Keywords
cardiac tamponade - computed tomography - pericardial hemangioma
Case Report
A 66-year-old man presented with dyspnea on exertion of 6 months' duration. He was
evaluated elsewhere with a chest radiograph and an echocardiogram. He was detected
to have pericardial effusion and had undergone pericardiocentesis which was exudative
in nature. Fluid cytology showed predominantly lymphocytes and was negative for malignant
cells. Adenosine deaminase (ADA) was also negative. Though he had temporary relief
of symptoms after aspiration, his symptoms worsened after a few days and was referred
to our hospital with severe breathlessness. On admission, his blood pressure was 120/80 mm
Hg and pulse rate was 72/min He was tachypnoeic. On examination, his heart sounds
were muffled. Respiratory system examination showed normal vesicular breath sounds.
Other systemic examinations were unremarkable. Chest radiograph showed marked enlargement
of cardiac silhouette with cardiothoracic ratio of 94.4%. There were no features of
pulmonary congestion ([Fig. 1]). Provisional diagnosis of tuberculous/malignant pericardial effusion was considered.
Tuberculosis was considered first because of the high prevalence in India. Malignancy
was also considered as possibility because of the recurrent pericardial effusion.
Complete hemogram was done. Hemoglobin was 14.6 g/dL. Total white blood cell count
was 10.4 × 103, and erythrocyte sedimentation rate was 21 mm/h (normal up to 10 mm/h).
Echocardiogram done at our center showed presence of massive pericardial effusion
([Fig. 2]). No evidence of any debris, loculations, or septations was identified. An urgent
pericardiocentesis was done and around 2,000 mL of straw-colored (nonhemorrhagic)
fluid was aspirated. Fluid analysis showed predominant lymphocytes with no malignant
cells, protein—4.0 g/dL, and sugar—132 mg/dL. TB gold, acid-fast bacillus (AFB) stain,
and AFB culture of fluid were negative. Pericardial fluid ADA (ADA level) was normal
(6 U/L).
Fig. 1 Frontal radiograph showing markedly enlarged cardiac silhouette with sharp margins
indicating massive pericardial effusion.
Fig. 2 Two-dimensional echocardiography showing significant pericardial effusion (white
arrow).
Tumor markers were done to exclude malignant etiology. Carcinoembryonic antigen (17.4 ng/mL,
normal <3 mg/mL), prostate-specific antigen, cancer antigen 19-9, and alpha-fetoprotein
were normal. He was symptomatically better and got discharged. Again, he presented
with the same symptoms after 2 weeks. He again underwent urgent pericardiocentesis
and 1,900 mL of straw-colored fluid was aspirated.
Computed tomography (CT) scan of the thorax and abdomen, and colonoscopy were done
to look for primary malignancy in view of recurrent pericardial effusion.
Electrocardiogram-gated cardiac multidetector CT scan of the thorax revealed a soft
tissue mass lesion with lobulated margins measuring 3.6 × 3.6 cm within the pericardium
abutting the main and left pulmonary arteries. Left anterior descending artery was
seen stretched posterior to the mass. There was no calcification. The lesion showed
intense peripheral enhancement with gradual filling in and homogenous enhancement
in delayed phase ([Fig. 3A–D]).
Fig. 3 MDCT (A–D) showing heterogeneous appearing pericardial hemangioma (white arrow in A–C) with peripheral enhancement in early phase and progressive enhancement in late phase
(axial sections A, B). Coronal images (C) and volume rendered images (D) show the lesion (T) below the MPA abutting LAD. LAD, left anterior descending artery;
MDCT, multidetector computed tomography; MPA, main pulmonary artery; T, tumor.
In view of recurrent symptoms, surgical resection of lesion was planned, limited thoracotomy
showed an encapsulated mass within the pericardium abutting main pulmonary artery,
anterior to left anterior descending artery ([Fig. 4]). The mass was removed and was sent for histopathological examination.
Fig. 4 Intraoperative picture showing pericardial lesion close to the main pulmonary artery.
RVOT, right ventricle outflow tract; T, tumor.
The gross specimen measured 4 × 3 × 3 cm with brownish cut section. Histopathology
showed a vascular neoplasm composed predominantly of capillary type hemangioma having
capillary-sized small thin-walled vascular channels with feeder vessels. Focal areas
with dilated cavernous blood-filled, thin-walled vascular spaces of cavernous type
hemangioma and malformed thick- and thin-walled vascular channels of arteriovenous
type of hemangioma were also seen. The vessels were lined by bland endothelial cells
([Fig. 5A–C]).
Fig. 5 (A) Scanner view of the intrapericardial mass shows a neoplasm composed of variably
sized blood vessels. The tumor is predominantly composed of small-sized thin-walled
vessels of capillary type hemangioma (thin arrow) along with large irregular thick-walled
vessels of arteriovenous type hemangioma (thick arrow). (B) Low power view shows vessels with arteriovenous type hemangioma (thick arrow) and
capillary type hemangioma (thin arrow). (C) Low power view showing capillary type hemangioma (single arrow) with few cavernous
type vascular channels (double-headed arrow).
Discussion
Primary cardiac tumors are exceedingly rare tumors.[1] Tumors primarily arising from pericardium are even rarer. According to a case series
studied by Meng et al and Patel and Sheppard,[2]
[3] the prevalence of primary pericardial neoplasms is around 0.001 to 0.007%. Of the
primary pericardial masses, pericardial cyst is the most common. Other common benign
neoplasms are lipoma, lipoblastoma, paraganglioma, germ cell tumor, pericardial hemangioma,
fibroma, and inflammatory pseudotumor. Mesothelioma is the most common primary pericardial
malignancy.[4]
Cardiac hemangiomas comprise only 2 to 5% of benign cardiac tumors. It can occur at
any age and can be single or rarely multiple. Rarely, it can be associated with hemangiomas
at extrathoracic sites such as gastrointestinal tract, skin, or face.[5] CT of the thorax and abdomen in our case did not reveal any other hemangiomas. Pericardial
hemangiomas are extremely rare and most are found incidentally during autopsy.[6] Most of the patients are asymptomatic. In symptomatic patients, the severity of
symptoms depends on the size and location of the tumor. The common symptoms are dysrhythmia,
breathlessness, chest pain, cough, or dysphagia. It can produce pericardial effusion
which may be serous or hemorrhagic, the mechanism of which is not well understood.
The possible explanation for serous effusion is that it could be due to pericardial
friction. Hemorrhagic effusion can occur due to rupture of tumorous microvessels.[7] Sometimes pericardial hemangiomas can cause tamponade and sudden death.[8] In the case series of 12 pericardial hemangiomas studied by Seitz et al, only three
patients presented with pericardial effusion. Two cases were detected as an incidental
finding.[7] Our case was symptomatic with pericardial effusion and features of cardiac tamponade.
Diagnostic modalities for the evaluation of cardiac tumors are transthoracic echocardiography,
CT, magnetic resonance imaging (MRI), or cardiac catheterization. Transesophageal
echocardiography is more useful than transthoracic echocardiography to detect cardiac
and pericardial tumors. Echocardiography is a sensitive noninvasive tool in suspected
intracardiac tumors as a preliminary investigation and can be used to exclude other
tumors such as left atrial myxoma. Though sensitivity of echocardiography in diagnosing
a pericardial tumor is limited, flow signals in Doppler echocardiography may give
a clue toward hemangioma. However, in our case, the tumor was not identified on echocardiography.
This may be attributed to the massive pericardial effusion which may have rendered
the visualization of the tumor difficult. Also, the procedure was done on an emergency
setting with the primary goal of relieving the patient distress by pericardiocentesis.
Other factors that may have contributed to the nonvisualization of the tumor may include
the small size of the tumor and its anterior location behind the sternum. A relook
echo was done after the CT which showed a suspicious focal lesion adjacent to the
right ventricular outflow tract.
A preoperative diagnosis was possible with CT scan which showed typical features of
a vascular tumor arising from the pericardium. Because of the vascularity, hemangiomas
show intense contrast enhancement in early arterial phase and this typical feature
was demonstrable in our case. However, in some previous reports of similar cases,
it was difficult to differentiate the benign pericardial tumors preoperatively without
histology in spite of multiple imaging modalities.[9]
[10] We feel that a multidetector CT is a good imaging modality for larger pericardial
hemangiomas presenting with significant pericardial effusion.
MRI is a very good modality and can be performed as a problem solving tool. Due to
excellent contrast resolution and multiplanar capability, MRI allows optimal evaluation
of myocardial infiltration, pericardial involvement, and extracardiac extension of
cardiac tumors. At MRI, the hemangioma shows intermediate T1-weighted signal intensity
and high T2-weighted signal intensity and nodular areas of intense enhancement in
early postcontrast images with progressive filling on delayed images.[4] Coronary angiography is another useful modality which can demonstrate the feeder
vessel to the tumor with characteristic tumor blush.[11] Tumor blush is commonly seen in the capillary and arteriovenous types of hemangiomas
due to rapid blood flow.[12]
Cardiac hemangiomas arise from the endocardium, myocardium, or epicardium/pericardium.
They can occur at any chamber and most commonly found to arise from the ventricles
followed by atria.[1] Histologically, it is classified into capillary (composed of smaller capillary-like
vessels), cavernous (composed of multiple thin-walled, dilated vessels), and arteriovenous
(composed of thick-walled dysplastic arteries, venous-like vessels, and capillaries).[13] Pericardial hemangiomas mostly arise from visceral pericardium and are usually cavernous
type.[4] In our case, the tumor was arising from visceral pericardium abutting the pulmonary
artery. Among the pericardial hemangiomas presented with pericardial effusion in the
case series of Seitz et al, the tumor was seen arising from anterior wall of left
ventricle in one case and in the second case, it was originating from pericardium
next to pulmonary artery as in our case.[7]
[14] In another similar case, the tumor was located in between left atrial appendage
and pulmonary vein.[11] All these three cases proved to be capillary type hemangioma on histology. Our case
is unique with histology showing predominantly capillary type with admixtures of cavernous
and arteriovenous components.
Surgical excision is the treatment of choice for cardiac hemangioma. The prognosis
of patients with surgically treated symptomatic lesions is excellent.[15] If untreated, it can cause complications such as cardiac tamponade and sudden death.
Spontaneous regression of the tumor has also been reported.[16] There are reports of tumor regression after steroid treatment and also with radiotherapy.[17] Our patient who presented with life-threatening cardiac tamponade was successfully
managed with surgical excision. Recurrence is possible especially if incompletely
removed. This patient was followed up for 4 years and no recurrence was noted. This
in turn shows that a complete removal was achieved during surgery.
Conclusion
Pericardial hemangiomas are extremely rare. Our case presented with recurrent episodes
of pericardial effusion and features of tamponade. A proper preoperative diagnosis
was possible as multidetector CT showed typical features of vascular pericardial lesion.
A complete removal of the tumor was attained surgically. We report this case as a
unique one due to the extreme rarity of pericardial location of hemangiomas and also
the presence of all three histologic types in the same tumor.
