Download Primary Cardiac Tumors

Primary Cardiac Tumors
Andrew Ferguson
UIC
January 3, 2008
Agenda
• Clinical Manifestations
– Systemic Findings
– Embolic Phenomena
– Cardiac Manifestations
• Diagnostic Evaluation
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Physical Examination
Echocardiography
Roentgenography
Cardiac Catheterization
MRI
PET
• Primary Tumors
– Benign
– Benign or Malignant
– Malignant
• Treatment/Prognosis
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Observation
Excision
Chemo/Radiation Tx
Recurrence
Cardiac Tumor Occurrence
• 0.002 to 0.3% incidence in autopsy series
• Metastatic involvement of the heart is 20
times more common than primary lesions
• Majority are benign (~75%)
Clinical Presentations
• Systemic
– Symptoms
– Biomarker abnormalities
• Embolic Phenomena
• Cardiac Manifestations
– Mechanical Issues
– Conduction Disturbances
Systemic Findings
• Non-specific signs/symptoms:
– Fever, cachexia, malaise, arthralgias,
Raynaud’s, rash, clubbing.
– Constitutional symptoms
• Laboratory findings:
– Hypergammaglobulinemia, elevated ESR,
thrombocytosis, thrombocytopenia,
polycythemia, leukocytosis, and anemia.
Etiology of Systemic Findings?
• More often seen in myxomas
• Synthesis and secretion of IL-6
– Inflammatory cytokine which induces the
acute phase response
• Prior to advanced imaging, not unusual for
alternate diagnoses of collagen vascular
disease, infection or non-cardiac
malignancy to be made
Embolic Phenomena
• Tumor fragments or its adherent thrombus
• Manifestation depends upon tumor location
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PE. Can lead to PAH/cor pulmonale
Systemic infarct (MI, CVA, etc)
Vascular aneurysms and visceral hemorrhage
Multiple emboli can mimic systemic vasculitis or IE
• Metastasis?
• May help lead to diagnosis (I.e. skin biopsy)
Cardiac Manifestations
• Obstruction of circulation
• Interference with valve function
• Direct Invasion
– Decreased myocardial contractility
– Conduction disturbance (block and
arrhythmias)
– Tamponade
Obstruction of Circulation
• Symptoms correlate with location
– i.e. left atrial tumors often mimic symptoms of MV
disease by obstructing AV flow or causing MR
(prolapse)
• To differentiate from other cardiac diseases,
tumor symptoms tend to have the following:
– Sudden onset
– Intermittent occurence
– Positional dependence
Diagnostic Evaluation
• Purpose is to guide therapy:
– Location
– Size
– Malignancy
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Physical Exam
Echocardiography
Roentgenography
Cardiac Catheterization
CT
MRI
Physical Exam
• Again, dependent on location
• LA tumors
– Pulmonary congestion, S4, loud (widely split?) S1, diastolic
murmur, MR murmur, tumor plop.
• RA tumors
– Right-sided congestion, diastolic murmur, TR, tumor plop.
• RV tumors
– Right-sided CHF, SEM, presystolic mm and diastolic rumble, S3,
delayed P2. (differentiate from PS?)
• LV tumors
– SEM, positional changes of murmur and BP, other findings
similar to AS, sub-aortic stenosis, HOCM, etc.
In the old days…
• Roetgenograms
– Raised the suspicion, but rarely diagnostic
• Cardiac Catheterization
– Was relied upon to aid in diagnosis of cardiac tumor
when suspicion was high.
– Alternate imaging modalities have helped remove this
potentially risky method.
– Should still be used when:
• Non-invasive eval did not fully define location/attachment
• All cardiac chambers have not been visualized
• Co-existing cardiac conditions are suspected and delineation
with cath may change the surgical/treatment approach
Examples
Radionuclide Imaging
• Inferior to echo/CT/MRI
• Gated blood pool identifies atrial,
ventricular and intramural tumors
• Radionuclide ventriculography has
relatively poor resolution compared to
echo or contrast angio
Echocardiography
• 2-D echo has become initial study of
choice
• M-mode can still be helpful
• Doppler useful for hemodynamic eval
• TEE often indicated for full visualization
– Tumor contour, cysts, calcification, stalk
CT
• Most useful when MRI is not available
• Advantages:
– Tissue discrimination:
• Tumor extension, attachment
– Reconstruction in any plane
– Evaluation of involvement of pericardial and
extracardiac structures
MRI
• Preferred over CT when available
• In addition to that gained by CT, MRI:
– T1- and T2-weighted sequences reflect the
chemical microenvironment within a tumor
Malignant vs. Benign Tumors
• ~75% of cardiac tumors are benign.
• Malignant tumors more likely to have:
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distant metastases
local mediastinal invasion
evidence of rapid growth
hemorrhagic effusion
precordial pain
right-sided location
combined intramural and intracavitary location
extension into pulmonary veins
TTE Example
• Benign Tumors
Myxoma
• Most common primary cardiac tumor
– ~30-50% of all cardiac tumors in path series
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Mean age 56 in sporadic cases (age 3-83)
70% female
86% occur in left atrium
90% are solitary, intracavitary and located
in atria.
Cardiac Myxoma
• Classic Triad:
– Obstructive cardiac
symptoms
– Embolic phenomena
– Constitutional
symptoms
• 20% Asymptomatic
CT Left Atrial Myxoma
QuickTime™ and a
TIFF (Uncompressed) decompressor
are needed to see this picture.
Can you find it?
QuickTime™ and a
TIFF (Uncompressed) decompressor
are needed to see this picture.
Uncommon location - ~10%
Atrial Myxoma Histo
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Mucopolysaccharid matrix and vascular channels with ‘myxoma’ cells
QuickTime™ and a
TIFF (Uncompressed) decompressor
are needed to see this picture.
Treatment
• Surgical resection is required:
– High risk of embolization
– Other CV complications, like sudden death
• Operative mortality <5%
• Generally curative
• Cardiac transplant considered for multiple
recurrent myxomas (occuring in 5%)
Papillary Fibroelastoma
• 2nd most common primary cardiac tumor
of adults
• Some synonyms:
– Giant Lambl excrescence
– Papilloma of valves
– Myxofibroma
– Myxoma of valves
– Hyaline fibroma
– Fibroma of valves
Clinical Features
• >80% are found on heart valves:
– Mainly left-sided valves
– Account for ~75% of all valve tumors
• Men and women affect ~equally
• Mean age 60 years at diagnosis
• Multiple tumors in 9% of patients
Clinical Features cont.
• Symptoms usually due to embolization of
either tumor or thrombus.
– Most commonly CVA/TIA
– Also angina, MI, SCD, CHF, syncope, or
systemic/pulmonic embolic events
• Up to 30% are asymptomatic and
diagnosed incidentally at time of an echo,
cardiac surgery or autopsy
Histopathology
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Benign endocardial papillomas
Characteristic frondlike appearance
Mean tumor size 9mm (range 2-70mm)
Core of loose connective tissue:
– Rich in glycosaminoglycans, collagen and
elastic fibers
– Fine meshwork of smooth muscle cells
• This core is surrounded by endothelium
Papillary fibroelastoma
Treatment
• Surprisingly no evidence
• Surgery definitively indicated in patients:
– Who have had embolic events
– With highly mobile or large (>1cm) tumors
• Some suggest careful observation is
acceptable for asymptomatic patients
• Recurrence after surgery has not been
reported
Lipoma
• Rare (~3% of primary cardiac tumors)
• Predominantly adults
• Occur in the subendocardium,
myocardium and subepicardium
• Symptoms related to local tissue
encroachment (arrhythmia, heart block,
and sudden death)
Histopathology
• Circumscribed,
spherical or elliptical
mass
• Composed of mature
adipocytes
• Differ from lipomatous
hypertrophy of IAS:
– Usually encapsulated
– No brown fat cells
– No myocytes found
Treatment
• Require resection due to the symptoms
they cause and their progressive growth.
Lipomatous septal hypertrophy
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Not a true tumor
Exaggerated growth of normal fat in IAS
Up to 2cm in thickness
Seen in elderly and obese people
Only consider surger if symptomatic:
– Atrial arrhythmias
– Heart block
Rhabdomyomas
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Primarily pediatric
Usually age <1 year
Highly associated with tuberous sclerosis
Found on ventricular walls or AV valves
Infrequent symptoms of obstruction and
arrhythmias
• Usually regress spontaneously
Fibromas
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2nd most common pediatric tumor
Rarely also occurs in adults
Usually arise in ventricular muscle
Heart failure due to:
– Obstruction
– Interference with valvular function
– Myocardial dysfunction
Treatment
• Symptomatic tumors are resected
• Very large tumors may require resection
Teratoma
• Obviously a pediatric issue
• Arise within pericardium
• Benign in nature but cause drastic
complications via tamponade
• High risk of death in-utero or after birth
• Requires fetal excision or C-section and
immediate operation
Angiomas
• Extremely rare
• Benign proliferations of endothelial cells
• Generally found incidentally, but can
cause symptoms commensurate with their
location
• Surgical resection if symptomatic
Paraganglioma
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Extremely rare
Arise from chromaffin cells, mainly in atria
Majority produce catecholamines
Positive biomarkers similar to pheo
20% of patients also have extracardiac
tumor
• Surgical excision is definitive treatment
Mesothelioma
• Can be both benign and malignant
• More commonly arise in the pericardium, also
rarely arise in the AV node
• Pericardial mesothelioma:
– Likely malignant
– May produce tamponade
– Resection is treatment of choice, but remains poor
prognosis if malignant
• AV node mesothelioma:
– Benign
– May produce heart block and sudden death
• Malignant Tumors
Sarcomas
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Constitute most of all malignant tumors
Overall, 2nd most common cardiac tumor
Virtually all cell types have been reported
Clinical presentation depends on location,
rather than its histopathology.
Angiosarcoma
• Composed of
malignant cells that
form vascular
channels
• Predominantly in the
right atrium
Rhabdomyosarcoma
• Constitute 20% of all
cardiac sarcomas
• Mostly adults, also
described in children
• Multiple sites are
common, no site
predilection
Fibrosarcoma
• Composed of spindle
cells
• White fleshy tumors
which infiltrate the
myocardium
• May have extensive
areas of necrosis and
hemorrhage
Leiomyosarcomas
• Spindle-celled, highgrade tumors
• Arise in the left atrium
• High rate of local
recurrence and
systemic spread
Treatment and Prognosis
• Generally, sarcomas proliferate rapidly
• Death usually follows from:
– Widespread myocardial infiltration
– Obstruction of blood flow
– Distant metastases
• Treatment of choice is complete resection:
– Most develop recurrent disease
– Median survival typically 6-12 months
• Chemotherapy in small studies has not shown survival
advantage
• Path features predict better prognosis:
– Left atrial origin
– Low mitotic count
– Absence of necrosis and metastasis
Primary Cardiac Lymphoma
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Very rare
Typically non-Hodgkin type
Mostly occur in the immunocompromised
Presentation:
– Progressive heart failure
– Chest pain
– Tamponade
– SVC syndrome
Characteristics
• Can be multiple, firm
white nodules
• Can be fish-flesh
homogenous
appearance
• More than one
cardiac chamber
• Pericardiac effusion is
common
Histopathology
• Mostly B-cell tumors
• Histologic types:
– Well-differentiated Bcell
– Follicular
– Diffuse large cell
– Undifferentiated
Burkitt-like
Treatment and Prognosis
• Small series has demonstrated a 38%
complete response with systemic therapy,
though question its duration
• Otherwise poor outcome is expected
Can’t Resist
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