AJR 2002; 178:727-729
© American Roentgen Ray Society
Perinatal Sonographic Diagnosis of Cardiac Fibroma with MR Imaging Correlation
Tae Hoon Kim1,
Yang Min Kim1,
Mi Young Han2,
Woong-Han Kim3,
Mee-Hye Oh4 and
Kwang Soo Han5
1
Department of Radiology, Sejong General Hospital, 91-121 Sosa-dong, Sosa-gu,
Pucheon, Kyunggi-do 422-711, South Korea.
2
Department of Pediatrics, Sejong General Hospital, Kyunggi-do 422-711, South
Korea.
3
Department of Cardiovascular Surgery, Sejong General Hospital, Kyunggi-do
422-711, South Korea.
4
Department of Pathology, Sejong General Hospital, Kyunggi-do 422-711, South
Korea.
5
Department of Obstetrics and Gynecology, Sejong General Hospital, Kyunggi-do
422-711, South Korea.
Received April 6, 2001;
accepted after revision June 14, 2001.
Address correspondence to T. H. Kim.
Introduction
Primary cardiac neoplasms are uncommon in infants and children. In an
autopsy series, the prevalence rates of cardiac tumors among all age groups
were found to range between 0.0017% and 0.28%
[1]. The most common primary
tumor in childhood is rhabdomyoma, which is followed in frequency by fibroma
and teratoma [2]. Fibromas are
intramural and usually arise from the free wall of the left ventricle or
intraventricular septum. Involvement of the right ventricle is extremely rare
[3]. We present the cases of
two patients with primary cardiac tumors that originated in the free wall of
the right ventricle. The tumors were diagnosed using sonography at 38 weeks'
gestation in one patient and on the third day of life in the other. MR imaging
was performed in both patients.
Case Report
A 3.5-kg female neonate, 39 weeks' gestational age, was born by cesarean
delivery to a 26-year-old woman whose pregnancy had been unremarkable until
the 38th week. Findings of a sonographic examination performed at 21 weeks'
gestation had been interpreted as normal. The fetal cardiac mass was detected
on routine sonography at 38 weeks' gestation. A four-chamber view of the fetal
heart revealed the presence of a homogeneously echogenic mass originating from
the free wall of the right ventricle and measuring 2.4 x 3.0 cm
(Fig. 1A). The mass did not
extend into the right atrium but slightly obstructed the right ventricular
inflow. The prenatal differential diagnosis included fibroma, teratoma, and
rhabdomyoma.
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Fig. 1A. —4-day-old female neonate with large right ventricular fibroma
diagnosed at 38 weeks' qestation. Four-chamber sonogram of fetal heart shows
large homogeneous echogenic mass (M) growing from free wall of right ventricle
that almost completely obliterates right ventricular cavity. LV = left
ventricle.
|
|
Echocardiography performed after the infant was delivered confirmed the
diagnosis of a large right ventricular tumor that was isointense relative to
muscle on T1-weighted MR images (Fig.
1B) and hypointense on T2-weighted MR images
(Fig. 1C). The
contrast-enhanced T1-weighted MR images
(Fig. 1D) showed a strongly
enhanced homogeneous solid mass with surface nodularities.
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Fig. 1B. —4-day-old female neonate with large right ventricular fibroma
diagnosed at 38 weeks' qestation. MR images show signal intensity of mass to
be slightly less than interventricular septum on T1-weighted image (B),
whereas mass appears hypointense on T2-weighted image (C) obtained 11
months later and displays homogeneous enhancement after infusion of contrast
material (D).
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Fig. 1C. —4-day-old female neonate with large right ventricular fibroma
diagnosed at 38 weeks' qestation. MR images show signal intensity of mass to
be slightly less than interventricular septum on T1-weighted image (B),
whereas mass appears hypointense on T2-weighted image (C) obtained 11
months later and displays homogeneous enhancement after infusion of contrast
material (D).
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Fig. 1D. —4-day-old female neonate with large right ventricular fibroma
diagnosed at 38 weeks' qestation. MR images show signal intensity of mass to
be slightly less than interventricular septum on T1-weighted image (B),
whereas mass appears hypointense on T2-weighted image (C) obtained 11
months later and displays homogeneous enhancement after infusion of contrast
material (D).
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When the infant was 28 days old, surgery was performed to treat progressive
hypoxia. During the procedure, a firm, solitary, whitish gray tumor was found
along the right ventricular wall. Because of the extensive involvement of the
tumor with the free wall of the right ventricle, complete excision was not
possible; a partial excisional biopsy was performed. The modified
Blalock-Taussing shunt was applied to preserve the pulmonary blood flow.
Histopathologic findings were consistent with a cardiac fibroma. The patient
had an uneventful postoperative course and was discharged from the hospital 12
days after the operation. At follow-up examination of the infant at 11 months
old, MR imaging showed an increase in tumor size to 3.0 x 3.6 cm and a
patent systemic—pulmonary shunt.
A second patient with a neonatal diagnosis of cardiac fibroma, a 3-day-old
female neonate, is shown in Figure
2A,2B.
She was initially evaluated for cardiac murmur detected after delivery. A
sonogram (Fig. 2A) was obtained
on her third day of life, and an MR image
(Fig. 2B) was obtained 3 months
later. Like the previously described patient, this infant had a large right
ventricular fibroma with homogeneous echogenecity.
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Fig. 2A. —Female neonate with large right ventricular fibroma.
Subcostal four-chamber sonogram obtained at 3 days shows large tumor (M) with
homogeneous echogenicity involving right ventricle. Right ventricular cavity
is partially obliterated.
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Fig. 2B. —Female neonate with large right ventricular fibroma.
T1-weighted MR image obtained at 3 months reveals large mass arising from
anterior free wall of right ventricle. Minimal pericardial effusion
(arrow) is visible.
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Discussion
Congenital cardiac fibromas are rare lesions that are more common in
infants and children than in adults. These tumors are benign proliferations of
connective tissue, and their tissues blend with or infiltrate normal
myocardium
[3,4].
In neonates and young infants, the tumors are cellular and have fibromyxoid
stroma with a varying number of collagen and elastic fibers. As an infant with
a fibroma matures, collagen and elastin depositions increase and cellularity
decreases. Therefore, the tumors are usually hypointense on T2-weighted MR
images and isointense relative to muscle on T1-weighted images
[3,4,5]
(Figs.
1A,1B,1C,1D
and
2A,2B).
The patterns of enhancement can range from little or no contrast material
enhancement to homogeneous enhancement to heterogeneous enhancement
[5]. One patient in our
institution had a fibroma that showed strong, homogeneous enhancement
(Fig. 1C).
Prenatal diagnosis of cardiac fibroma based on sonographic findings is
unusual, although a few cases of fetal cardiac fibroma have been described
[2,
6]. Sonographic findings are a
homogeneous echogenic mass usually arising from the interventricular septum
and the free wall of the left ventricle. However, the tumors can show the
nonhomogeneous echogenicity in a case of cystic degeneration. Involvement of
the right ventricle is extremely uncommon
[3]. In our two patients with
fibromas diagnosed during the perinatal period, the tumors were growing from
the free wall of the right ventricle. Sonography showed a large homogeneous
echogenic mass along the free wall of the right ventricle. Pericardial
effusion was evident in both patients.
The differential diagnosis for a cardiac tumor includes rhabdomyoma and
teratoma. Rhabdomyomas are the most common primary cardiac tumors and may be
associated with tuberous sclerosis
[3,
6]. Rhabdomyomas are usually
sessile and have an intracavitary component. Sonography shows that most
rhabdomyomas are multiple and have a homogeneous echogenicity. These tumors
are usually hyperintense on T2-weighted images and isointense or slightly
hyperintense compared with the adjacent myocardium on T1-weighted images
[4,
7]. Teratomas usually arise
from the pericardium. The lesions on sonograms are inhomogeneous with solid
and cystic areas. T1-weighted MR images show a heterogeneous mass with
low-signal-intensity cystic components and high-signal-intensity walls
[8].
In summary, we should include fibroma in the differential diagnosis in
patients whose perinatal sonograms reveal solid masses originating in the free
wall of the right ventricle and showing a homogeneous echogenicity. MR imaging
provides additional information on the characterization of the tissue
components.
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Introduction
Case Report
