1. Dr.P.V.Nishanth

2. lntroduction
Represent an important group of cardiovascular
abnormalities because early and accurate diagnosis
may be curative and sometimes avoids unnecessary
surgery.
frequency of only 0.001-0.03%

3. • About 75 percent of all primary cardiac tumors
-benign neoplasms.
• Remaining 25 percent of primary cardiac tumors
-malignant neoplasms-metastatic MC

5. CLINICAL PRESENTATION
Four general categories—
Systemic manifestations
Embolic manifestations
Cardiac manifestations
Phenomena secondary to metastatic diseases.

6. Systemic Manifestations:
Produced by secretory products released by the
tumor and/or by tumor necrosis
Constitutional symptoms of fever, chills, fatigue,
malaise, and weight loss.
Leukocytosis, polycythemia/ anemia, throm-
bocytosis/ thrombocytopenia,
hypergammaglobulinemia, and increased ESR
Mimic those of several connective tissue diseases

7. Embolic Phenomena
Systemic emboli-typically by a left-sided tumor
Right-sided tumors - concurrent right-to-left
shunting through a patent foramen ovale.
Brain -MC site -involvement of both hemispheres and
multiple regions is seen more than 40 percent of the
time

8. • Cerebral embolism -transient ischemic attack or an
ischemic stroke, but lCH may occur as well.
• Mild vertigo to seizure and even a comatose state.
• Delayed aneurysm formation presumably at the site
of previous cerebral tumor emboli
• Tumor emboli to a coronary artery-myocardial
infarction
• Pulmonary embolization is typically caused by a right-
sided tumor

9. Benign- cardiac myxomas are most frequently
associated with embolic findings, especially when the
tumor possesses a villous surface
Other benign primary cardiac neoplasms that are
known to produce emboli –
Papillary fibroelastomas
hemangiomas/lymphangiomas
Malignant tumors can embolise

10. Cardiac Manifestations
Direct mechanical interference with
myocardial/valvular function
 lnterruption of coronary blood flow
 lnterference with electrophysiological conduction
Stimulation of pericardial fluid accumulation

11. Intramural or myocardial – asymptomatic, especially
if the sizes are small.
Located within or pressing on major cardiac
conduction pathways -complete heart block or
asystole in more severe cases
Compress the cardiac cavities
Obstruct the ventricular outflow tract
Contribute to insufficiency of the mitral valve

12. lntracavitary
• Left atrial-can interfere with the mitral valve
• Signs & symptoms-sudden in onset, intermittent, and
positional-
• Fatigue, dyspnea, orthopnea, PND, chest pain, pulmonary
edema, and peripheral edema
• S3 loud and widely split S1
• Holosystolic murmur most prominent at the apex with
radiation to the axilla,
• Diastolic murmur from turbulent blood flow through the
mitral orifice
• Tumor plop -the tumor striking the endocardial wall or
the abrupt halt of tumor excursions occurs later than an
opening snap but earlier than an S3.

13. Right atrium-right heart failure
Often delayed with an average time interval from
presentation to the correct diagnosis of 3 years
Rapidly progressive right heart failure and also new-
onset heart murmurs because of mechanical
interference with the tricuspid valve by the tumor
Elevated JVP with prominent a-wave and steep y
descent, and an early diastolic murmur or holosystolic
murmur
SVC syndrome - findings of peripheral edema, HSM,
ascites,

14. • Right ventricular tumors –
• Intracavitary component may obstruct the filling or
the outflow of the RV –RHF
• Auscultation may reveal a systolic ejection murmur at
the left sternal border, an S3, and a delayed P2.
• An elevated JVP and Kussmaul sign may also be
present.
• These findings may vary significantly depending on
the position of the patient

15. Left ventricular tumors
obstruct the LVOT and produce findings of LVF and
syncope, as well as atypical chest pain from
obstruction of a coronary artery either by direct
tumor involvement or tumor emboli.

16. Metastatic Diseases
Late stage with systemic dissemination present.
Present with symptoms secondary to the metastatic
disease
Common sites of metastases -lung, brain, and bone,
although metastases to other sites reported.

17. DIAGNOSTIC APPROACH

19. Aetiology
Can often be determined by considering four factors:
 (1) Histology based likelihood
 (2) Age of the patient at time of presentation
 (3) Tumor location
 (4) Non-invasive tissue characterisation

20. Histology based likelihood
• 90% of primary cardiac tumours excised surgically are
benign, with nearly 80% of these tumours
representing myxomas
• Papillary fibroelastomas (26%)
• Fibromas (6%)
• Lipomas (4%)
• Calcified amorphous tumours, haemangiomas,
teratomas, unilocular developmental cysts, and
rhabdomyomas

21. 10% of primary cardiac tumours excised at surgery are
malignant, of which more than 90% are sarcomas.
The remaining few are represented by lymphomas

22. Age at presentation

23. Location

26. Non-invasive tissue
characterisation
Echocardiography:
echogenicity of the mass and whether calcification is
present.
Vascularity can also be assessed using colour flow
Doppler and echocardiographic contrast.
Strain imaging also has potential in identifying the
non-contractile nature of masses such as fibromas

27. CT- regarding vascularity by contrast enhancement,
presence of calcification, and presence of fat.
MRI also provides information regarding vascularity,
presence of fat, degree of tissue oedema, and possibly
iron content

28. Primary benign cardiac
tumours
Cardiac myxoma
30-50% of all benign tumors¸25% of all tumors and
cysts of heart
Usually solitary and develop in the atria, 75%
originating in the left atrium and 15-20% in the right
atrium
Arise from or near the interatrial septum at the
border of the fossa ovalis membrane

29. Other sites-post.wall
 ant.wall
 appendage
RA myxoma-broader base larger area
Ventricular-free wall/IVS
 sessile/pedunculated
Multiple- 5%
 biatrial common.

30. Occur in all age groups, most frequently between the
third and sixth decades
Women are more commonly affected
Myxomas usually occur sporadically,

31. Familial- 7%-Carneys complex
Younger age,
Multiple
Atypical locations.
Increased risk of recurrence after resection
Other features- spotty skin pigmentation, endocrine
hyperactivity and other tumors such as testicular
Sertoli cell tumor, psammomatous melanotic
schwannoma, pituitary adenoma, and thyroid .
 mutations in PRKAR1A, a regulatory subunit 1A of
cAMP-dependent protein kinase A

32. Morphology
• Range in size from 1-15 cm in diameter.
• Polypoid with a smooth or gently lobulated surface,
often pedunculated, and characteristically arise from
a narrow stalk.
• Polypoid myxomas rarely embolise
• Villous or papillary myxomas have multiple fine
fragile villous extensions -greatest risk of
embolisation.

33. Clinical features
Determined by their location, size, and mobility.
One or more features of the triad of embolism,
intracardiac obstruction, and constitutional
symptoms.

34. Left atrial-
Dysnea /Fatigue
MR-pounding of valve by tumor
Postural variation
Symptoms out of sync with mitral valve disease
Unexplained emboli

35. RA-RHF
D/D of RHF
Ventricular-sessile
 Obs. Less common
 Emboli-left -64%
 RT-10%-PAH/ recurrent PE

36. X ray
Evidence of elevated left atrial pressure -53 percent of
patients with left atrial myxoma
 Cardiomegaly is seen in 37 and 50 percent of left and
right atrial myxomas, respectively.
Intracardiac tumor calcification is a rare finding in
left atrial myxomas but is found in 56 percent of
patients with right atrial myxoma

37. ECHO
Appear as homogenous echo masses
Echo free spaces-hemorrhage
Areas of calcfcaton

38. M-mode
LA-tumor fills LA in systole
 Diastole-prolapses into mitral valve orifice mass of
echoes appear behind AML
EF slope decreases

41. • CE CT - overall attenuation lower than that of
myocardium
• CMR shows heterogeneous signal intensity in 90
percent of cardiac myxomas,
• T1- images - isointense signal
• Cine gradient-echo CMR - superior to other imaging
modalities

42. Histologically-sparsely distributed uniform spindle-
and stellate-shaped cells within an extensive myxoid
stroma.
Generally hypocellular
stroma and the tumor cells stain positive with PAS,
whereas only the stroma shows positive staining with
Alcian blue

43. D/D
Vegetation-leaflets /clinical setting
Mural thrombi-setting-MS/DCM
 appendage
 laminated immoble
 broad base

44. Treatment
Surgical en bloc resection with a margin of normal
tissue, if anatomically feasible, is considered curative
and is the treatment of choice

45. Overall risk of recurrence after resection is 13%, but is
much more common with familial myxomas than
with sporadic tumours (22% vs 3%).
Hazard of recurrence increases linearly for 4 years
after resection, after which risk of recurrence is low.
 Based on this observation, semi-annual surveillance
echocardiographic follow-up has been recommended
for 4 years following surgery

46. • Immediate postoperative mortality in most series
ranges from 0 to 7.5 percent.
• Common postoperative complications include
arrhythmias
• Recurrence of myxoma in the brain-
• Growth of the embolized tumor fragments,
• Difficult to manage, but chemotherapy is not
recommended because embolic myxomas do not truly
represent meta-static diseases.
• Rare but potentially life-threatening complication is
the development of cerebral aneurysm secondary to
embolic tumor fragments.

47. Papillary fibroelastoma
MC from valvular endocardium
10% of primary cardiac tumours
Second most common primary cardiac tumour
Above 60 yrs of age
Ventricular surface of semilunar valves and atrial
surface of AV valves
Adults-aortic valve ( 37 to 45 percent)
Children-tricuspid valve.

48. Characteristic flower-like appearance with multiple
papillary fronds attached to the endocardium by a
short pedicle, -typical ‘sea anemone’ appearance when
immersed in saline
Usually solitary (91 percent) and <1 cm in diameter
but can be larger, particularly when they occur in the
cardiac chambers

49. • Often asymptomatic.
• MC -systemic embolisation resulting from attached
thrombi as well as from fragmentation of the papillary
fronds themselves -50% of symptomatic patients
• Rarely, patients present with subacute bacterial
endocarditis–like findings, and pulmonary embolism
and sudden death have also been reported.

50. Men and women are equally affected.
There is a strong association with hypertrophic
obstructive cardiomyopathy (HOCM), as well as
surgical, radiation, and haemodynamic trauma

51. Echo- usually appearing as a small, mobile,
pedunculated valvular mass.
 They usually have a well defined ‘head’ and
characteristically have a stippled edge with a
‘shimmer’ or ‘vibration’ at the tumour blood interface
TEE - definitive imaging modality

53. CT/MRI- temporal and spatial resolution is often still
inadequate to characterise most of these tumours
since they are usually small
Preoperatively CT angiograms -coronary vasculature,
in order to avoid manipulating the tumours into the
coronary ostia at the time of coronary angiography.

55. Lipoma and lipomatous
hypertrophy
• <5% of surgically excised primary cardiac tumours
• MC site-LV/RA
• Usually arise from the epicardial surface, most often
from a broad pedicle, and grow into the pericardial
space.
• Subendocardial lipomas are often small and sessile
grow as broad based pedunculated masses protruding
into the cardiac chambers.

56. • Asymptomatic but may cause symptoms due to local
compression or arrhythmias
• More echo dense than myxoma
MRI -diagnostic – superior in differentiating between
fat and connective tissue- high in signal intensity on
T1 and show evidence of signal dropout on fat
saturation sequences

57. Lipomatous hypertrophy of the atrial septum
may often be confused with a cardiac tumour.
 It is a benign non-neoplastic condition that results
from adipose cell hyperplasia and is associated with
increasing age and obesity.
 It involves the limbus of the fossa ovalis, sparing the
fossa ovalis membrane and resulting in a
characteristic dumbbell shape
Interatrial septum -diameter exceeding 2 cm in
transverse dimension

58. Does not cause any symptoms
In rare instances in which the tumor protrudes into
the right atrium and the superior vena cava, patients
can present with symptoms secondary to blood flow
obstruction

59. • CT and CMR –
• IAS is thickened to up to 7 cm, whereas normally it is
less than 1 cm
• LHAS with symptomatic arrhythmias can be managed
medically, whereas surgical excision should be
restricted to the rare cases in which the disease causes
symptomatic hemodynamic obstruction

61. Rhabdomyoma
MC in children (80 percent younger than the age of 1)
most common cardiac mass in childhood -50% to
75% of pediatric cardiac tumors
Commonly associated with tuberous sclerosis-
predilection for the ventricles, and they often involve
the interventricular septum.
1 to 3 cm in size/multiple.

62. Sixty percent of the older children and less than 25%
of adults with tuberous sclerosis, however, will have
detectable cardiac masses
Occasional cases are seen in the absence of tuberous
sclerosis-Approximately 50% of the lesions are single
Often do not demonstrate spontaneous regression,
and they may require surgery

63. The lesion typically appears as a yellow-gray, firm,
circumscribed lobulated mass, and the size of the
tumor can range from less than 1 mm to 9 cm

64. Fetal/Infant Adult
Result in stillbirth or early most common –arrhythmias
postnatal death - significant Sporadic
hemodynamic impairment. Spontaneous regression rare
Obstruction may occur to
either the RV/LVOT-
prominent intracavitary
component, and significant
cardiac murmurs
Can regress spontaneously
Always assoc. wth tuberous
sclerosis

65. • high incidence of ventricular pre-excitation and Wolff
Parkinson White syndrome, and may increase the risk
of arrhythmia
• A characteristic and peculiar feature of
rhabdomyomas is spontaneous regression in size or
number or both in most patients <4 years of age

67. Appear well circumscribed and slightly brighter than
the surrounding normal myocardium.
Appear hypodense on contrast CT- isointense to
myocardium on T1 weighted images and hyperintense
on T2 weighted images.
.

68. As spontaneous tumour resolution is common,
management is expectant in asymptomatic patients
 Occasionally, surgical resection is necessary if the
tumours are large, resulting in structural or
haemodynamic complications.

69. Cardiac fibroma
It is the most common resected cardiac neoplasm in
children and the second most common benign
primary cardiac tumour found at autopsy in children
characteristically solitary (unlike rhabdomyomas)
and are invariably located in the ventricles
Ventricular septum/ the LV free wall/ the right
ventricle/ the atria in that order

70. Typically well circumscribed, and often centrally
calcified without cystic change, necrosis or
haemorrhage.
They usually affect children, a third of whom are
younger than 1 year at presentation.
 Most cardiac fibromas appear to occur sporadically

71. Gorlin syndrome- basal cell carcinomas of the skin,
odontogenic keratocysts, rib and vertebral anomalies,
and multiple skin lesions

72. One third of patients present with arrhythmias, one
third with heart failure or cyanosis, and one third are
detected incidentally.
Less common presenting findings include sudden
death and atypical chest pain
 ECG –LVH/ RVH/ BBB /AV block/ VT
Xray- cardiomegaly with or without focal bulge, and
calcification -15 percent of cases

73. Echo-discrete often obstructive, echogenic,
noncontractile mass ranging in size from 1-10 cm in
diameter in a ventricular wall.
 The tumour may mimic hypertrophic
cardiomyopathy or ventricular septal hypertrophy

74. • CT-homogenous masses with soft tissue attenuation
that may be either infiltrative or sharply marginated.
• Calcification is often seen.
• MRI-homogeneous and hypointense on T2 weighted
images and isointense relative to muscle on T1
weighted images.
• Little or no contrast material enhancement.
• MRI also demonstrates the extent of myocardial
infiltration which can guide tumour resection.

75. • Surgery appears to be the optimal treatment in
patients with symptomatic resectable tumours.
• The role of surgery in patients with asymptomatic
tumours is less clear, as cardiac fibromas can remain
dormant for many years and even regress.
• However, because of fatal arrhythmias, surgery is
often recommended despite absence of symptoms.
• Transplantation is considered for large and
unresectable tumours

77. Hemangiomas and
Lymphangiomas
Less than 2 percent of primary cardiac neoplasms
Occur in any age group ranging from a few months to
the seventh decade of life

78. • Clinical presentation of is variable
• Arrhythmias
• CHF
• pericardial effusion
• Ventricular outflow tract obstruction
• Giant cardiac hemangioma can result in Kasabach-
Merritt syndrome -thrombosis, consumptive
thrombocytopenia, and coagulopathy.
• Occasionally be associated with hemangioma in
extracardiac sites

79. • Echo-sensitive -cardiac hemangioma appearing
typically as a hyperechoic lesion.
• CAG-can sometimes demonstrate blood supply to the
tumor, with the presence of “tumor blush
• Chest CT- heterogeneous signal with intense
enhancement in most cases after contrast material
administration.
• On CMR-with intermediate signal intensity on T1-
weighted images and hypointense signal on T2-
weighted images and there may be rapid
enhancement during contrast infusion

80. Radical resection -recommended because of the
potential for recurrence, especially if the resection is
incomplete
The postoperative prognosis is excellent in resectable
cases
Conservative management may be considered in
asymptomatic patients, particularly if complex and
potentially hazardous excision is required

82. Malignant primary cardiac tumours
• Exceedingly rare.
• 15% of primary cardiac tumours
• Vast majority (95 per cent) – sarcomas
• 5%- primary cardiac lymphomas and mesotheliomas
• secondary cardiac malignancy- 30 times more
common-lung and breast cancer.

83. General features
High mitotic activity (>5 mitotic figures/10 high-
power fields), extensive tumor necrosis, and poor
cellular differentiation presence of metastases -
poorer prognosis.
CT or CMR - large, heterogeneous, broad-based
masses that frequently occupy most of the affected
cardiac chambers

84. Sarcoma of life
• 3 & 5 decades
rd th
• M=F
• Commonly affect the left side, mostly the left atrium
• Rapidly progressive with a median survival of 1 year
due to widespread local infiltration, intracavitary
obstruction
• Metastases-often already present at the time of initial
presentation

85. • Angiosarcomas-
• 30 to 37 percent of the cases
90 percent -right atrium(differentiating feature in
that most of the other sarcomas have a left atrial
predilection,)
• Dyspnea, chest pain, heart murmur, constitutional
symptoms, arrhythmias, superior vena cava
syndrome, and evidence of congestive heart failure.
• pericardial effusion and cardiac tamponade
• metastatic disease –stroke like neurologic symptoms
secondary to cerebral metastases

86. • Echocardiography – broad based right atrial mass
near the inferior vena cava.
• CTand MRI - avid, arterial phase enhancement
permitting a definitive diagnosis.
• Transvenous echo-guided cardiac biopsy/biopsy of
the metastatic lesion in a more accessible location or
cytology examination on pericardiocentesis fluid
• Novel lymphatic endothelial markers including D2-40

87. Treatment
• Mean survival of 9 to 10 mon. -late detection of the
disease—most patients present with advanced-stage
disease.
• Integrated approach -combination of surgery,
irradiation, adjuvant/neoadjuvant chemotherapy, and
immunotherapy using interleukin-12 (IL-12).
• Advanced-stage unresectable disease, palliative
treatment including -metallic stents for SVC
syndrome and for severe RVOTO

88. Rhabdomyosarcomas
• Most common primary sarcoma of the heart in
children
• Average age of disease presentation is in the second
decade of life
• M>F
• Multiple lesions are frequently present (60 percent).
• Embryonal type and pleomorphic type of -primary
tumors in the heart
• Alveolar type - metastatic disease to the heart.

89. congestive heart failure, arrhythmias, cardiac
murmurs, and constitutional symptoms
 Nonspecific ECG and chest radiography findings are
often present.
TTE/TEE guided biopsy -attempted for tissue
diagnosis, a negative result cannot be relied on
because there is a high rate of false negatives
Chest CT or CMR -delineation of the nature, origin,
and extent of the lesion, especially if a malignant
lesion is suspected

90. • Metastases-MC to the lung and lymph nodes,
• Survival is usually less than 1 year.
• High risk biopsy and extensive myocardial and
pericardial extension are associated with the worst
prognosis.
• Highly infiltrative nature of tumor often precludes
surgery.
• Tumor has a poor response to radiation and
chemotherapy
• Heart transplant -if no obvious distant metastases are
present

91. Leiomyosarcomas
• Mean age of presentation is in the fourth decade, and
there is no apparent sex predilection.
• Dyspnea, pericardial effusions, chest pain, atrial
arrhythmias, and congestive heart failure.
• 70 to 80 percent -the left atrium, and they tend to
extend into the pulmonary trunk.
• Typically solitary but can be multiple in 30 percent of
patients

92. Prognosis is poor with a mean survival of 6 months
after diagnosis.
Because of the tendency of leiomyosarcomas to recur,
cardiac transplantation is not a realistic option

94. LYMPHOMAS
1.3 to 2 percent of all primary cardiac tumors
Immunocompromised individuals - more common
PTLD -chronic immunosuppression and Epstein-Barr
virus infection.
Lymphomas -HIV and PTLD -extracardiac
involvement at presentation, and isolated cardiac
involvement is rare.

95. • Right side of the heart in 69 to 72 percent of the cases
• Single lesion in 66 percent and multiple lesions in 34
percent of the cases.
• 80 percent -immunocompetent individuals -diffuse
large cell B-cell lymphoma type
• Immunocompromised patients- small noncleaved or
immunoblastic lymphomas

96. • 62 to 67 years with a range of 13 to 90 years
• M>F
• chest pain, congestive heart failure, pericardial
effusion, palpitation, and arrhythmias
Frequently involve the epicardium and extend to
involve the pericardium
• TEE- excellent for initial visualization
• CT and CMR are superior at delineating the
infiltrative nature of the tumor and CMR has the
highest sensitivity for detecting primary cardiac
lymphomas

97. • Sensitive to chemotherapy - anthracycline-based
chemotherapy with or without radiation therapy
-mainstay of treatment for primary cardiac
lymphomas
• Radical surgical excision is generally discouraged

98. Metastatic disease of the heart
and pericardium
20 to 40 times more common than primary cardiac
neoplasia
metastatic melanoma-46% to 64%
lung carcinoma-36%
leukemia, lymphoma-20%,
carcinoma of the breast 7%,
carcinoma of the esophagus 6%

99. Frequent presence of pericardial effusions,
unexplained shortness of breath, and the new
development of an arrhythmia in a patient with a
known malignancy

100. Four pathways
Direct extension-lung carcinomas, as well as primary
mediastinal tumors such as malignant thymoma
Retrograde extension-lymphatics-lung and
esophageal carcinoma-pericardium frequently is
involved

101. Hematogenous spread -melanoma, sarcomas,
leukemia, and renal cell carcinoma –manifested most
often by multiple intramyocardial metastatic deposits
Transvenous extension to the right side of theheart
-renal cell carcinoma, adrenal carcinoma, or
hepatocellular carcinoma
Transvenous extension to the left atrium is seen most
often in primary lung carcinoma

102. Conclusions
Cardiac tumours are being increasingly recognised
antemortem, permitting earlier diagnosis and
treatment
Aetiology can often be determined by considering the
histology based likelihood, the age of the patient at
time of presentation, tumour location and non
invasive imaging.

103. CT and MRI are complimentary techniques, often
better suited for intramyocardial and pericardial
lesions as well as for assessment of extracardiac
spread.
For benign cardiac tumours, an early diagnosis and
appropriate treatment is not only possible but often
curative.
Unfortunately the outcome for malignant primary
tumours, even despite early diagnosis and aggressive
treatment, remains dismal.