Primary Cardiac Tumors Andrew Ferguson UIC January 3, 2008

Primary Cardiac Tumors

Andrew Ferguson

UIC

January 3, 2008

Agenda

• Clinical Manifestations– Systemic Findings– Embolic Phenomena– Cardiac Manifestations

• Diagnostic Evaluation– Physical Examination– Echocardiography– Roentgenography– Cardiac Catheterization– MRI– PET

• Primary Tumors– Benign– Benign or Malignant– Malignant

• Treatment/Prognosis– 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

– 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

• 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:– 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

• 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 aTIFF (Uncompressed) decompressor

are needed to see this picture.

Can you find it?

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are needed to see this picture.

Uncommon location - ~10%

Atrial Myxoma Histo• Mucopolysaccharid matrix and vascular channels with ‘myxoma’ cells

QuickTime™ and aTIFF (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

• 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

• 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

• 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

• 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

• 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

• 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, high-grade 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

• 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 B-cell

– 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

THANK YOU