1. Primary Cardiac Tumors
Andrew Ferguson
UIC
January 3, 2008

2. Agenda Primary Tumors Treatment/Prognosis 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

3. 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%)

4. Clinical Presentations
Systemic
Symptoms
Biomarker abnormalities
Embolic Phenomena
Cardiac Manifestations
Mechanical Issues
Conduction Disturbances

5. Systemic Findings Non-specific signs/symptoms: Laboratory findings:
Fever, cachexia, malaise, arthralgias, Raynaud’s, rash, clubbing.
Constitutional symptoms
Laboratory findings:
Hypergammaglobulinemia, elevated ESR, thrombocytosis, thrombocytopenia, polycythemia, leukocytosis, and anemia.

6. 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

7. 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)

8. Cardiac Manifestations
Obstruction of circulation
Interference with valve function
Direct Invasion
Decreased myocardial contractility
Conduction disturbance (block and arrhythmias)
Tamponade

9. 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

10. Diagnostic Evaluation
Purpose is to guide therapy:
Location
Size
Malignancy
Physical Exam
Echocardiography
Roentgenography
Cardiac Catheterization CT
MRI

11. Physical Exam Again, dependent on location LA tumors RA 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.
Differ from PS d/t rapidly progressive sxs, no poststenotic dilatation and no ejection click.

12. In the old days… Roetgenograms Cardiac Catheterization
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

13. Examples

14. 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

15. 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

16. 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

17. 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

18. 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

19. TTE Example

20. Benign Tumors

21. 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.

22. Cardiac Myxoma Classic Triad: 20% Asymptomatic
Obstructive cardiac symptoms
Embolic phenomena
Constitutional symptoms
20% Asymptomatic

25. CT Left Atrial Myxoma

26. Can you find it?
Uncommon location - ~10%

28. Atrial Myxoma Histo
Mucopolysaccharid matrix and vascular channels with ‘myxoma’ cells
Microscopically, myxomas have an abundant mucopolysaccharide matrix and vascular channels with varying numbers of distinctive stellate or plump "myxoma" cells (left slide). Tumor-associated thrombus, degeneration, hemorrhage, calcification, inflammatory infiltrate (basophils) or superinfection may lead to an incorrect diagnosis of mural thrombi or endocardial vegetations (right slide). Similar cardiac presentation of sarcomas have been rarely described and may present initial diagnostic confusion.

29. Treatment Surgical resection is required: Operative mortality <5%
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%)

30. 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

31. 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

32. 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

33. 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

34. Papillary fibroelastoma

35. 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

36. 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)

37. 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

38. Treatment
Require resection due to the symptoms they cause and their progressive growth.

39. 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

40. 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

41. 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

42. Treatment Symptomatic tumors are resected
Very large tumors may require resection

43. 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

44. Angiomas Extremely rare Benign proliferations of endothelial cells
Generally found incidentally, but can cause symptoms commensurate with their location
Surgical resection if symptomatic

45. 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

46. 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

47. Malignant Tumors

48. 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.

49. Angiosarcoma Composed of malignant cells that form vascular channels
Predominantly in the right atrium

50. Rhabdomyosarcoma Constitute 20% of all cardiac sarcomas
Mostly adults, also described in children
Multiple sites are common, no site predilection

51. Fibrosarcoma Composed of spindle cells
White fleshy tumors which infiltrate the myocardium
May have extensive areas of necrosis and hemorrhage

52. Leiomyosarcomas Spindle-celled, high-grade tumors
Arise in the left atrium
High rate of local recurrence and systemic spread

53. 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

54. Primary Cardiac Lymphoma
Very rare
Typically non-Hodgkin type
Mostly occur in the immunocompromised
Presentation:
Progressive heart failure
Chest pain
Tamponade
SVC syndrome

55. Characteristics Can be multiple, firm white nodules
Can be fish-flesh homogenous appearance
More than one cardiac chamber
Pericardiac effusion is common

56. Histopathology Mostly B-cell tumors Histologic types:
Well-differentiated B-cell
Follicular
Diffuse large cell
Undifferentiated Burkitt-like
Lymphoma cells surrounding and infiltrating myocytes

57. Treatment and Prognosis
Small series has demonstrated a 38% complete response with systemic therapy, though question its duration
Otherwise poor outcome is expected

58. Can’t Resist
THANK YOU