Imaging in cardiac masses by Dr. Bom BC

1. IMAGING IN CARDIAC TUMORS
DR BOM B. C.
MD RESIDENT 2nd YEAR

2. INTRODUCTION
• Metastatic tumors of the heart are more
common than primary cardiac tumors, which
are very rare.
• Patients with cardiac tumors may be
asymptomatic, or they may present with
arrhythmias or
• hemodynamic, embolic, or constitutional
symptoms.

3. • The reported incidence of primary cardiac
tumors ranges from 0.001% to 0.03%.
• Most cardiac tumors have benign histologic
features (75%).
• Primary cardiac tumors are often evaluated by
echocardiography, but CT, MRI, or both can be
useful in their assessment.

5. Relative incidence of benign heart
tumors

7. Relative incidence of primary
malignant heart tumors

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

9. 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,
thrombocytosis/ thrombocytopenia,
hypergammaglobulinemia, and increased ESR
• Mimic those of several connective tissue diseases

10. 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

11. • 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

12. • 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

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

14. • 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

15. 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.

16. • 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 steepy
descent, and an early diastolic murmur or
holosystolic murmur
• SVC syndrome - findings of peripheral edema, HSM,
ascites,

17. 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

18. 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.

19. 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.

20. Diagnostic Approach

21. 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

22. 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.
• —10% of primary cardiac tumours excised at surgery
are malignant, of which more than 90% are
sarcomas. The remaining few are represented by
lymphomas

23. Location

25. 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
• —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

26. Primary benign cardiac tumours
Myxoma:
The most common cardiac tumor “myxoma” accounts
for about 50% of all benign cardiac tumors {Sutton}
and 25% of all cardiac tumors.
• The incidence of cardiac myxoma is 0.5 per million
population per year with a female predominance.
• The most common anatomic location is the left
atrium (75% to 80%), followed by the right atrium
(10% to 20%).

27. • Myxomas less commonly involve either ventricle or involve
both atria (5% to 10%), and the mitral valve is rarely involved.
• Left atrial myxomas are typically pedunculated and arise from
the interatrial septum, near the fossa ovalis. Right atrial
myxomas tend to be sessile and may arise from areas of the
atrium other than the septum.
• On CT, a myxoma has the appearance of a filling defect in the
chamber of origin . The mass is usually heterogeneous and
may have areas of calcification.

28. • Sporadic vs familial
• Majority sporadic; some are familial (autosomal
dominant transmission) or part of a syndrome
1. Carney complex – spotty skin pigmentation,
myxomas, endocrine over-activity, schwannomas
2. NAME syndrome – nevi, atrial myxoma, myxoid
neurofibroma, ephelides
3. LAMB syndrome – lentigines, atrial myxoma, blue
nevi

32. Imaging
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

33. 2D Transthoracic/Transoesophageal
Echocardiography
B mode:—
• Appear as homogenous echo masses
• Echo free spaces-hemorrhage
• —Areas of calcification.
M mode
• —LA-tumor fills LA in systole
• —Diastole - prolapses into mitral valve orifice
mass of echoes appear behind AML
• —EF slope decreases

35. CE CT - overall attenuation lower than that of
myocardium.

36. CMR shows heterogeneous signal intensity in 90
percent of cardiac myxomas,
•T1- images - isointense signal
•Cine gradient-echo CMR - superior to other
imaging modalities
Cardiac Catheterization: risk of tumor emboli:
for suspected CAD.

37. FIGURE 22.41. Leh Atrial Myxoma-MR. Two-chamber,
long-axis gradient-echo cine image shows a leh atrial
myxoma (arrow). The myxoma has very low signal on this
gradient-echo image.

38. 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.

39. • —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

40. •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.

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

44. Rhabdomyoma
• Rhabdomyomas are the most common
pediatric (50-75%) cardiac tumor, typically
occurring in children younger than 1 year of
age(-80%).
• Rarely, they have been reported in adults.
• They are usually multiple and occur most
commonly in either ventricle, although the
atria may also be involved.
• They do not typically occur in association with
the heart valves.

45. • Patients with rhabdomyoma are usually
asymptomatic.
• However, the tumors may cause arrhythmias or
obstruction, resulting in acute heart failure and
sudden death.
• The incidence of cardiac rhabdomyoma in patients
with tuberous sclerosis is 30% to 50%.
• Rhabdomyomas are thought to be hamartomatous
lesions.
• They do not grow rapidly and may regress over time ,
thus, they may be monitored in asymptomatic
individuals.

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

47. •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

48. • Echocardiography is usually used for
evaluation of these tumors.
• On CT scans,
1. Noncontrast-enhanced rhabdomyomas are
typically denser than the adjacent
myocardium and may have areas of fat
density.
2. Enhancement of the rhabdomyoma is
demonstrated following administration of
iodinated contrast.

49. s
Left Ventricular Rhabdomyoma-MR.
Coronal spin-echo image through the
aorta (Ao) and leh ventricle (LV)
demonstrates a high-signal polypoid
mass near the outflow tract of the LV
(arrow). This young patient had
tuberous sclerosis, and a presumptive
diagnosis of ventricular rhabdomyoma
was made.

50. Lipoma
• Cardiac lipomas are usually solitary and may occur in the
myocardial tissue, usually in a subepicardial location
(Compression of the heart, pericardial effusion).
• Because affected patients are usually asymptomatic, the
lesions are usually found incidentally.
• Cardiac lipomas can be associated with rhabdomyomas and
tuberous sclerosis. Although these lesions may be first
detected by echocardiography, CT or MRI can document their
fatty nature, establishing the diagnosis.
• They are typically located on the epicardial surface.
• If subendocardial :with intracavitary extension, may produce
symptoms characteristic of their location; Most common
chambers affected: LV, RA & IAS

51. Coronal turbo fast low-
angle shot (FLASH) MRI
scan of a patient with a
right atrial lipoma shows a
high-signal intensity mass
(L) in the lateral wall of the
right atrium. High signal
intensity on T1 imaging is
strongly suggestive of
fatty tissue and identifies
this mass as a lipoma.

52. Cardiac fibroma
• It is the most common resected cardiac neoplasm in
children and the second most common benign
primary cardiac tumor found at autopsy in children
• Characteristically solitary (unlike rhabdomyomas)
• Are invariably located in the ventricles, Ventricular
septum/ the LV free wall/ the right ventricle/ the
atria in that order.
• 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

53. • Gorlin syndrome - basal cell carcinomas of the skin,
odontogenic keratocysts, rib and vertebral
anomalies, and multiple skin lesions
• ECG –LVH/ RVH/ BBB /AV block/ VT
• Xray- cardiomegaly with or without focal bulge, and
calcification -15 percent of cases
• 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

54. • CT scan :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.

55. 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. • 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 associated with hemangioma in
extracardiac sites

56. • 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

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

60. General features
• High mitotic activity (>5 mitotic figures/10
highpower 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.

61. Sarcoma
• 3rd & 5th decades of life: 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

62. 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). Most diagnosed when metastasis is
present, common being lung.
• 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

63. • Echocardiography – broad based right atrial mass
near the inferior vena cava.
• CT and 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

65. 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.

66. • 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

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

68. 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.
• 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.

69. LYMPHOMAS
• Although up to 25% of patients with
lymphoma have cardiac involvement at
autopsy, primary cardiac lymphoma
(lymphoma limited to the heart and/or
pericardium) is very rare.
• Primary cardiac lymphoma is usually a B-cell
lymphoma. The most common location is the
right heart, usually arising from the right
atrium.
• Associated pericardial effusion is present.

70. • 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

72. Secondary Cardiac Tumors
• Metastatic cardiac involvement is much more (20-40 times)
common than primary cardiac neoplasms, but it may be
undetected before death.
• Autopsy studies have found cardiac metastases to be present
in up to 20% of patients with neoplasm.
• They occur most frequently in patients with lymphoma,
leukemia, or melanoma(40% to 50%) and in patients with
lung or breast cancer (10% to 33%).
• Direct extension of tumor is the most common route and
typically occurs in lung and breast cancers.
• Symptoms tend to be related to associated pericardial
involvement.

73. • Renal cell carcinoma, adrenal carcinoma,
hepatocellular carcinoma, and uterine
leiomyosarcoma may involve the heart by
extension through the IVC.
• Thyroid carcinoma may extend into the heart
through the SVC.
• Lung cancer may also spread along the
pulmonary veins to involve the left atrium.

74. • Finally, lymphangitic metastases may occur as
well as hematogenous metastases.
• Leukemia and lymphoma are the most
common tumors to cause cardiac metastases
by the lymphangitic route, in which case
mediastinal nodes are invariably involved.

77. 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.

78. • —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.

79. • Textbook of Radiology and Imaging- David
Sutton
• Fundamentals of Diagnostic Radiology-Bryants
& Helms
• CT & MRI of whole body- John Haaga

80. THANK YOUTHANK YOU