Cardiac
Neoplasms:
Secondary cardiac neoplasms
(metastases) are much
more common than primary lesions (about 40 to 1000 times more common)
and can
be identified in 10-20% of patients with an underlying malignancy at
autopsy.
Malignancies commonly associated with cardiac metastases include lung,
breast,
esophagus, kidney, sarcoma, and lymphoreticular
malignancies [8,11,14,16]. Melanoma also has
a
propensity to metastasize to the heart [8]. Cytologic
studies are positive in 80-90% of patients with malignant pericardial
effusions
[8]. Primary cardiac malignancies are very rare [11]. The incidence of
all
primary cardiac tumors is estimated to be between 0.002-0.19%
[14]. Approximately 25% of primary cardiac tumors are malignant [14].
Fibroma:
- Clinical:
Cardiac fibromas is a very rare benign congenital neoplasm that
typically
affects children, one-third of whom are under 1 year of age [6]. It is
the
second most common primary benign cardiac neoplasm in children 9after rhabdomyoma) [16]. About 15% of cardiac fibromas
occur in adolescents and adults. Cardiac fibroma
are
almost always solitary and are located in the ventricular myocardium-
typically
the interventricular septum and they
affect the left
ventricle more than the right [1,14]. The
central
portion of the tumor often has multiple areas of calcification and
cystic
degeneration. One third of affected patients are asymptomatic [6].
Common
clinical findings include heart failure, arrhythmia, and sudden death
[6].
Treatment is surgical excision for symtomatic
patients as cardiac fibromas may remain
stable for
many years or regress [6]. However, other authors recommend resection
even in
asymptomatic patients due to the increased risk for sudden cardiac
death [14].
Recurrence following surgical excision is rare [6].
The lesion is associated with polyposis
syndromes
and Gorlin's syndrome (basal cell nevus
syndrome [autosomal dominant] consisting of
multiple nevoid basal
cell carinomas of the skin, odontogenic
keratocysts of the mandible, bifid ribs,
and a
tendency toward neoplastic growth in severalorgan systems [7]). However, less than
14% of
patients with Gorlins syndrome have
cardiac fibromas [6]. Tumors associated
with polyposis
syndromes appear to occur more commonly in the atria [14].
- X-ray:
The most common radiographic finding is cardiomegaly
[6]. A focal bulge may be seen if the tumor involves the ventricular
free wall
[6]. Tumor calcification can be seen in about 25% of cases- and this
aids in
differentiation from rhabdomyomas. CT will
reveal a
homogeneous mural mass (hemorrhage and necrosis are absent)-
it may be sharply marginated or
infiltrative [7,16].
The lesion often does not enhance significantly after contrast
administration
[7], but enhancement has been reported [6,7].
An
associated
pericardial
effusion
may
be seen [6]. On MR, the lesions
appear as a
discrete mural mass or focal myocardial thickening. The lesion is isointense (or hyperintense
or hypointense) to myocardium on T1 images
and hypointense to the myocardium on T2
images [6]. There is
only slight or no enhancement following contrast administration
compared with
the surrounding myocardium because of the minimal vascular supply [14].
However, other authors report that the lesion enhances heterogeneously
[6].
Hemangioma:
- Clinical:
Cardiac hemangiomas are rare tumors
that account
for 5-10% of benign cardiac tumors [11]. Most patients are asymptomatic
[6].
The tumor can arise from the endocardium,
myocardium,
or epicardium [14]. They are usually found
in the
ventricles (particularly in the lateral wall of the left ventricle and
less
commonly in the anterior wall of the RV or the septum), although they
may
occasionally be multifocal [14,16]. However,
other
authors have indicated that they usually arise from the right atrium
and have
an intracavitary component. TheyThe
most common clinical presentation is dyspnea
on
exertion [11]. Patients may also have skin hemangiomas
[1]. Cardiac hemangiomas can occur in the
setting of Kasabach-Merritt syndrome-
which is characterized by mltiple systemic
hemangiomas
associated with recurrent thrombocytopenia and consumptive coagulopathy
[6]. Surgical excision is the treatment of choice with an excellent
long term
prognosis [6].
- X-ray:
At echocardiography the lesion appears hyperechoic
[6]. The lesion is usually heterogeneous with areas of interspersed fat
on
non-contrast CT and enhances intensely with
contrast
[6]. Phleboliths are characteristic of an hemangioma
[16].On MR, similar
to hepatic hemangiomas, the lesion has iso- to intermediate intensity on T1 images and
become hyperintense on T2 FSE images [6].
Intense, inhomogeneous
enhancement has been reported following gadolinium administration [11,14].
Lipoma:
- Clinical:
Cardiac lipomas are very rare,
typically solitary
benign neoplasms [12]. Despite this fact, lipomas are the second most common primary
benign cardiac
neoplasm [16]. Cardiac lipomas have no sex
predilection and can occur at any age, but are more commonly found in
adults
[6]. Nearly all cardiac lipomas are epicardial lesions, but myocardial or endocardial
lesions may also occur (50% being subendocardial,
25%
myocardial,
and
25%
subepicardial [12])
[6]. However,
other authors report that half of cardiac lipomas
originate from the subendocardial layer
and the other
half from the subepicardial or myocardial
layers and
grow into the pricardial sac [16]. The
most commonly
reported locations for the lesion are the right atrium, the left
ventricle, and
the interatrial septum [12]. The lesion
may be
asymptomatic. Symptoms include cardiac murmur, arrhythmias due to
involvement
of the cardiac conduction system, or symptoms of cardiac compression [6,7]. Multiple cardiac lipomas
have
been described in association with tuberous sclerosis [12]. Surgery is
the
treatment of choice, but tumors which infiltrate the heart may not be resectable [6].
- X-ray:
The most common radiologic finding is cardiomegaly
[6]. CT and MR images can demonstrate the fatty nature of the lesion.
|
The patient below presented with chest pain. CXR revealed an
abnormal contour to the right heart border. Echocardiography suggested
an atrial mass and this was confirmed on
CT imaging. Note low attenuation areas within the periphery of the
lesion of fat attenuation.
|
|
 
|
|
MR imaging confirmed the presence of fat within the lesion (T1
and T1 fat sat images shown below). Despite the presence of soft tissue
elements within the lesion, the tumor was found to be an intramyocardial lipoma
at pathologic analysis.
|
|
 
|
Lipomatous Hypertrophy of the Atrium:
- Clinical:
The condition is not a true neoplasm (unlike a lipoma),
but
rather
a
focal
collection
of mature adipose tissue. It is found in
obese,
female, and elderly patients. There may be an association with cardiac
arrhythmias. It is defined as any deposition of fat in the interatrial
septum at the level of the fossa ovalis
which exceeds 2 cm in transverse dimension (the condition spares the fossa ovalis) [7].
The term
"hypertrophy" is actually a misnomer as the condition is caused by an
increase in the number of adipocytes, not
hypertrophy
[7]. Because of the presence of brown fat, this entity may show FDG
uptake on
PET imaging [14].
Lymphoma
(Primary Cardiac Lymphoma):
- Clinical:
Primary cardiac lymphoma is very rare - accounting for 1.3% of
primary cardiac tumors and less than 1% of extranodal lymphomas
[19,20]. The lesion is typically an aggressive non-Hodgkins
B-cell type (diffuse large cell lympphoma) [6,20]. To be considered a
primary cardiac lymphoma, these tumors involve
only the
heart or pericardium at the time of diagnosis, with no evidence of extracardiac lymphoma [6]. The lesion is seen
with
increased
frequency in immunocompromised patients
with HIV due to Ebstein-Barr virus associated lymphoproliferative
disorders [6,16]. However, even in HIV and
organ transplant patients, it still represents less than 5% of all
lymphomas in these patients [20].
Patients present with
unresponsive, rapidly
progressive heart failure, dyspnea, arrhythmias, chest pain, and SVC
syndrome.
The
prognosis is poor and there is no evidence that surgery improves
prognosis [6].
The lesion most
commonly
involves the right heart (particularly the right atrium) with frequent
involvement of more than one chamber [11]. Pericardial thickening or
effusion are a common early feature of the disease [20]. Infiltration
of atrial or ventricular walls with extension along the epicardial
surfaces encasing adjacent structures (including the coronary arteries
and aortic root) is also a notable feature [20]. On CT, the lesion is
often isoattenuating to hypoattenuating relative to myocardium and
demonstrates heterogeneous enhancement [20]. Left ventricular
involvement
is associated with a worse prognosis [19].
- X-ray:
The CXR usually demonstrates cardiac enlargement and evidence of
heart
failure. CT demonstrates a mass that is hypo- to isodense
to myocardium with heterogeneous enhancement. There is often an
associated
large pericardial effusion [14]. In fact, a pericardial effusion may be
the
only finding [16]. The tumor can appear iso
or hypointense to myocardium on both T1
and T2 FSE sequences
and appear poorly marginated and
heterogeneous
following contrast administration [14].
Malignant
Cardiac Sarcomas:
- Clinical:
About 25% of primary cardiac tumors are malignant and the majority
are sarcomas (sarcoma is the most common primary cardiac
malignancy
[16]). Angiosarcoma (37% of cases) is the
most common
primary cardiac malignancy in an adult [11]), followed by
undifferentiated
sarcoma (24% of cases), leiomyosarcoma
(8-9% of
cases), rhabdomyosarcoma (more prevalent
in children
[11]), and malignant fibrous histiocytoma
(11-24% of
cases). Primary cardiac sarcomas affect adults and are extremely rare
in
infants and children [6]. Dyspnea is the
most common
presenting symptom [6]. Nearly 80% of patients with cardiac sarcomas
have
distant mets at presentation. Prognosis is
uniformly
poor.
Angiosarcoma: Cardiac angiosarcomas
account is the most common cardiac sarcoma [4,6].
Patients
generally
present
between
the
ages of 20-50 years (middle aged men
[14]). They most commonly occur in the right atrium (80% of cases [6])
and
produce valvular obstruction and right
ventricular
failure. Pericardial involvement is common [4,6].
Multiple
lesions
occur
in
60%
of patients [16]. Metastases occur in 66-89% of
cases at the time of presentation [6], most commonly to the lung [11].
Prognosis is poor [4].
The typical CT findings of angiosarcoma
include an
irregular, nodular, broad-based right atrial
mass
with heterogeneous enhancement and pericardial infiltration (thickening
or
effusion) [16]. On MRI the tumor is typically heterogeneous with low
signal
intensity on T1 and increased intensity on T2 FSE images [14]. Areas of
high
signal are seen due to necrosis or blood filled spaces/hemorrhage
within the
tumor. A "cauliflower" of papillary appearance has also been
reported. There is heterogeneous enhancement of the mass following
contrast
administration [10] with marked surface enhancement (a "sun ray"
appearance has also been described [11]). Because of the tendency of
the tumor
to involve the pericardium, pericardial thickening or nodularity,
or a hemorrhagic pericardial effusion may also be detected. [2,11]
Malignant Fibrous Histiocytoma:
Although
rare for the tumor to occur in the heart, it is the next most common
cardiac
sarcoma. It may occur more commonly in women. The lesion commonly
arises from
the posterior or septal wall of the left
atrium. It
can obstruct atrial filling and outflow
leading to
left sided heart failure. [2]
Undifferentiated sarcoma: The tumor can occur at any age, but
patients are typically in their 4th or 5th decade, and the lesion has a
propensity to arise in the left atrium [4,16].
The
tumor
typically
appears
isointense
compared to the
myocardium on FSE images [14].
Leiomyosarcoma: Uncommon cardiac
tumor (1%
of all primary cardiac tumors [11]) with a predilection for the
posterior wall
of the left atrium (and invasion of the pulmonary veins is frequently
seen)
[14]. They usually affect patients during the 4th decade [14]. The most
common
presenting symptom is dyspnea and cardiac
failure
from mitral obstructive symptoms [11]. The lesion can be multiple in up
to 30%
of cases [4,11] Prognosis is poor. On
MR the
lesion is usually sessile, lobulated and
appears hypointense to myocardium on T1
images, hyperintense
on T2, and demonstrates marked contrast enhancement [11].
Fibrosarcoma: Fibrosarcoma
tends to occur in the left atrium and usually amnifests
as CHF [14].
Extraskeletal cardiac osteosarcoma: Account for 3-9% of cardiac
sarcomas
[16]. The tumor is usually centered in the left atrium and patients
present
with pulmonary congestion [14]. The lesion commonly contains large foci
of
calcification [14].
Mesothelioma: Pericardial mesothelioma
- Clinical:
The tumor can occur at any age with a mean age of 46 years at
presentation
[6]. Patients present with chest pain, dyspnea,
cough,
and
palpitations
[6].
Although
there appears to be a relationship to
asbestos exposure, a definite association has not been established due
to the
rarity of this lesion [6]. Surgery combined with radiation therapy may
provide
some palliation, but the prognosis is extremely poor [6]. The tumor
usually
forms multiple coalescing masses that envelop the pericardial space
[11]. On CT
there is nodular, irregular, diffuse pericardial thickening and a
pericardial
effusion [6].
Myxoma:
- Clinical:
Primary cardiac tumors are uncommon and approximately 75% of primary
cardiac
tumors are benign. Myxomas account for
nearly half of
the benign cardiac tumors encountered and are therefore the most common
primary
cardiac tumor [9,22]. It is a benign neoplasm which arises most
commonly
from the
inter-atrial septum in the region of the fossa ovalis (78%
of cases [16])- extending into the left
atrium (60% to 75%) more commonly
than the right atrium (20% to 28%) [14]. Ventricular myxomas
are uncommon. Myxomas can present at any
age (mean,
50 years) and there is a female predominance (1.7:1 to 4:1) [6]. Left atrial lesions may prolapse
through the mitral valve and cause atrial
obstruction
and hemodynamic alterations with dyspnea
or syncope.
The signs and symptoms are typically indistinguishable from mitral
valve
disease. Constitutional symptoms such as weight loss, fever, fatigue,
and arthralgias are seen in about
one-third of patients [6] and
may be related to an autoimmune reaction initiated by the tumor [9].
Peripheral
emboli or tumor emboli may also occur in 22% to 60% of patients
(particularly with myxomas with irregular surfaces which have a
tendency to induce thrombi formation [22])- most commonly to
the
brain (resulting in stroke), kidneys, or peripheral arteries (resulting
in limb
ischemia or infarction). Right sided myxomas
will embolize to the lungs. About 20% of
patients are
asymptomatic [6]. Cardiac arrhythmias occur in 20% of affected patients
[6].
Treatment is surgical excision with an excellent long term
prognosis and
low risk of recurrence. [5,6]
Cardiac myxomas can be a sporadic
lesion (93% of
cases). About 86% of these lesions occur in the left atrium, 94% are
single,
and the estimated risk of a second myxoma
developing
following surgical resection is 1-3% [6]. The lesion usually arises
from the interatrial septum immediately
adjacent to the fossa ovalis
[11].
About 7% of cardiac myxomas exhibit
atypical
biologic behavior, including multicentricity,
atypical
location
(in
cardiac
chambers
other than the left atrium), recurrence
after surgical excision (12-22%), and an association with unusual
conditions
such as Carney complex [6]. These patients are usually younger (mean
age 28
years), more commonly male, and exhibit a familial predisposition for
cardiac myxomas [6]. The lesions are
associated with a higher risk
of recurrence (12-22% of cases) [9]. The Carney complex is an autosomal-dominant, inherited disorder in which
cardiac myxomas occur in association with cutaneous
and mammary myxomas (myxoid
fibroadenomas [9]), spotty skin
pigmentations,
endocrine overactivity (Cushing syndrome,
sexual precocity,
and acromegaly), psammomatous
melanotic schwannoma,
primary
pigmented
nodular
adrenal
disease,
and testicular neoplasms
(particularly Sertoli cell tumor) [6].
Cardiac myxomas occur in about two-thirds
of patients with Carney
complex [6], often at a younger age [16]. The myxomas
can also occur outside the left atrium and have a higher rate of
recurrence
[16].
- X-ray:
The CXR can be normal in up to 37% of cases [9]. Radiographically
visible calcification is present in up to 20% of cases (more common
with right atrial lesions- up to half of
right atrial
tumors can have visible calcification on CXR [9]) [6]. Patients with
left atrial tumors may exhibit findings
similar to mitral valve
disease with left atrial enlargement (for
left atrial tumors). Pleural
effusion
in
up
to
15%
of cases [9].
On CT the lesion is usually a well-defined, lobulated,
intracavitary cardiac mass [6]. The lesion
usually
arises from a narrow stalk- most often from the fossa
ovalis (this differs from thrombi that most
commonly
originate from the atrial appendage) [13].
The lesion
is usually of lower density than unopacified
blood on
non-contrast images (HU 43 +/- 14 [17]), but the lesion is best
identified
after contrast administration [6]. The lesion is commonly heterogeneous
reflecting hemorrhage, necrosis, fibrosis, and calcification [6] and is
of
lower density than the myocardium [9].
On MR the tumor is usually heterogeneous [9] and has a
signal intensity similar to cardiac muscle on T1-images [6] and
has
increased signal on T2 FSE images [7]. Following gadolinium
administration the
lesion enhances heterogeneously [6]. Myxomatous
components of the lesion appear of low signal on T1 and high signal on
T2
images [6]. Calcification will be of low signal on T1 and T2 images
[6]. The
tumor appears of low signal intensity on GRE cine images and these
images can
be used to demonstrate tumor motion and prolapse
across the atrioventricular valve [6].
On PET imaging, cardiac myxomas generally demonstrate very low-grade
or no significant FDG activity [18].
|
Right atrial myxoma: The patient below presented
with pulmonary infiltrates and fevers. Chest CT was performed and
demonstrated a possible lesion in the right atrial
appendage. A follow-up cardiac MR confirmed a solid mass in the right atrial appendage (white arrows). The mass was
surgically resected and found to be a myxoma.
|
|
  
|
- Clinical:
Papillary fibroelastomas are benign endocardial papillomas
that
account for 10% of cardiac neoplasms and
predominantly affect the cardiac valves (90% of lesions occur on
cardiac
valves) [7,11,16]. It is a rare lesion, but
it is the
second most common benign cardiac neoplasm [6,22] (however, other
authors
report
that lipomas are the second most common
benign
neoplasm [16]). Most lesions are solitary and small (10-20mm) [16]. The
lesion
is characterized by avascular papillary
fronds of
connective tissue covered by a single layer of hypertrophied
endothelium, with stroma of central
amorphous fibrous core and outer loose
connective tissue [16].
The lesion is more common on the aortic (29-44%) and mitral (25-35%)
valves,
than on the tricuspid (15-17%) and pulmonary (8-13%) valves
[7,21,22]. About
16%
of
lesions arise from non-valvular surfaces
(endothelial
surface of the LV apex, the chordae tendineae, or LV outflow tract) [7,16,22].
Men and women are affected equally with a mean age at presentation of
60 years
(peak incidence is in the 7th and 8th decades of life [21]) [6]. Most
lesions are asymptomatic and are found incidentally at
autopsy,
cardiac surgery, or cardiac catheterization [6,7].
Symptomatic
patients
present
as
a
result of systemic embolization
of the tumor or thrombi on the tumor surface and may complain of chest
pain,
TIA's or stroke, dyspnea, or sudden death
(due to
coronary artery ostia occlusion) [14]. The
embolic
fragments may arise from the tumor itself or from platelet/fibrin clots
on the
lesion surface [6,16]. Treatment is surgical
excision
and recurrence has not been reported [6].
Differential considerations would include a vegetation or thrombus
[11].
Vegetations are usually associated with destruction of the valve
leaflets and valvular incompetence [11]. Myxomas
can also occur on valves, but they are generally larger [11].
- X-ray:
Most lesions are imaged with echocardiography which reveals a small
(under 1-1.5 cm), mobile, pedunculated,
homogeneous
valvular or endocardial
mass
which flutters or prolapses with cardiac
motion [6].
The lesion is usually not evident on routine CT or MR imaging
[7]. If
seen, they are usually of intermediate or isointense signal intensity
on T1 images
and hyperintense on T2 FSE images [14,22].
The lesion can be seen
on cine gradient images where it appears as a hypointense
mobile mass with turbulent flow surrounding it [11,14,22].
Paraganglioma
(Chemodectoma):
- Clinical:
Cardiac paragangliomas are extremely
rare neoplasms that arise from intrinsic
cardiac paraganglial (chromaffin)
cells
which
are
predominantly
located
in the atria (LA posterior wall or roof
and
less commonly the interatrial septum), AV
groove, or
near the roots of the great vessels [6,15,16].
A
chemodectoma is a non-functioning paraganglioma
[16]. Most lesions have been reported in adults (mean age 40 years)
[6]. The
majority of reported lesions have been catecholamine-producing tumors.
Up to
one-half of affected patients present with symptoms of catecholamine
excess [15]- hypertension, palpitations, and
flushing. Other clinical
manifestations can result from mass effects of the tumor including
invasion of
the conducting system, compression of coronary blood flow, pericardial
involvement, or disruption of valvular
function [15].
Lab analysis will reveal elevated levels of urinary norepinephrine
and vanillylmandelic acid [6]. Up to 20%
of affected
patients will have associated paragangliomas
in other
locations. Osseous metastases are found in 5% of cases. Surgery is the
treatment of choice and it is often successful. A hypertensive crisis
is a
potential complication of surgical resection [6].
- X-ray:
Chest radiographs will demonstrate a middle mediastinal
mass that splays the carina and may simulate left atrial
enlargement [6]. Prior to CT scanning patients should be premedicated
with alpha and beta blockers to prevent a hypertensive crisis [6]. CT
will
demonstrate a markedly enhancing mass adherent to or involving the left
atrium
(less commonly the mass can be located anterior to the aortic root).
The lesion
is typically located in the roof of the left atrium. Unlike myxomas,
the lesion often has a broad base attachment [7]. About 50% of lesion have areas of central low attenuation due
to
necrosis. The lesions may contain calcification [6]. The lesion
demonstrates
intense, heterogeneous contrast enhancement [16]. The lesion may be
missed on
unenhanced or poorly enhanced scans as the mass may appear isodense
to adjacent cardiovascular structures [6]. MR will demonstrate a low
signal
mass on T1 images which is very hyperintense
on T2
images [6,7].
Rhabdomyoma/Rhabdomyosarcoma:
- Clinical:
Rhabdoid tumors arise from striated
muscle. Rhabdomyoma is the most common
primary pediatric cardiac
tumor- accounting for up to 90% of cardiac tumors in infants and
children [6].
The lesion is usually found in patients under
1 year
of age [6]. Presentation in adults in rare [16].
It is
a hamartomatous lesion and is associated
with
tuberous sclerosis in 50% of patients [11]. The tumors are frequently
multiple
(up to 90% of cases [11]) and intramural. The lesion most commonly
arises from
the ventricular wall (with equal frequency on the left or right [16])
and interventricular septum. Atrial
wall involvement is much less common. Most patients are asymptomatic,
but the
tumor may produce obstruction to blood flow, murmur, arrhythmia, heart
failure,
or fetal hydrops. Spontaneous tumor
regression may
occur, particularly in patients less than 4 years of age, and surgical
resection is not required unless there are clinical symptoms (such as dysrhythmias or heart failure) [14]. The
prognosis is good,
and management is conservative in the absence of life threatening
symptoms.
Rhabdomyosarcomas are the most common
cardiac
malignancy in infants and children [4,11].
The pleomorphic subtype is uncommon, but
is more frequent in
adults [11]. There is a slight male predilection [4]. Congestive heart
failure
is a common presenting symptom [11]. The lesion is often mutliple
and can invade the pericardium [4]. The lesion is also more likely to
involve
the cardiac valves than any other primary cardiac sarcoma [11]. On CT
the
lesion appears as a smooth or irregular low attenuation mass in the
cardiac
chamber. The lesion is isointense to
myocardium on T1
images and enhancement is generally homogeneous, although areas of low
signal
can be seen in association with central necrosis [11]. The lesion can
have
homogeneous or heterogeneous signal on MR images [4].
- X-ray:
Echocardiography: Rhabdomyomas appear
as a solid echogenic mass that may have an
intracavitary
component. When the lesions are small and multiple and diffuse
myocardial
thickening is the predominant finding [6]. On MR imaging, the tumor
typically
appears homogeneously iso- to marginally hyperintense to cardiac muscle on T1 images and
a slightly
increased signal intensity on T2 images [6,11].
Purely
intramural
lesions
may
not
be identified on MR imaging, although they
may be
seen following contrast enhancement and appear hypointense
to normal myocardium (i.e.- the lesion shows
minimal
or no enhancement) [11,14].
Teratoma:
- Clinical:
Teratomas are typically benign solitary,
cystic, intrapericardial lesions. The
lesion typically affects
infants and children [6]. They generally arise from the base of the
heart near
the origin of the great vessels. Patients usually present with
respiratory
distress and cyanosis secondary to cardiac tamponade
and compression of right sided vascular structures. If patients present
with tamponade an emergent pericardioexcntesis
is required. Treatment of the lesion is then surgical with a good
prognosis
[6].
- X-ray:
The lesion can be seen on prenatal US which demonstrates an intrapericardial multilocular
cystic mass associated with pericardial effusion and findings of fetal hydrops [6]. Chest radiographs demonstrate an
enlarged cardiomediastinal silhouette [6].
The presence of a formed
tooth can confirm the diagnosis if visible radiographically.
Echocardiography
will
reveal
an
intrapericardial
heterogeneous and complex multilocular
cystic mass,
typically positioned on the right side of the heart [6]. MR imaging
will
usually demonstrate a large mass of heterogeneous signal intensity.
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