusion)
2.Those with highly mobile or large (≥1 cm) tumors
 Recurrence not reported
Lipomas
 Lipomas &fibrolipomas - characterized by a
predominance of benign fatty cells.
 About half of these tumors occur in the subendocardial
region.Others in the myocardial and subepicardial regions
 They may also occur on valves
Lipoma
Lipomas
 Symptoms- generally related to local tissue encroachment
(arrhythmias, conduction block, sudden death)
valvular tumors – insufficiency & symptoms of heart
failure
 Diagnosis - echocardiography and the distinctive
fat pattern on MRI.
 Require resection.
Lipomatous hypertrophy
of the interatrial septum
 Exaggerated growth of normal fat existing within the
septum and is not a true tumor.
 Developmental disorder caused by expansion of
adipose tissue trapped in the interatrial septum during
embryogenesis
 The septal hypertrophy may be as much as 2 cm in
thickness and is seen primarily in older patients and in
those who are obese
Lipomatous hypertrophy of
the interatrial septum
 Associated with the presence of CAD in proportion to
the degree of atrial septal thickness
 Indistinguishable from lipoma except that the former
occurs in the atrial septum with a typical distribution
(generally sparing the fossa ovalis).
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 In the absence of symptoms of atrial arrhythmias,
heart block or rare vena caval obstruction, they do not
require resection
Pericardial lipomas
 Incidental finding and clinically insignificant.
 Rarely assumes gigantic proportions and its
appearance on a chest radiograph may be mistaken for
a huge pericardial effusion or massive cardiomegaly
 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.
Rhabdomyomas
 Develop almost exclusively in children, mostly <1 year of
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age
80 to 90% are associated with tuberous sclerosis
Usually found in the ventricular walls/AV valves.
Most regress spontaneously
Resection is usually not required unless symptomatic
Symptoms –due to obstruction of blood flow through
the heart or consist of rhythm disturbances
Present with features of preexcitation on the ECG
Rhabdomyositis
 A rare form of cardiomyopathy in infants
 Tumor nodules are not grossly apparent
 Microscopically, the cardiac muscle fibers and
conduction system are diffusely involved with
rhabdomyomatous histologic changes
 Recurrent atrial tachycardia and sudden death from
intractable ventricular tachycardia
Why rhabdomyomas regress?
 High expression of ubiquitin which starts expressing
from 32 week onwards
 Absent mitotic activity
 Ubiquitin is responsible for the degradation of
rhabdomyoma cells and hence tumor regression
 Characteristic spider cells are formed in the process.
Spider cells in rhabdomyoma
Fibromas
 Second most common pediatric cardiac tumor and can
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also occur in adults
Histologically similar to fibromas arising elsewhere in
the body.
Usually arise in the ventricular muscle and may
become quite large.
Do not regress spontaneously.
Arise approximately 5 times more frequently in the LV
than RV
Fibromas
 Most common symptom, due to obstruction of blood
flow or interference with valvular function.
 Myocardial dysfunction and conduction disturbances
 Echocardiography, supplemented with CT/MRI
confirms the diagnosis.
 Symptomatic tumors should be resected. Complete
resection of very large tumors may require cardiac
transplantation.
Fibromas
Teratomas
 Arise within the pericardium, but do not originate
from cardiac structures
 Although generally benign,can have serious
mechanical consequences by causing tamponade or
through direct pressure on the heart.
 Risk of death in-utero or immediately after birth
Teratomas
 Treatment therefore requires either fetal tumor
excision, or caesarean section and immediate
operation on the newborn
 Have a single blood supply and are not invasive,
properly timed tumor surgery is straightforward and
successful
Purkinje cell
tumors/hamartomas
 Consist of small, flat sheets of cells most frequently
located in the left ventricle, and on the endocardial
and epicardial surfaces
 Undetectable by echocardiographic or radiologic
techniques.
 Tumors of young children and present with incessant
ventricular tachycardia
 ECGs 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.
Paragangliomas
 Neuroendocrine tumors that can be hormonally active
or inactive.
 In tumors not producing catecholamines, symptoms
are due to cardiac compression or tamponade.
 In contrast, cardiac paragangliomas that are
hormonally active primarily produce norepinephrine
and may cause systemic symptoms (eg, headache,
sweating, tachycardia, hypertension)
 Hormonally inactive tumors are more frequent in the
pericardium
Paragangliomas
 May be localized with echocardiography.
 Extremely vascular
 Coronary angiography -to plan the operative resection
Paragangliomas
 Paragangliomas (benign/malignant) within the
pericardium parasitize the cardiac blood supply and
hence are very difficult to excise
 All intrapericardial paragangliomas require resection
 Cardiopulmonary bypass and even circulatory arrest
may be required due to the high degree of vascularity,
or to moderate the extreme hypertension possible
from tumor manipulation or hormonally active tumors
 If complete resection is not possible, cardiac
transplantation may be required
PRIMARY MALIGNANT TUMORS
 Malignant tumors constitute approx 15% of primary
cardiac tumors
 Sarcomas are the most common
 Virtually all types of sarcomas have been reported in
the heart
 Prognosis depends on Mitotic activity, extent of tumor
necrosis and cellular differentiation.
Malignant cardiac tumors
 Clinical presentation is largely determined by the
location of the tumor, rather than its histopathology.
 The diagnostic approach relies upon
echocardiography, MRI, and CT to define the presence
of a tumor and its anatomic relationship to normal
structures
 Increased mitoses
Angiosarcomas
 Composed of malignant cells that form vascular
channels.
 Pathology may overlap with Kaposi's sarcoma, which
can also involve the myocardium
 Arise predominantly in the right atrium
Rhabdomyosarcomas
 Constitute as many as 20% of all primary cardiac
sarcomas
 Most commonly found in adults, although they have
also been described in children.
 Multiple sites of myocardial involvement are common,
No predominant localization within any area of the
heart.
Fibrosarcomas
 Fibrosarcomas and malignant fibrous histiocytomas
are white fleshy ("fish flesh") tumors that are
composed of spindle cells, and may have extensive
areas of necrosis and hemorrhage
 These tumors tend to extensively infiltrate the
myocardium
Leimyosarcomas
 Spindle-celled, high-grade tumors that arise more
frequently in the left atrium
 These sarcomas have both a high rate of local
recurrence and systemic spread.
Mesothelioma
 Although most arise in the pleura, these can also arise
from the pericardium, where they are usually
malignant
 Those arising in the pericardium produce tamponade
and constriction
 Seen with echocardiography, CT scan, MRI and
sometimes by chest x-ray
 Pericardiocentesis may yield a cytologic diagnosis
Mesothelioma
 More rarely they may arise as benign tumors of the AV
node where they may produce 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
 The addition of radiation and/or chemotherapy has
been attempted but has not been shown to be of value.
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.
Treatment and prognosis
 The median survival is typically 6 to 12 months
although long-term survival has been reported with
complete resection.
 Low-grade sarcomas may have a better prognosis
 Adjuvant chemotherapy and radiation therapy have
been used infrequently either after resection to
improve results or in metastatic involvement.
 Rhabdomyosarcomas may have a better outcome with
chemotherapy
Treatment and prognosis
 The poor results with surgical resection have led to
occasional attempts to treat patients with cardiac
transplantation, if extracardiac disease is not present
 cardiac autotransplantation is another novel
approach.
Here, the heart is excised, tumor 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
Lymphomas and other 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
 However, 38 percent of cases achieved a complete
response with systemic therapy.
 Other tumors may also arise in the heart, including
paragangliomas and extramedullary plasmacytomas
SECONDARY CARDIAC TUMORS
 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
 Malignant melanomas are particularly likely to metastasize
to the heart. 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. There is also
a high prevalence of secondary cardiac involvement with
leukemia and lymphoma
Secondary cardiac tumors
 Cardiac or pericardial metastasis should be considered
whenever a patient with known malignancy develops
cardiovascular symptoms, particularly if this occurs in
conjunction with cardiomegaly, a new or changing
heart murmur, electrocardiographic conduction delay,
or arrhythmia.
 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
Secondary cardiac tumors
 The specific symptoms reflect the site of cardiac
involvement.
 The diagnostic evaluation is the same as that for
primary cardiac tumors and relies upon
echocardiography, MRI, and CT.
 Resection of cardiac metastases has been used to
provide symptom palliation and prolong life
Summary
 Cardiac tumours are
rare.
 A cardiac mass most
likely represents a
thrombus or vegetation.
 Most cardiac tumours
are secondary, ie,
originate from a primary
tumour elsewhere.
 Most primary cardiac
tumours are benign
 In addition to history, a
non- invasive diagnosis
of cardiac tumours can
usually be made by the
following:
-Histology based
likelihood.
-Tumour location.
-Age at presentation.
-Imaging characteristics
Benign tumors of the heart
Malignant tumors of the heart
Imaging features of cardiac
features – in a nut shell
Summary
 The most common benign
cardiac tumour is a
myxoma.
 Myxomas are generally
found in the left atrium
arising from a stalk attached
to the fossa ovalis
membrane.
 The most common primary
cardiac tumours in children
are rhabdomyomas and
fibromas, both of which are
benign.
 Rhabdomyomas usually
decrease in size with age.
 The most common cardiac
tumour involving valves is
a papillary fibroelastoma.
 The most common
primary malignant tumour
is a sarcoma.
 Angiosarcomas are the
most common histologic
subtype of sarcoma and are
usually found in the right
atrium