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CASE REPORT
Year : 2021 | Volume
: 10
| Issue : 4 | Page : 121-123
Incidental tumor blush of a mass encasing right coronary artery
Shakerian Farshad, Pouraliakbar Hamidreza, Soheila Salari, Hosseini Mehdi, Kazemi Hssan
Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran
Correspondence Address:
Dr. Soheila Salari
Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran
Iran
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/rcm.rcm_55_21
A 64-year-old woman admitted to our hospital (a tertiary care center) with symptoms of chest pain and dyspnea of functional class 2. Coronary angiography showed no stenosis, but injection of right coronary artery (RCA) showed suspicious shadow around the vessel. Computed tomographic angiography was done for further evaluation of probable iatrogenic aortic dissection, and incidental tumor was found encasing RCA proximal anterior to its origin of aorta. Magnetic resonance imaging suggested lymphangioma as the most probable cause (based on the tissue characterization criteria). According to benign nature of the tumor, follow-up by imaging was recommended.
Keywords: Atypical presentation, cardiac lymphangioma, heart tumor
Primary cardiac tumors are rare. Their incidence is ranging from 0.0017% to 0.33% at autopsy.[1] Benign tumors account for 75% of all primary cardiac tumors, of which 75% are myxomas. Among adults, the most frequently encountered nonmyxomatous benign tumors are lipomas and fibromas.[1]
Cystic lymphangioma, which is usually confined to the head and neck, is a well-recognized tumor that occurs during childhood.[2] However, cardiac lymphangioma is exceptionally uncommon and a particularly rare form of cardiac disease.[1] Here, we report a case of cardiac tumor encasing right coronary artery (RCA).
Case ReportThe patient was a 64-year-old woman with symptoms of exertional chest pain and dyspnea. She had hypertension, diabetes, and dyslipidemia. Her medical history was otherwise unremarkable. Echocardiography was almost normal (ejection fraction: 50%). Coronary angiography showed normal left coronary system; however, in the first injection in RCA, dye staining outside it was noted [Video 1] and [Video 2].
RCA or even aortic dissection with/without perforation was our immediate concern. Close follow-up and serial echocardiography showed nothing significant, and the patient was totally asymptomatic.
Computed tomography angiography with contrast showed tumoral lesion with nodular calcification around RCA. Benign tumors such as hemangioma and germ cell tumor were suggested [Figure 1]. Cardiac magnetic resonance was done and suggested lymphangioma as the most probable cause [Figure 2].
Figure 1: Computed tomography. Tumoral lesions are isodense in computed tomography without contrast and without calcification and shows nodular enhancement in early phase. (a) Cardiac computed tomography without contrast and an isodense lesion (white arrow). (b) Isodense lesions in right anterolateral aortopulmonary window in early phase of cardiac computed tomography angiography. (c) Coronal reconstruction of cardiac computed tomography angiography; multiple tubular tumoral lesions with small nodular enhancement (white arrow) along the right coronary artery (yellow arrow). (d and e) Tumoral lesion with small peripheral nodular enhancement (white arrow) adjacent to right coronary artery (yellow arrow)
Figure 2: Cardiac magnetic resonance. Cardiac magnetic resonance (Mass Protocol): Tumoral lesions were seen around the right coronary artery and anterior to it with mild ring enhancement and central cyst change. In the first mass perfusion sequence, the mass had no remarkable enhancement. In the late enhancement-sequences, the mass had mild peripheral enhancement. Mild pericardial effusion was also noted. Due to magnetic resonance imaging tissue characterization criteria: Lymphangioma was the most probable diagnosis. Multiple tubular-shaped tumoral lesions were depicted from right anterolateral aspect of aortopulmonary window with extension along the right coronary artery and central cystic change and also peripheral enhancement after intravenous gadolinium enhancement at some parts are seen. (a) High signal T2 lesions (white arrow) in STIR sequence. (b) Central hyposignal lesion in T1-weighted sequence. (c) Peripheral gadolinium enhancement in postcontrast T1-weighted sequence. (d and e) Peripheral gadolinium enhancement in late phaseWe followed the patient by imaging and 6-month follow-up showed no change in symptoms or the tumor itself.
DiscussionCardiac tumors are rare and most (>80%) of these tumors are benign; myxoma is by far the most common. Myxoma constitutes approximately 50% of all benign cardiac tumors in adults, but only a small percentage of such tumors in children. Rhabdomyoma is the most common benign tumor in children and accounts for 40%–60% of cases. Other benign cardiac tumors include fibromas, lipomas, hemangiomas, papillary fibroelastomas, cystic tumors of the atrioventricular node, and paragangliomas. The remaining 20% of primary cardiac tumors are malignant and usually described as sarcomas.[3]
Cardiac lymphangioma is a very rare tumor of the heart, first reported in 1911 by Armstrong and Monckeberg.[3] Only nine cases of cardiac lymphangioma have been reported in the medical literature.[3],[4] More than half of the reported cases have occurred in patients under 10 years of age.[3],[5],[6],[7],[8]
However, cardiac lymphangioma is exceptionally uncommon and a particularly rare form of cardiac disease and is considered to be a malformation that arises from sequestration of lymphatic tissue that fails to communicate normally with the rest of the lymphatic system.[4],[9]
Cardiac lymphangiomas are commonly revealed during congestive heart failure, syncopal or embolic pathology, arrhythmias, palpitations, or cardiac tamponade.[3],[5],[7]
Our patient was neither in heart failure nor had any features of tamponade but had a history of exertional breathlessness and palpitation without any history of syncope or any embolic phenomena.
Cardiac lymhangiomas most frequently occur in the pericardial space, sometimes compressing adjacent structures; a chylous pericardial effusion may be present.[8],[9]
In our case, the mass was arising from RCA. Although there was some pericardial effusion in our case, cystic mass was arising from RCA and other sites were disease-free.
Cystic lymphangiomas have a potential risk of recurrence, especially if there has been an incomplete resection. A real treatment is complete surgical resection.[10]
Surgical treatment for cardiac lymphangioma is similar to that for other benign tumors of the heart.[10] The operative method is chosen on the basis of the size and site of the tumor. Once the disease is diagnosed, surgical intervention should be performed. If the size of the mass increased gradually, some complications could occur, such as severe arrhythmia, tumor embolism, and cardiac arrest. These complications could hinder surgical resection and decrease the survival rate.
Follow-up
Our patient was not satisfied with the surgery and underwent monthly follow-up for clinical symptoms.
ConclusionCardiac cystic lymphangioma being a rare benign cardiac tumor can have various clinical pictures and also can occur in adult age. Available imaging studies until today can only provide information regarding anatomic details, but definitive diagnosis is via biopsy which is unnecessary in most occasions regarding benign nature of the condition.
Ethical clearance
Patient consent was obtained
Acknowledgment
The authors gratefully acknowledge all who contributed to the work in presenting this case Dr. F. Shakerian, Dr. S. Salari, Dr. H. Puraliakbar, Dr. M. Hosseini, and Dr. Kazemi H.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
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