1. CARDIAC TUMOURS
HA MWAKYOMA, MD

2. Agenda Clinical Manifestations Diagnostic Evaluation Primary Tumors
Systemic Findings
Embolic Phenomena
Cardiac Manifestations
Diagnostic Evaluation
Physical Examination
Echocardiography
Roentgenography
Cardiac Catheterization
MRI
Primary Tumors
Benign
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
Pulmonary Embolism. Can lead to Pulmonary arterial/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.
RA tumors
Right-sided congestion, diastolic murmur.
RV tumors
Right-sided CHF, SEM, presystolic mm and diastolic rumble.
LV tumors
SEM, positional changes of murmur and BP, other findings similar to AS, sub-aortic stenosis, HOCM.
Differ from PS d/t rapidly progressive sxs, no poststenotic dilatation and no ejection click.

12. 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

13. BENIGN TUMOURS
Relative incidence of benign tumours of the heart in % group
Benign tumours Adult Children Infant
Myxoma % % %
Papillary fibroelastoma 16% % %
Lipoma % % %
Rhabdomyoma % % %
Fibroma % % %
Teratoma % % %
Hemangioma % % %
Others % % %

14. 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.

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

19. ATRIAL MYXOMA

20. shows different aspects and colours, from the red-wine, and haemorrhagic aspect, until pearly-white colour.

21. The left atrium has been opened to reveal the most common primary cardiac neoplasm--an atrial myxoma.

22. LA myxoma showing myxoid stroma and polygonal cells

23. This high power microscopic appearance of cardiac myxoma shows minimal cellularity. Only scattered spindle cells with scant pink cytoplasm are present in a loose myxoid stroma.

24. Atrial myxoma. Shows the hystology of the components of myxoma: free-floating spindle and stellate cells -sometimes syncytial-; myxoid ground substance, and a surface

25. Shows a very characteristic appearance of myxoma cells arranged in a cuff around a small central space

26. 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

27. 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

28. Clinical Features cont.
Symptoms usually due to embolization of either tumor or thrombus.
Most commonly CVA/TIA
Also angina, MI, CHF, syncope, or systemic/pulmonic embolic events
Up to 30% are asymptomatic and diagnosed incidentally at time of an echo, cardiac surgery or autopsy

29. 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

30. Papillary fibroelastoma

31. Papillary fibroelastoma

32. 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)

33. 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

34. CARDIAC LIPOMA

35. MICRO: LIPOMA

36. Lipomatous septal hypertrophy
Not a true tumor
Exaggerated growth of normal fat
Up to 2cm in thickness
Seen in elderly and obese people
Only consider surgery if symptomatic:
Atrial arrhythmias
Heart block

37. 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

38. This two year old child died suddenly
This two year old child died suddenly. At autopsy, a large firm, white tumor mass was found filling much of the left ventricle. This is a cardiac rhabdomyoma. Such primary tumors of the heart are rare.

39. 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

40. 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

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

42. Paraganglioma Extremely rare
Arise from chromaffin cells, mainly in atria
Majority produce catecholamines
Positive biomarkers similar to pheochromocytomas
20% of patients also have extracardiac tumor
Surgical excision is definitive treatment

43. 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

44. MALIGNANT TUMOURS

45. 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.

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

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

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

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

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

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

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