Abstract
Background
Cardiac hemangioma is relatively rare for primary cardiac tumors. The
diagnose is mainly based on surgical resection and biopsy, imaging
examinations only provide limited diagnostic clues. For those giant
cardiac hemangiomas, which may raise a risk of rupture, need thoroughly
surgical removal. However, meticulous follow-up is required due to its
possibility of recurrence.
Case presentation
A 62-year-old woman presented with symptoms of respiratory distress and
exercise dyspnea for two years. 8 months ago, the chest CT scan revealed
an occupation in anterior mediastinum, therefore she was admitted to
thoracic surgery department with an impression diagnosis of invasive
thymoma.
No myasthenia gravis or dysphagia was noted at her admission, blood test
for tumor markers was negative, the respiratory and cardiac portions of
the physical examination had no abnormalities. Thoracic enhanced CT scan
surprisingly revealed the mass was closely related to the right coronary
artery(RCA). Therefore, the patient was referred to cardiac surgery
department, and a detailed examination was arranged (Fig 1, A-F):
Coronary CT angiography confirmed the RCA was surrounded by a giant
mass, with inner irregular enhancement. PET-CT indicated a slightly
increase of the FDG metabolism. Coronary arteriography showed a smooth
shape of the RCA, with small branches stretched into the mass.
A “surgical resection” plan was made by the cardio-thoracic team.
During surgery, a median sternotomy approach was used, the mass was
confirmed to be in the pericardial cavity, other than thymus (Fig 2A).
The giant reddish and smooth mass was attached to the right ventricle
(RV), the border between the mass and RV was fuzzy, and a groove implied
the RCA’s trace. Cavernous hemangioma was considered by visual judgement
and frozen pathological examination. After cardiopulmonary bypass was
established with bicaval cannulation, the cardioplegic arrest was
obtained. The tumor was carefully and maximally resected, and a
pericardium patch was used to reconstruct the RV myocardium (Fig 2B).
The treatment of RCA was based on the concept of ”reservation”, the
wrapped RCA was fully preserved, intraoperative Transit Time Flow
Measurement confirmed a satisfactory flow (Fig 2C). The postoperative
course was uneventful, and the patient was discharged with no
complications.
Pathological examination demonstrated amounts of vascular structures,
stained vascular endothelial cells, which was categorized as a cavernous
hemangioma (8.5cm×6.0cm×3.0cm) (Fig 2D-H), immunohistochemical analysis
showed positive CD31, CD34, ERG staining.
Discussion
It is relatively rare for primary cardiac tumors, of cardiac
hemangiomas, is amount for 5%-10% of the benign neoplasms [1].
Whether these benign tumors need surgical treatment is still
controversial, however, due to the location, size of the lesions are
various, the surgical indications may raise for the conspicuousness of
clinical symptoms. Cardiac hemangiomas may occur in the epicardium,
myocardium, or endocardium, and all cardiac chambers. Presentations
include arrhythmias, conduction disturbances, congestive heart failure,
coronary insufficiency, chest pain, cough, and dysphagia [2].
Based on the morphologic pattern of their vascular channels, endothelial
cells, and supporting stroma, cardiac hemangiomas can be classified into
three groups: capillary, arteriovenous, and cavernous hemangiomas
[3]. Imaging examination, including echocardiography, computed
tomography, or magnetic resonance imaging, only provides limited
diagnostic clues. In this case, enhanced CT indicated irregular vascular
enhancement, and angiography indicated collateral vessels, which are
helpful for diagnosis.
Most of the results of surgical removal are satisfied, but there are
still cases that cannot be excised [4,5]. The decision to operate on
this giant tumor was based on the risk of rupture, the presence of
symptoms, and the inconsistencies in tumor location across imaging
modalities. Although the tumor often adheres to surrounding tissues, it
could be separated finely. In this case of a giant cardiac hemangioma,
with the RCA being wrapped, the operation difficulty was evident raised.
Fully preservation of the RCA, thus avoiding coronary bypass grafting
was considered as a highlight of the operation. Because of the
possibility of recurrence, fully resection is quite necessary, but
attention should be paid to minimize the damage of ventricular muscle to
avoid interrupting cardiac function.
Conclusion
In conclusion, we reported the surgical resection of a giant cardiac
hemangioma with the right coronary artery being wrapped under
cardiopulmonary bypass. Although the definitive diagnosis of primary
cardiac tumors is difficult, the postoperative CT angiography in this
patient before discharge showed thorough remove of the tumor and
sufficient reservation of the RCA. Meticulous follow-up is required for
this benign tumor after the resection to beware of recurrence.
Conflict of interest:
None.
References
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Figure 1. Images of the preoperative examination. Coronary CT
angiography (A-C) confirmed the RCA was surrounded by an
8.4cm×4.5cm×3.0cm mass (yellow asterisk). Irregular vascular enhancement
could be seen. Three-dimensional reconstruction showed the right
ventricle was oppressed (yellow arrow). PET-CT (E) indicated a slightly
increase of the FDG metabolism. Coronary arteriography (F) showed an
abnormally originated of the right coronary artery (RCA, white arrow)
from the left coronary sinus, left main (LM, white arrow) could be seen,
small branches stretched into the mass from the coronary artery was
demonstrated (yellow asterisk).
Figure 2. The reddish and smooth tumor was located pericardium (A,
yellow asterisk), the border between the mass and RV was fuzzily along
the outflow tract (A, dotted line). After resection of the tumor, the
RCA was totally reserved (B, yellow dotted line), and a pericardium
patch was used to reconstruct the injured RV myocardium (B). TTFM show a
satisfied flow of the original RCA, with 81ml/min of blood flow, and 0.6
pulsatility index (PI) (C). Pathological examination categorized a
cavernous hemangioma (8.5cm×6.0cm×3.0cm) (D, yellow asterisk), the trace
of RCA was showed at this anterior of the gross specimen (D, yellow
dotted line), hematoxylin and eosin staining (×200) showing large
vessels with a considerable amount of blood enclosed within the vascular
endothelium (E), with immunohistochemical analysis (×200) of positive
CD31, CD34, ERG staining (F-H).